Myelofibrosis pathophysiology: Difference between revisions

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*[[Polyclonal]] [[Mesenchymal cell|mesenchymal cells]] of the [[bone marrow]] such as [[fibroblasts]], [[Osteoblast|osteoblasts]], [[Pericyte|pericytes]], [[endothelial cells]], [[Adipocyte|adipocytes]], and [[Reticular cell|reticular cells]] create a functional microenvironment, which maintains [[hematopoiesis]]. This maintenance takes place through [[cellular]] interactions via growth factors, adhesion molecules, [[Cytokine|cytokines]], and [[extracellular matrix]] components along with the help of [[oxygen]] and [[calcium]].<ref name="pmid24583557">{{cite journal |vauthors=Bedekovics J, Méhes G |title=[Pathomechanism and clinical impact of myelofibrosis in neoplastic diseases of the bone marrow] |language=Hungarian |journal=Orv Hetil |volume=155 |issue=10 |pages=367–75 |date=March 2014 |pmid=24583557 |doi=10.1556/OH.2014.29823 |url=}}</ref>
*[[Polyclonal]] [[Mesenchymal cell|mesenchymal cells]] of the [[bone marrow]] such as [[fibroblasts]], [[Osteoblast|osteoblasts]], [[Pericyte|pericytes]], [[endothelial cells]], [[Adipocyte|adipocytes]], and [[Reticular cell|reticular cells]] create a functional microenvironment, which maintains [[hematopoiesis]]. This maintenance takes place through [[cellular]] interactions via growth factors, adhesion molecules, [[Cytokine|cytokines]], and [[extracellular matrix]] components along with the help of [[oxygen]] and [[calcium]].<ref name="pmid24583557">{{cite journal |vauthors=Bedekovics J, Méhes G |title=[Pathomechanism and clinical impact of myelofibrosis in neoplastic diseases of the bone marrow] |language=Hungarian |journal=Orv Hetil |volume=155 |issue=10 |pages=367–75 |date=March 2014 |pmid=24583557 |doi=10.1556/OH.2014.29823 |url=}}</ref>
*[[Myelofibrosis]] is the result of [[Pathology|pathologic]] interaction between [[hematopoietic]] [[Progenitor cell|progenitor]] and [[stromal cells]] leading to the activation and expansion of the [[stroma]] and the accumulation of [[reticulin]] and [[collagen]] fibers produced by [[Mesenchymal cell|mesenchymal cells]].<ref name="pmid24583557">{{cite journal |vauthors=Bedekovics J, Méhes G |title=[Pathomechanism and clinical impact of myelofibrosis in neoplastic diseases of the bone marrow] |language=Hungarian |journal=Orv Hetil |volume=155 |issue=10 |pages=367–75 |date=March 2014 |pmid=24583557 |doi=10.1556/OH.2014.29823 |url=}}</ref>
*[[Myelofibrosis]] is the result of [[Pathology|pathologic]] interaction between [[hematopoietic]] [[Progenitor cell|progenitor]] and [[stromal cells]] leading to the activation and expansion of the [[stroma]] and the accumulation of [[reticulin]] and [[collagen]] fibers produced by [[Mesenchymal cell|mesenchymal cells]].<ref name="pmid24583557">{{cite journal |vauthors=Bedekovics J, Méhes G |title=[Pathomechanism and clinical impact of myelofibrosis in neoplastic diseases of the bone marrow] |language=Hungarian |journal=Orv Hetil |volume=155 |issue=10 |pages=367–75 |date=March 2014 |pmid=24583557 |doi=10.1556/OH.2014.29823 |url=}}</ref>
*The development and progression of [[myelofibrosis]] involves the activation of Janus kinase-signal transducer and activator of transcription (JAK/STAT) pathway, which paves the way for the overproduction of abnormal [[megakaryocytes]].<ref name="pmid28028029">{{cite journal |vauthors=Vainchenker W, Kralovics R |title=Genetic basis and molecular pathophysiology of classical myeloproliferative neoplasms |journal=Blood |volume=129 |issue=6 |pages=667–679 |date=February 2017 |pmid=28028029 |doi=10.1182/blood-2016-10-695940 |url=}}</ref><ref name="pmid27756071">{{cite journal |vauthors=Alshemmari SH, Rajan R, Emadi A |title=Molecular Pathogenesis and Clinical Significance of Driver Mutations in Primary Myelofibrosis: A Review |journal=Med Princ Pract |volume=25 |issue=6 |pages=501–509 |date=2016 |pmid=27756071 |pmc=5588514 |doi=10.1159/000450956 |url=}}</ref><ref name="pmid26408371">{{cite journal |vauthors=de Freitas RM, da Costa Maranduba CM |title=Myeloproliferative neoplasms and the JAK/STAT signaling pathway: an overview |journal=Rev Bras Hematol Hemoter |volume=37 |issue=5 |pages=348–53 |date=2015 |pmid=26408371 |pmc=4685044 |doi=10.1016/j.bjhh.2014.10.001 |url=}}</ref>
*The development and progression of [[myelofibrosis]] involves the activation of Janus kinase-signal transducer and activator of transcription ([[JAK-STAT signaling pathway|JAK/STAT]]) pathway, which paves the way for the overproduction of abnormal [[megakaryocytes]].<ref name="pmid28028029">{{cite journal |vauthors=Vainchenker W, Kralovics R |title=Genetic basis and molecular pathophysiology of classical myeloproliferative neoplasms |journal=Blood |volume=129 |issue=6 |pages=667–679 |date=February 2017 |pmid=28028029 |doi=10.1182/blood-2016-10-695940 |url=}}</ref><ref name="pmid27756071">{{cite journal |vauthors=Alshemmari SH, Rajan R, Emadi A |title=Molecular Pathogenesis and Clinical Significance of Driver Mutations in Primary Myelofibrosis: A Review |journal=Med Princ Pract |volume=25 |issue=6 |pages=501–509 |date=2016 |pmid=27756071 |pmc=5588514 |doi=10.1159/000450956 |url=}}</ref><ref name="pmid26408371">{{cite journal |vauthors=de Freitas RM, da Costa Maranduba CM |title=Myeloproliferative neoplasms and the JAK/STAT signaling pathway: an overview |journal=Rev Bras Hematol Hemoter |volume=37 |issue=5 |pages=348–53 |date=2015 |pmid=26408371 |pmc=4685044 |doi=10.1016/j.bjhh.2014.10.001 |url=}}</ref>
*The abnormally proliferated [[megakaryocytes]] produce [[Cytokine|cytokines]] such as [[Platelet-derived growth factor|platelet-derived growth factor (PDGF)]], [[Transforming growth factor-β|transforming growth factor (TGF) beta]], and [[Basic fibroblast growth factor|basic fibroblast growth factor (bFGF)]] which are involved in the abnormal [[proliferation]] of [[fibroblasts]], resulting in [[fibrosis]].<ref name="pmid10550553">{{cite journal |vauthors=Le Bousse-Kerdilès MC, Martyré MC |title=Dual implication of fibrogenic cytokines in the pathogenesis of fibrosis and myeloproliferation in myeloid metaplasia with myelofibrosis |journal=Ann. Hematol. |volume=78 |issue=10 |pages=437–44 |date=October 1999 |pmid=10550553 |doi= |url=}}</ref><ref name="pmid17910625">{{cite journal |vauthors=Kuter DJ, Bain B, Mufti G, Bagg A, Hasserjian RP |title=Bone marrow fibrosis: pathophysiology and clinical significance of increased bone marrow stromal fibres |journal=Br. J. Haematol. |volume=139 |issue=3 |pages=351–62 |date=November 2007 |pmid=17910625 |doi=10.1111/j.1365-2141.2007.06807.x |url=}}</ref><ref name="pmid8435338">{{cite journal |vauthors=Reilly JT, Barnett D, Dolan G, Forrest P, Eastham J, Smith A |title=Characterization of an acute micromegakaryocytic leukaemia: evidence for the pathogenesis of myelofibrosis |journal=Br. J. Haematol. |volume=83 |issue=1 |pages=58–62 |date=January 1993 |pmid=8435338 |doi= |url=}}</ref><ref name="pmid12153156">{{cite journal |vauthors=Schmitt A, Drouin A, Massé JM, Guichard J, Shagraoui H, Cramer EM |title=Polymorphonuclear neutrophil and megakaryocyte mutual involvement in myelofibrosis pathogenesis |journal=Leuk. Lymphoma |volume=43 |issue=4 |pages=719–24 |date=April 2002 |pmid=12153156 |doi=10.1080/10428190290016809 |url=}}</ref><ref name="pmid10942376">{{cite journal |vauthors=Schmitt A, Jouault H, Guichard J, Wendling F, Drouin A, Cramer EM |title=Pathologic interaction between megakaryocytes and polymorphonuclear leukocytes in myelofibrosis |journal=Blood |volume=96 |issue=4 |pages=1342–7 |date=August 2000 |pmid=10942376 |doi= |url=}}</ref><ref name="pmid27252511">{{cite journal |vauthors=Zahr AA, Salama ME, Carreau N, Tremblay D, Verstovsek S, Mesa R, Hoffman R, Mascarenhas J |title=Bone marrow fibrosis in myelofibrosis: pathogenesis, prognosis and targeted strategies |journal=Haematologica |volume=101 |issue=6 |pages=660–71 |date=June 2016 |pmid=27252511 |pmc=5013940 |doi=10.3324/haematol.2015.141283 |url=}}</ref>
*The abnormally proliferated [[megakaryocytes]] produce [[Cytokine|cytokines]] such as [[Platelet-derived growth factor|platelet-derived growth factor (PDGF)]], [[Transforming growth factor-β|transforming growth factor (TGF) beta]], and [[Basic fibroblast growth factor|basic fibroblast growth factor (bFGF)]] which are involved in the abnormal [[proliferation]] of [[fibroblasts]], resulting in [[fibrosis]].<ref name="pmid10550553">{{cite journal |vauthors=Le Bousse-Kerdilès MC, Martyré MC |title=Dual implication of fibrogenic cytokines in the pathogenesis of fibrosis and myeloproliferation in myeloid metaplasia with myelofibrosis |journal=Ann. Hematol. |volume=78 |issue=10 |pages=437–44 |date=October 1999 |pmid=10550553 |doi= |url=}}</ref><ref name="pmid17910625">{{cite journal |vauthors=Kuter DJ, Bain B, Mufti G, Bagg A, Hasserjian RP |title=Bone marrow fibrosis: pathophysiology and clinical significance of increased bone marrow stromal fibres |journal=Br. J. Haematol. |volume=139 |issue=3 |pages=351–62 |date=November 2007 |pmid=17910625 |doi=10.1111/j.1365-2141.2007.06807.x |url=}}</ref><ref name="pmid8435338">{{cite journal |vauthors=Reilly JT, Barnett D, Dolan G, Forrest P, Eastham J, Smith A |title=Characterization of an acute micromegakaryocytic leukaemia: evidence for the pathogenesis of myelofibrosis |journal=Br. J. Haematol. |volume=83 |issue=1 |pages=58–62 |date=January 1993 |pmid=8435338 |doi= |url=}}</ref><ref name="pmid12153156">{{cite journal |vauthors=Schmitt A, Drouin A, Massé JM, Guichard J, Shagraoui H, Cramer EM |title=Polymorphonuclear neutrophil and megakaryocyte mutual involvement in myelofibrosis pathogenesis |journal=Leuk. Lymphoma |volume=43 |issue=4 |pages=719–24 |date=April 2002 |pmid=12153156 |doi=10.1080/10428190290016809 |url=}}</ref><ref name="pmid10942376">{{cite journal |vauthors=Schmitt A, Jouault H, Guichard J, Wendling F, Drouin A, Cramer EM |title=Pathologic interaction between megakaryocytes and polymorphonuclear leukocytes in myelofibrosis |journal=Blood |volume=96 |issue=4 |pages=1342–7 |date=August 2000 |pmid=10942376 |doi= |url=}}</ref><ref name="pmid27252511">{{cite journal |vauthors=Zahr AA, Salama ME, Carreau N, Tremblay D, Verstovsek S, Mesa R, Hoffman R, Mascarenhas J |title=Bone marrow fibrosis in myelofibrosis: pathogenesis, prognosis and targeted strategies |journal=Haematologica |volume=101 |issue=6 |pages=660–71 |date=June 2016 |pmid=27252511 |pmc=5013940 |doi=10.3324/haematol.2015.141283 |url=}}</ref>
*[[Myelofibrosis]] can result in the setting of [[Somatic mutation|somatic mutations]] in specific [[Gene|genes]] or it can also be secondary to other primary disorders.
*[[Myelofibrosis]] can result in the setting of [[Somatic mutation|somatic mutations]] in specific [[Gene|genes]] or it can also be secondary to other primary disorders.
*The [[Somatic mutation|somatic mutations]] driving the disorder can mainly involve the [[Myeloproliferative leukemia vir|myeloproliferative leukemia virus (MPL) oncogene]], the [[Calreticulin|calreticulin (CALR) gene]], or [[Janus kinase 2|Janus kinase 2 (JAK2)]] [[gene]].<ref name="pmid27756071">{{cite journal |vauthors=Alshemmari SH, Rajan R, Emadi A |title=Molecular Pathogenesis and Clinical Significance of Driver Mutations in Primary Myelofibrosis: A Review |journal=Med Princ Pract |volume=25 |issue=6 |pages=501–509 |date=2016 |pmid=27756071 |pmc=5588514 |doi=10.1159/000450956 |url=}}</ref><ref name="pmid24325356">{{cite journal |vauthors=Klampfl T, Gisslinger H, Harutyunyan AS, Nivarthi H, Rumi E, Milosevic JD, Them NC, Berg T, Gisslinger B, Pietra D, Chen D, Vladimer GI, Bagienski K, Milanesi C, Casetti IC, Sant'Antonio E, Ferretti V, Elena C, Schischlik F, Cleary C, Six M, Schalling M, Schönegger A, Bock C, Malcovati L, Pascutto C, Superti-Furga G, Cazzola M, Kralovics R |title=Somatic mutations of calreticulin in myeloproliferative neoplasms |journal=N. Engl. J. Med. |volume=369 |issue=25 |pages=2379–90 |date=December 2013 |pmid=24325356 |doi=10.1056/NEJMoa1311347 |url=}}</ref>
*The [[Somatic mutation|somatic mutations]] driving the disorder can mainly involve the [[Myeloproliferative leukemia vir|myeloproliferative leukemia virus (MPL) oncogene]], the [[Calreticulin|calreticulin (CALR) gene]], or [[Janus kinase 2|Janus kinase 2 (JAK2)]] [[gene]].<ref name="pmid27756071">{{cite journal |vauthors=Alshemmari SH, Rajan R, Emadi A |title=Molecular Pathogenesis and Clinical Significance of Driver Mutations in Primary Myelofibrosis: A Review |journal=Med Princ Pract |volume=25 |issue=6 |pages=501–509 |date=2016 |pmid=27756071 |pmc=5588514 |doi=10.1159/000450956 |url=}}</ref><ref name="pmid24325356">{{cite journal |vauthors=Klampfl T, Gisslinger H, Harutyunyan AS, Nivarthi H, Rumi E, Milosevic JD, Them NC, Berg T, Gisslinger B, Pietra D, Chen D, Vladimer GI, Bagienski K, Milanesi C, Casetti IC, Sant'Antonio E, Ferretti V, Elena C, Schischlik F, Cleary C, Six M, Schalling M, Schönegger A, Bock C, Malcovati L, Pascutto C, Superti-Furga G, Cazzola M, Kralovics R |title=Somatic mutations of calreticulin in myeloproliferative neoplasms |journal=N. Engl. J. Med. |volume=369 |issue=25 |pages=2379–90 |date=December 2013 |pmid=24325356 |doi=10.1056/NEJMoa1311347 |url=}}</ref>
*The [[fibrosis]] of [[bone marrow]] leads to [[Extramedullary hematopoiesis|extramedullary hematopoiesis (EMH)]] involving the [[Reticuloendothelial system|reticuloendothelial organs]] such as the [[liver]] and [[spleen]]. Rarely, the [[Extramedullary hematopoiesis|extramedullary hematopoiesis (EMH)]] can also involve [[ectopic]] [[hematopoietic]] [[tissue]] which includes the [[skin]], [[Lymph node|lymph nodes]], [[Lung|lungs]], [[gastrointestinal tract]], [[peritoneum]], [[central nervous system]], and [[genital]] and [[urinary tracts]].<ref name="pmid11843900">{{cite journal |vauthors=Mak YK, Chan CH, So CC, Chan MK, Chu YC |title=Idiopathic myelofibrosis with extramedullary haemopoiesis involving the urinary bladder in a Chinese lady |journal=Clin Lab Haematol |volume=24 |issue=1 |pages=55–9 |date=February 2002 |pmid=11843900 |doi= |url=}}</ref><ref name="pmid28821362">{{cite journal |vauthors=Philipponnet C, Ronco P, Aniort J, Kemeny JL, Heng AE |title=Membranous Nephropathy and Intrarenal Extramedullary Hematopoiesis in a Patient With Myelofibrosis |journal=Am. J. Kidney Dis. |volume=70 |issue=6 |pages=874–877 |date=December 2017 |pmid=28821362 |doi=10.1053/j.ajkd.2017.06.022 |url=}}</ref><ref name="pmid27883206">{{cite journal |vauthors=Yang M, Roarke M |title=Diffuse pulmonary extramedullary hematopoiesis in myelofibrosis diagnosed with technetium-99m sulfur colloid bone marrow scintigraphy and single photon emission computerized tomography/CT |journal=Am. J. Hematol. |volume=92 |issue=3 |pages=323–324 |date=March 2017 |pmid=27883206 |doi=10.1002/ajh.24616 |url=}}</ref><ref name="pmid27883206">{{cite journal |vauthors=Yang M, Roarke M |title=Diffuse pulmonary extramedullary hematopoiesis in myelofibrosis diagnosed with technetium-99m sulfur colloid bone marrow scintigraphy and single photon emission computerized tomography/CT |journal=Am. J. Hematol. |volume=92 |issue=3 |pages=323–324 |date=March 2017 |pmid=27883206 |doi=10.1002/ajh.24616 |url=}}</ref><ref name="pmid27521149">{{cite journal |vauthors=Pizzi M, Gergis U, Chaviano F, Orazi A |title=The effects of hematopoietic stem cell transplant on splenic extramedullary hematopoiesis in patients with myeloproliferative neoplasm-associated myelofibrosis |journal=Hematol Oncol Stem Cell Ther |volume=9 |issue=3 |pages=96–104 |date=September 2016 |pmid=27521149 |doi=10.1016/j.hemonc.2016.07.002 |url=}}</ref>
*The [[fibrosis]] of [[bone marrow]] leads to [[Extramedullary hematopoiesis|extramedullary hematopoiesis (EMH)]] involving the [[Reticuloendothelial system|reticuloendothelial organs]] such as the [[liver]] and [[spleen]]. Rarely, the [[Extramedullary hematopoiesis|extramedullary hematopoiesis (EMH)]] can also involve [[ectopic]] [[hematopoietic]] [[tissue]] which includes the [[skin]], [[Lymph node|lymph nodes]], [[Lung|lungs]], [[gastrointestinal tract]], [[peritoneum]], [[central nervous system]], [[genital]], and [[urinary tracts]].<ref name="pmid11843900">{{cite journal |vauthors=Mak YK, Chan CH, So CC, Chan MK, Chu YC |title=Idiopathic myelofibrosis with extramedullary haemopoiesis involving the urinary bladder in a Chinese lady |journal=Clin Lab Haematol |volume=24 |issue=1 |pages=55–9 |date=February 2002 |pmid=11843900 |doi= |url=}}</ref><ref name="pmid28821362">{{cite journal |vauthors=Philipponnet C, Ronco P, Aniort J, Kemeny JL, Heng AE |title=Membranous Nephropathy and Intrarenal Extramedullary Hematopoiesis in a Patient With Myelofibrosis |journal=Am. J. Kidney Dis. |volume=70 |issue=6 |pages=874–877 |date=December 2017 |pmid=28821362 |doi=10.1053/j.ajkd.2017.06.022 |url=}}</ref><ref name="pmid27883206">{{cite journal |vauthors=Yang M, Roarke M |title=Diffuse pulmonary extramedullary hematopoiesis in myelofibrosis diagnosed with technetium-99m sulfur colloid bone marrow scintigraphy and single photon emission computerized tomography/CT |journal=Am. J. Hematol. |volume=92 |issue=3 |pages=323–324 |date=March 2017 |pmid=27883206 |doi=10.1002/ajh.24616 |url=}}</ref><ref name="pmid27883206">{{cite journal |vauthors=Yang M, Roarke M |title=Diffuse pulmonary extramedullary hematopoiesis in myelofibrosis diagnosed with technetium-99m sulfur colloid bone marrow scintigraphy and single photon emission computerized tomography/CT |journal=Am. J. Hematol. |volume=92 |issue=3 |pages=323–324 |date=March 2017 |pmid=27883206 |doi=10.1002/ajh.24616 |url=}}</ref><ref name="pmid27521149">{{cite journal |vauthors=Pizzi M, Gergis U, Chaviano F, Orazi A |title=The effects of hematopoietic stem cell transplant on splenic extramedullary hematopoiesis in patients with myeloproliferative neoplasm-associated myelofibrosis |journal=Hematol Oncol Stem Cell Ther |volume=9 |issue=3 |pages=96–104 |date=September 2016 |pmid=27521149 |doi=10.1016/j.hemonc.2016.07.002 |url=}}</ref>
*[[Extramedullary hematopoiesis|Extramedullary hematopoiesis (EMH)]] in the [[spleen]] of patients with [[Primary myelofibrosis|primary myelofibrosis (PMF)]] can lead to abnormal [[angiogenesis]] in the [[Organ (anatomy)|organ]] and it has been documented that [[Monocyte|monocytes]] expressing the angiopoietin-2 receptor (Tie2) play a role in starting/maintaining this [[pathological]] [[angiogenesis]].<ref name="pmid27281335">{{cite journal |vauthors=Campanelli R, Fois G, Catarsi P, Poletto V, Villani L, Erba BG, Maddaluno L, Jemos B, Salmoiraghi S, Guglielmelli P, Abbonante V, Di Buduo CA, Balduini A, Iurlo A, Barosi G, Rosti V, Massa M |title=Tie2 Expressing Monocytes in the Spleen of Patients with Primary Myelofibrosis |journal=PLoS ONE |volume=11 |issue=6 |pages=e0156990 |date=2016 |pmid=27281335 |pmc=4900622 |doi=10.1371/journal.pone.0156990 |url=}}</ref>
*[[Extramedullary hematopoiesis|Extramedullary hematopoiesis (EMH)]] in the [[spleen]] of patients with [[Primary myelofibrosis|primary myelofibrosis (PMF)]] can lead to abnormal [[angiogenesis]] in the [[Organ (anatomy)|organ]] and it has been documented that [[Monocyte|monocytes]] expressing the angiopoietin-2 receptor (Tie2) play a role in starting/maintaining this [[pathological]] [[angiogenesis]].<ref name="pmid27281335">{{cite journal |vauthors=Campanelli R, Fois G, Catarsi P, Poletto V, Villani L, Erba BG, Maddaluno L, Jemos B, Salmoiraghi S, Guglielmelli P, Abbonante V, Di Buduo CA, Balduini A, Iurlo A, Barosi G, Rosti V, Massa M |title=Tie2 Expressing Monocytes in the Spleen of Patients with Primary Myelofibrosis |journal=PLoS ONE |volume=11 |issue=6 |pages=e0156990 |date=2016 |pmid=27281335 |pmc=4900622 |doi=10.1371/journal.pone.0156990 |url=}}</ref>
===Sites of Extramedullary Hematopoiesis===
===Sites of Extramedullary Hematopoiesis===
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::*[[Multiple sclerosis]] [MS]
::*[[Multiple sclerosis]] [MS]
::*[[Sjögren's syndrome|Sjogren's syndrome]]
::*[[Sjögren's syndrome|Sjogren's syndrome]]
::*[[Juvenile idiopathic arthritis]])
::*[[Juvenile idiopathic arthritis]]
:*[[Endocrine]] disorders ([[Hyperparathyroidism|primary hyperparathyroidism]])<ref name="pmid17988303">{{cite journal |vauthors=Lim DJ, Oh EJ, Park CW, Kwon HS, Hong EJ, Yoon KH, Kang MI, Cha BY, Lee KW, Son HY, Kang SK |title=Pancytopenia and secondary myelofibrosis could be induced by primary hyperparathyroidism |journal=Int J Lab Hematol |volume=29 |issue=6 |pages=464–8 |date=December 2007 |pmid=17988303 |doi=10.1111/j.1365-2257.2006.00877.x |url=}}</ref>
:*[[Endocrine]] disorders such as:
:**[[Primary hyperparathyroidism]]<ref name="pmid17988303">{{cite journal |vauthors=Lim DJ, Oh EJ, Park CW, Kwon HS, Hong EJ, Yoon KH, Kang MI, Cha BY, Lee KW, Son HY, Kang SK |title=Pancytopenia and secondary myelofibrosis could be induced by primary hyperparathyroidism |journal=Int J Lab Hematol |volume=29 |issue=6 |pages=464–8 |date=December 2007 |pmid=17988303 |doi=10.1111/j.1365-2257.2006.00877.x |url=}}</ref>
:*[[SPD|Delta-storage pool deficiency (SPD)]]<ref name="pmid11042524">{{cite journal |vauthors=Mouly S, Youssefian T, Souni F, Cramer E, Lefrere F, Varet B, Hermine O |title=Acquired delta-storage pool deficiency associated with idiopathic myelofibrosis |journal=Leuk. Lymphoma |volume=37 |issue=5-6 |pages=623–7 |date=May 2000 |pmid=11042524 |doi=10.3109/10428190009058516 |url=}}</ref>
:*[[SPD|Delta-storage pool deficiency (SPD)]]<ref name="pmid11042524">{{cite journal |vauthors=Mouly S, Youssefian T, Souni F, Cramer E, Lefrere F, Varet B, Hermine O |title=Acquired delta-storage pool deficiency associated with idiopathic myelofibrosis |journal=Leuk. Lymphoma |volume=37 |issue=5-6 |pages=623–7 |date=May 2000 |pmid=11042524 |doi=10.3109/10428190009058516 |url=}}</ref>
:*Ghosal syndrome<ref name="pmid22983925">{{cite journal |vauthors=Datta K, Karmakar M, Hira M, Halder S, Pramanik K, Banerjee G |title=Ghosal hematodiaphyseal dysplasia with myelofibrosis |journal=Indian J Pediatr |volume=80 |issue=12 |pages=1050–2 |date=December 2013 |pmid=22983925 |doi=10.1007/s12098-012-0872-z |url=}}</ref>
:*Ghosal syndrome<ref name="pmid22983925">{{cite journal |vauthors=Datta K, Karmakar M, Hira M, Halder S, Pramanik K, Banerjee G |title=Ghosal hematodiaphyseal dysplasia with myelofibrosis |journal=Indian J Pediatr |volume=80 |issue=12 |pages=1050–2 |date=December 2013 |pmid=22983925 |doi=10.1007/s12098-012-0872-z |url=}}</ref>
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==Gross Pathology==
==Gross Pathology==
*On [[gross]] [[pathology]], [[pancytopenia]] and [[Extramedullary hematopoiesis|extramedullary hematopoiesis (EMH)]] are the characteristic findings. These are manifested as [[anemia]], susceptibility to various [[Infection|infections]], [[lymphadenopathy]], [[hepatomegaly]], and [[splenomegaly]].<ref name="pmid26891375">{{cite journal |vauthors=Cervantes F, Correa JG, Hernandez-Boluda JC |title=Alleviating anemia and thrombocytopenia in myelofibrosis patients |journal=Expert Rev Hematol |volume=9 |issue=5 |pages=489–96 |date=May 2016 |pmid=26891375 |doi=10.1586/17474086.2016.1154452 |url=}}</ref><ref name="pmid25944376">{{cite journal |vauthors=Hernández-Boluda JC, Martínez-Trillos A, García-Gutiérrez V, Ferrer-Marín F, Xicoy B, Alvarez-Larrán A, Kerguelen A, Barba P, Gómez M, Herrera JC, Correa JG, Cervantes F |title=Long-term results of prednisone treatment for the anemia of myelofibrosis |journal=Leuk. Lymphoma |volume=57 |issue=1 |pages=120–4 |date=2016 |pmid=25944376 |doi=10.3109/10428194.2015.1046866 |url=}}</ref><ref name="pmid26537421">{{cite journal |vauthors=Ungprasert P, Chowdhary VR, Davis MD, Makol A |title=Autoimmune myelofibrosis with pancytopenia as a presenting manifestation of systemic lupus erythematosus responsive to mycophenolate mofetil |journal=Lupus |volume=25 |issue=4 |pages=427–30 |date=April 2016 |pmid=26537421 |doi=10.1177/0961203315615221 |url=}}</ref><ref name="pmid26422082">{{cite journal |vauthors=Tang VK, Huh YO, Tayar JH, Rojas Hernandez CM |title=Primary autoimmune myelofibrosis as etiology of pancytopenia mimicking myelodysplastic syndrome |journal=Leuk. Lymphoma |volume=57 |issue=3 |pages=731–4 |date=2016 |pmid=26422082 |doi=10.3109/10428194.2015.1076931 |url=}}</ref><ref name="pmid27870387">{{cite journal |vauthors=Tefferi A |title=Primary myelofibrosis: 2017 update on diagnosis, risk-stratification, and management |journal=Am. J. Hematol. |volume=91 |issue=12 |pages=1262–1271 |date=December 2016 |pmid=27870387 |doi=10.1002/ajh.24592 |url=}}</ref><ref name="pmid28011890">{{cite journal |vauthors=Panda A, Chandrashekhara SH, Nambirajan A, Mishra P |title=Idiopathic myelofibrosis with disseminated hepatosplenic, mesenteric, renal and pulmonary extramedullary haematopoeisis, portal hypertension and tuberculosis: initial presentation and 2 years follow-up |journal=BMJ Case Rep |volume=2016 |issue= |pages= |date=December 2016 |pmid=28011890 |doi=10.1136/bcr-2016-217854 |url=}}</ref><ref name="pmid14528107">{{cite journal |vauthors=Gruner BA, DeNapoli TS, Elshihabi S, Britton HA, Langevin AM, Thomas PJ, Weitman SD |title=Anemia and hepatosplenomegaly as presenting features in a child with rickets and secondary myelofibrosis |journal=J. Pediatr. Hematol. Oncol. |volume=25 |issue=10 |pages=813–5 |date=October 2003 |pmid=14528107 |doi= |url=}}</ref><ref name="pmid10460376">{{cite journal |vauthors=Guermazi A, de Kerviler E, Cazals-Hatem D, Zagdanski AM, Frija J |title=Imaging findings in patients with myelofibrosis |journal=Eur Radiol |volume=9 |issue=7 |pages=1366–75 |date=1999 |pmid=10460376 |doi=10.1007/s003300050850 |url=}}</ref><ref name="pmid20235305">{{cite journal |vauthors=Merry GM, Aronowitz PB |title=Myelofibrosis with massive hepatosplenomegaly and osteolytic bone lesions |journal=J Hosp Med |volume=5 |issue=3 |pages=E27–8 |date=March 2010 |pmid=20235305 |doi=10.1002/jhm.459 |url=}}</ref>
*On [[gross]] [[pathology]], [[Extramedullary hematopoiesis|extramedullary hematopoiesis (EMH)]] is the characteristic finding which manifested as:<ref name="pmid26891375">{{cite journal |vauthors=Cervantes F, Correa JG, Hernandez-Boluda JC |title=Alleviating anemia and thrombocytopenia in myelofibrosis patients |journal=Expert Rev Hematol |volume=9 |issue=5 |pages=489–96 |date=May 2016 |pmid=26891375 |doi=10.1586/17474086.2016.1154452 |url=}}</ref><ref name="pmid25944376">{{cite journal |vauthors=Hernández-Boluda JC, Martínez-Trillos A, García-Gutiérrez V, Ferrer-Marín F, Xicoy B, Alvarez-Larrán A, Kerguelen A, Barba P, Gómez M, Herrera JC, Correa JG, Cervantes F |title=Long-term results of prednisone treatment for the anemia of myelofibrosis |journal=Leuk. Lymphoma |volume=57 |issue=1 |pages=120–4 |date=2016 |pmid=25944376 |doi=10.3109/10428194.2015.1046866 |url=}}</ref><ref name="pmid26537421">{{cite journal |vauthors=Ungprasert P, Chowdhary VR, Davis MD, Makol A |title=Autoimmune myelofibrosis with pancytopenia as a presenting manifestation of systemic lupus erythematosus responsive to mycophenolate mofetil |journal=Lupus |volume=25 |issue=4 |pages=427–30 |date=April 2016 |pmid=26537421 |doi=10.1177/0961203315615221 |url=}}</ref><ref name="pmid26422082">{{cite journal |vauthors=Tang VK, Huh YO, Tayar JH, Rojas Hernandez CM |title=Primary autoimmune myelofibrosis as etiology of pancytopenia mimicking myelodysplastic syndrome |journal=Leuk. Lymphoma |volume=57 |issue=3 |pages=731–4 |date=2016 |pmid=26422082 |doi=10.3109/10428194.2015.1076931 |url=}}</ref><ref name="pmid27870387" /><ref name="pmid28011890">{{cite journal |vauthors=Panda A, Chandrashekhara SH, Nambirajan A, Mishra P |title=Idiopathic myelofibrosis with disseminated hepatosplenic, mesenteric, renal and pulmonary extramedullary haematopoeisis, portal hypertension and tuberculosis: initial presentation and 2 years follow-up |journal=BMJ Case Rep |volume=2016 |issue= |pages= |date=December 2016 |pmid=28011890 |doi=10.1136/bcr-2016-217854 |url=}}</ref><ref name="pmid14528107">{{cite journal |vauthors=Gruner BA, DeNapoli TS, Elshihabi S, Britton HA, Langevin AM, Thomas PJ, Weitman SD |title=Anemia and hepatosplenomegaly as presenting features in a child with rickets and secondary myelofibrosis |journal=J. Pediatr. Hematol. Oncol. |volume=25 |issue=10 |pages=813–5 |date=October 2003 |pmid=14528107 |doi= |url=}}</ref><ref name="pmid10460376">{{cite journal |vauthors=Guermazi A, de Kerviler E, Cazals-Hatem D, Zagdanski AM, Frija J |title=Imaging findings in patients with myelofibrosis |journal=Eur Radiol |volume=9 |issue=7 |pages=1366–75 |date=1999 |pmid=10460376 |doi=10.1007/s003300050850 |url=}}</ref><ref name="pmid20235305">{{cite journal |vauthors=Merry GM, Aronowitz PB |title=Myelofibrosis with massive hepatosplenomegaly and osteolytic bone lesions |journal=J Hosp Med |volume=5 |issue=3 |pages=E27–8 |date=March 2010 |pmid=20235305 |doi=10.1002/jhm.459 |url=}}</ref>
**[[Lymphadenopathy]]
**[[Hepatomegaly]]
**[[Splenomegaly]]
==Microscopic Pathology==
==Microscopic Pathology==
===On Light Microscopy===
===On Light Microscopy===
*Low [[Red blood cell|red blood cell (RBC)]] count manisfesting as [[anemia]].<ref name="pmid16202684">{{cite journal |vauthors=Thiele J, Kvasnicka HM |title=Hematopathologic findings in chronic idiopathic myelofibrosis |journal=Semin. Oncol. |volume=32 |issue=4 |pages=380–94 |date=August 2005 |pmid=16202684 |doi=10.1053/j.seminoncol.2005.04.010 |url=}}</ref><ref name="pmid26891375">{{cite journal |vauthors=Cervantes F, Correa JG, Hernandez-Boluda JC |title=Alleviating anemia and thrombocytopenia in myelofibrosis patients |journal=Expert Rev Hematol |volume=9 |issue=5 |pages=489–96 |date=May 2016 |pmid=26891375 |doi=10.1586/17474086.2016.1154452 |url=}}</ref><ref name="pmid28009442">{{cite journal |vauthors=Hernández-Boluda JC, Correa JG, García-Delgado R, Martínez-López J, Alvarez-Larrán A, Fox ML, García-Gutiérrez V, Pérez-Encinas M, Ferrer-Marín F, Mata-Vázquez MI, Raya JM, Estrada N, García S, Kerguelen A, Durán MA, Albors M, Cervantes F |title=Predictive factors for anemia response to erythropoiesis-stimulating agents in myelofibrosis |journal=Eur. J. Haematol. |volume=98 |issue=4 |pages=407–414 |date=April 2017 |pmid=28009442 |doi=10.1111/ejh.12846 |url=}}</ref>
*Low [[Red blood cell|red blood cell (RBC)]] count manifesting as [[anemia]].<ref name="pmid16202684">{{cite journal |vauthors=Thiele J, Kvasnicka HM |title=Hematopathologic findings in chronic idiopathic myelofibrosis |journal=Semin. Oncol. |volume=32 |issue=4 |pages=380–94 |date=August 2005 |pmid=16202684 |doi=10.1053/j.seminoncol.2005.04.010 |url=}}</ref><ref name="pmid26891375">{{cite journal |vauthors=Cervantes F, Correa JG, Hernandez-Boluda JC |title=Alleviating anemia and thrombocytopenia in myelofibrosis patients |journal=Expert Rev Hematol |volume=9 |issue=5 |pages=489–96 |date=May 2016 |pmid=26891375 |doi=10.1586/17474086.2016.1154452 |url=}}</ref><ref name="pmid28009442">{{cite journal |vauthors=Hernández-Boluda JC, Correa JG, García-Delgado R, Martínez-López J, Alvarez-Larrán A, Fox ML, García-Gutiérrez V, Pérez-Encinas M, Ferrer-Marín F, Mata-Vázquez MI, Raya JM, Estrada N, García S, Kerguelen A, Durán MA, Albors M, Cervantes F |title=Predictive factors for anemia response to erythropoiesis-stimulating agents in myelofibrosis |journal=Eur. J. Haematol. |volume=98 |issue=4 |pages=407–414 |date=April 2017 |pmid=28009442 |doi=10.1111/ejh.12846 |url=}}</ref>
*[[Erythroblastosis]]<ref name="pmid12412736">{{cite journal |vauthors=Tóth P, Tóth Z |title=Idiopathic myelofibrosis with prominent postsplenectomy erythroblastosis terminating in acute myeloid transformation |journal=Haematologia (Budap) |volume=32 |issue=2 |pages=155–61 |date=2002 |pmid=12412736 |doi= |url=}}</ref><ref name="pmid8638637">{{cite journal |vauthors=Osman Y, Kishi K, Narita M, Saito H, Masuko M, Koike T, Shibata A |title=Idiopathic myelofibrosis with unusually high erythroblastosis in the peripheral blood |journal=Am. J. Hematol. |volume=52 |issue=2 |pages=122–3 |date=June 1996 |pmid=8638637 |doi=10.1002/(SICI)1096-8652(199606)52:2<122::AID-AJH12>3.0.CO;2-J |url=}}</ref>
*[[Erythroblastosis]]<ref name="pmid12412736">{{cite journal |vauthors=Tóth P, Tóth Z |title=Idiopathic myelofibrosis with prominent postsplenectomy erythroblastosis terminating in acute myeloid transformation |journal=Haematologia (Budap) |volume=32 |issue=2 |pages=155–61 |date=2002 |pmid=12412736 |doi= |url=}}</ref><ref name="pmid8638637">{{cite journal |vauthors=Osman Y, Kishi K, Narita M, Saito H, Masuko M, Koike T, Shibata A |title=Idiopathic myelofibrosis with unusually high erythroblastosis in the peripheral blood |journal=Am. J. Hematol. |volume=52 |issue=2 |pages=122–3 |date=June 1996 |pmid=8638637 |doi=10.1002/(SICI)1096-8652(199606)52:2<122::AID-AJH12>3.0.CO;2-J |url=}}</ref>
*Fish-shaped [[Red blood cells|red blood cells (RBCs)]] on [[peripheral blood smear]]<ref name="pmid28731851">{{cite journal |vauthors=Robier C, Körber C, Quehenberger F, Neubauer M, Wölfler A |title=The frequency of occurrence of fish-shaped red blood cells in different haematologic disorders |journal=Clin. Chem. Lab. Med. |volume=56 |issue=2 |pages=323–326 |date=January 2018 |pmid=28731851 |doi=10.1515/cclm-2017-0378 |url=}}</ref>
*Fish-shaped [[Red blood cells|red blood cells (RBCs)]] on [[peripheral blood smear]]<ref name="pmid28731851">{{cite journal |vauthors=Robier C, Körber C, Quehenberger F, Neubauer M, Wölfler A |title=The frequency of occurrence of fish-shaped red blood cells in different haematologic disorders |journal=Clin. Chem. Lab. Med. |volume=56 |issue=2 |pages=323–326 |date=January 2018 |pmid=28731851 |doi=10.1515/cclm-2017-0378 |url=}}</ref>
Line 85: Line 89:
*[[Teardrop cells]]<ref name="pmid26118701">{{cite journal |vauthors=Egelé A, van Gelder W, Riedl J |title=Automated detection and classification of teardrop cells by a novel RBC module using digital imaging/microscopy |journal=Int J Lab Hematol |volume=37 |issue=6 |pages=e153–6 |date=December 2015 |pmid=26118701 |doi=10.1111/ijlh.12399 |url=}}</ref>
*[[Teardrop cells]]<ref name="pmid26118701">{{cite journal |vauthors=Egelé A, van Gelder W, Riedl J |title=Automated detection and classification of teardrop cells by a novel RBC module using digital imaging/microscopy |journal=Int J Lab Hematol |volume=37 |issue=6 |pages=e153–6 |date=December 2015 |pmid=26118701 |doi=10.1111/ijlh.12399 |url=}}</ref>
*Increased microvascular [[density]], bizarre [[Blood vessel|vessel]] architecture, and increased number of [[pericytes]] on [[bone marrow]] study<ref name="pmid22250648">{{cite journal |vauthors=Madelung A, Bzorek M, Bondo H, Zetterberg E, Bjerrum OW, Hasselbalch HC, Scheding S, Ralfkiaer E |title=A novel immunohistochemical sequential multi-labelling and erasing technique enables epitope characterization of bone marrow pericytes in primary myelofibrosis |journal=Histopathology |volume=60 |issue=4 |pages=554–60 |date=March 2012 |pmid=22250648 |doi=10.1111/j.1365-2559.2011.04104.x |url=}}</ref><ref name="pmid10880370">{{cite journal |vauthors=Lundberg LG, Lerner R, Sundelin P, Rogers R, Folkman J, Palmblad J |title=Bone marrow in polycythemia vera, chronic myelocytic leukemia, and myelofibrosis has an increased vascularity |journal=Am. J. Pathol. |volume=157 |issue=1 |pages=15–9 |date=July 2000 |pmid=10880370 |pmc=1850191 |doi=10.1016/S0002-9440(10)64511-7 |url=}}</ref>
*Increased microvascular [[density]], bizarre [[Blood vessel|vessel]] architecture, and increased number of [[pericytes]] on [[bone marrow]] study<ref name="pmid22250648">{{cite journal |vauthors=Madelung A, Bzorek M, Bondo H, Zetterberg E, Bjerrum OW, Hasselbalch HC, Scheding S, Ralfkiaer E |title=A novel immunohistochemical sequential multi-labelling and erasing technique enables epitope characterization of bone marrow pericytes in primary myelofibrosis |journal=Histopathology |volume=60 |issue=4 |pages=554–60 |date=March 2012 |pmid=22250648 |doi=10.1111/j.1365-2559.2011.04104.x |url=}}</ref><ref name="pmid10880370">{{cite journal |vauthors=Lundberg LG, Lerner R, Sundelin P, Rogers R, Folkman J, Palmblad J |title=Bone marrow in polycythemia vera, chronic myelocytic leukemia, and myelofibrosis has an increased vascularity |journal=Am. J. Pathol. |volume=157 |issue=1 |pages=15–9 |date=July 2000 |pmid=10880370 |pmc=1850191 |doi=10.1016/S0002-9440(10)64511-7 |url=}}</ref>
*[[Leukocytosis]] and [[thrombocytosis]] in the initial stages followed by [[leukopenia]] and [[thrombocytopenia]] in the advanced stages.<ref name="pmid29631428">{{cite journal |vauthors=Magyari F, Bedekovics J, Décsy J, Ilonczai P, Illés Á, Simon Z |title=[Investigation and treatment of prefibrotic/early primary myelofibrosis. A case study] |language=Hungarian |journal=Orv Hetil |volume=159 |issue=15 |pages=603–609 |date=April 2018 |pmid=29631428 |doi=10.1556/650.2018.30995 |url=}}</ref><ref name="pmid27315113">{{cite journal |vauthors=Barraco D, Lasho TL, Gangat N, Finke C, Elala YC, Pardanani A, Tefferi A |title=Leukocytosis and presence of CALR mutation is associated with non-hepatosplenic extramedullary hematopoiesis in primary myelofibrosis |journal=Blood Cancer J |volume=6 |issue= |pages=e436 |date=June 2016 |pmid=27315113 |pmc=5141359 |doi=10.1038/bcj.2016.44 |url=}}</ref><ref name="pmid27293069">{{cite journal |vauthors=Beauverd Y, Alimam S, McLornan DP, Radia DH, Harrison CN |title=Disease characteristics and outcomes in younger adults with primary and secondary myelofibrosis |journal=Br. J. Haematol. |volume=175 |issue=1 |pages=37–42 |date=October 2016 |pmid=27293069 |doi=10.1111/bjh.14173 |url=}}</ref><ref name="pmid25519030">{{cite journal |vauthors=Ota S, Hiramatsu Y, Kondo E, Kasahara A, Takada S, Umena S, Noguchi T, Tanimoto M, Matsumura T |title=Severe case of peripheral leukocytosis initially diagnosed as myelodysplastic syndrome/myeloproliferative neoplasm, unclassifiable, but possibly prefibrotic primary myelofibrosis |journal=Acta Med. Okayama |volume=68 |issue=6 |pages=363–8 |date=December 2014 |pmid=25519030 |doi=10.18926/AMO/53025 |url=}}</ref><ref name="pmid29096334">{{cite journal |vauthors=Scotch AH, Kosiorek H, Scherber R, Dueck AC, Slot S, Zweegman S, Boekhorst PAWT, Commandeur S, Schouten H, Sackmann F, Fuentes AK, Hernández-Maraver D, Pahl HL, Griesshammer M, Stegelmann F, Döhner K, Lehmann T, Bonatz K, Reiter A, Boyer F, Etienne G, Ianotto JC, Ranta D, Roy L, Cahn JY, Harrison CN, Radia D, Muxi P, Maldonado N, Besses C, Cervantes F, Johansson PL, Barbui T, Barosi G, Vannucchi AM, Paoli C, Passamonti F, Andreasson B, Ferrari ML, Rambaldi A, Samuelsson J, Birgegard G, Xiao Z, Xu Z, Zhang Y, Sun X, Xu J, Kiladjian JJ, Zhang P, Gale RP, Mesa RA, Geyer HL |title=Symptom burden profile in myelofibrosis patients with thrombocytopenia: Lessons and unmet needs |journal=Leuk. Res. |volume=63 |issue= |pages=34–40 |date=December 2017 |pmid=29096334 |doi=10.1016/j.leukres.2017.10.002 |url=}}</ref><ref name="pmid25521305">{{cite journal |vauthors=Wassie E, Finke C, Gangat N, Lasho TL, Pardanani A, Hanson CA, Ketterling RP, Tefferi A |title=A compendium of cytogenetic abnormalities in myelofibrosis: molecular and phenotypic correlates in 826 patients |journal=Br. J. Haematol. |volume=169 |issue=1 |pages=71–6 |date=April 2015 |pmid=25521305 |doi=10.1111/bjh.13260 |url=}}</ref><ref name="pmid27264006">{{cite journal |vauthors=Guglielmelli P, Rotunno G, Pacilli A, Rumi E, Rosti V, Delaini F, Maffioli M, Fanelli T, Pancrazzi A, Pieri L, Fjerza R, Pietra D, Cilloni D, Sant'Antonio E, Salmoiraghi S, Passamonti F, Rambaldi A, Barosi G, Barbui T, Cazzola M, Vannucchi AM |title=Prognostic impact of bone marrow fibrosis in primary myelofibrosis. A study of the AGIMM group on 490 patients |journal=Am. J. Hematol. |volume=91 |issue=9 |pages=918–22 |date=September 2016 |pmid=27264006 |doi=10.1002/ajh.24442 |url=}}</ref><ref name="pmid28153839">{{cite journal |vauthors=Xu Z |title=MDS/MPN with ring sideroblasts and thrombocytosis masquerading as prefibrotic/early primary myelofibrosis |journal=Blood |volume=129 |issue=5 |pages=657 |date=February 2017 |pmid=28153839 |doi=10.1182/blood-2016-11-749937 |url=}}</ref><ref name="pmid23498669">{{cite journal |vauthors=Cheminant M, Delarue R |title=[Investigation and management of patients presenting with thrombocytosis] |language=French |journal=Rev Med Interne |volume=34 |issue=8 |pages=465–71 |date=August 2013 |pmid=23498669 |doi=10.1016/j.revmed.2013.02.020 |url=}}</ref>
*[[Leukocytosis]] and [[thrombocytosis]] in the initial stages followed by [[leukopenia]] and [[thrombocytopenia]] in the advanced stages<ref name="pmid29631428">{{cite journal |vauthors=Magyari F, Bedekovics J, Décsy J, Ilonczai P, Illés Á, Simon Z |title=[Investigation and treatment of prefibrotic/early primary myelofibrosis. A case study] |language=Hungarian |journal=Orv Hetil |volume=159 |issue=15 |pages=603–609 |date=April 2018 |pmid=29631428 |doi=10.1556/650.2018.30995 |url=}}</ref><ref name="pmid27315113">{{cite journal |vauthors=Barraco D, Lasho TL, Gangat N, Finke C, Elala YC, Pardanani A, Tefferi A |title=Leukocytosis and presence of CALR mutation is associated with non-hepatosplenic extramedullary hematopoiesis in primary myelofibrosis |journal=Blood Cancer J |volume=6 |issue= |pages=e436 |date=June 2016 |pmid=27315113 |pmc=5141359 |doi=10.1038/bcj.2016.44 |url=}}</ref><ref name="pmid27293069">{{cite journal |vauthors=Beauverd Y, Alimam S, McLornan DP, Radia DH, Harrison CN |title=Disease characteristics and outcomes in younger adults with primary and secondary myelofibrosis |journal=Br. J. Haematol. |volume=175 |issue=1 |pages=37–42 |date=October 2016 |pmid=27293069 |doi=10.1111/bjh.14173 |url=}}</ref><ref name="pmid25519030">{{cite journal |vauthors=Ota S, Hiramatsu Y, Kondo E, Kasahara A, Takada S, Umena S, Noguchi T, Tanimoto M, Matsumura T |title=Severe case of peripheral leukocytosis initially diagnosed as myelodysplastic syndrome/myeloproliferative neoplasm, unclassifiable, but possibly prefibrotic primary myelofibrosis |journal=Acta Med. Okayama |volume=68 |issue=6 |pages=363–8 |date=December 2014 |pmid=25519030 |doi=10.18926/AMO/53025 |url=}}</ref><ref name="pmid29096334">{{cite journal |vauthors=Scotch AH, Kosiorek H, Scherber R, Dueck AC, Slot S, Zweegman S, Boekhorst PAWT, Commandeur S, Schouten H, Sackmann F, Fuentes AK, Hernández-Maraver D, Pahl HL, Griesshammer M, Stegelmann F, Döhner K, Lehmann T, Bonatz K, Reiter A, Boyer F, Etienne G, Ianotto JC, Ranta D, Roy L, Cahn JY, Harrison CN, Radia D, Muxi P, Maldonado N, Besses C, Cervantes F, Johansson PL, Barbui T, Barosi G, Vannucchi AM, Paoli C, Passamonti F, Andreasson B, Ferrari ML, Rambaldi A, Samuelsson J, Birgegard G, Xiao Z, Xu Z, Zhang Y, Sun X, Xu J, Kiladjian JJ, Zhang P, Gale RP, Mesa RA, Geyer HL |title=Symptom burden profile in myelofibrosis patients with thrombocytopenia: Lessons and unmet needs |journal=Leuk. Res. |volume=63 |issue= |pages=34–40 |date=December 2017 |pmid=29096334 |doi=10.1016/j.leukres.2017.10.002 |url=}}</ref><ref name="pmid25521305">{{cite journal |vauthors=Wassie E, Finke C, Gangat N, Lasho TL, Pardanani A, Hanson CA, Ketterling RP, Tefferi A |title=A compendium of cytogenetic abnormalities in myelofibrosis: molecular and phenotypic correlates in 826 patients |journal=Br. J. Haematol. |volume=169 |issue=1 |pages=71–6 |date=April 2015 |pmid=25521305 |doi=10.1111/bjh.13260 |url=}}</ref><ref name="pmid27264006">{{cite journal |vauthors=Guglielmelli P, Rotunno G, Pacilli A, Rumi E, Rosti V, Delaini F, Maffioli M, Fanelli T, Pancrazzi A, Pieri L, Fjerza R, Pietra D, Cilloni D, Sant'Antonio E, Salmoiraghi S, Passamonti F, Rambaldi A, Barosi G, Barbui T, Cazzola M, Vannucchi AM |title=Prognostic impact of bone marrow fibrosis in primary myelofibrosis. A study of the AGIMM group on 490 patients |journal=Am. J. Hematol. |volume=91 |issue=9 |pages=918–22 |date=September 2016 |pmid=27264006 |doi=10.1002/ajh.24442 |url=}}</ref><ref name="pmid28153839">{{cite journal |vauthors=Xu Z |title=MDS/MPN with ring sideroblasts and thrombocytosis masquerading as prefibrotic/early primary myelofibrosis |journal=Blood |volume=129 |issue=5 |pages=657 |date=February 2017 |pmid=28153839 |doi=10.1182/blood-2016-11-749937 |url=}}</ref><ref name="pmid23498669">{{cite journal |vauthors=Cheminant M, Delarue R |title=[Investigation and management of patients presenting with thrombocytosis] |language=French |journal=Rev Med Interne |volume=34 |issue=8 |pages=465–71 |date=August 2013 |pmid=23498669 |doi=10.1016/j.revmed.2013.02.020 |url=}}</ref>
*[[Basophilia]]<ref name="pmid25900789">{{cite journal |vauthors=Koumas S, Prokopiou C, Lerni M, Seimeni O, Neokleous N |title=Isochromosome 17q10 associated with basophilia in primary myelofibrosis while with JAK2 inhibitor |journal=Ann. Hematol. |volume=94 |issue=8 |pages=1421–2 |date=August 2015 |pmid=25900789 |doi=10.1007/s00277-015-2380-5 |url=}}</ref><ref name="pmid27874969">{{cite journal |vauthors=Rautenbach Y, Goddard A, Clift SJ |title=Idiopathic myelofibrosis accompanied by peritoneal extramedullary hematopoiesis presenting as refractory ascites in a dog |journal=Vet Clin Pathol |volume=46 |issue=1 |pages=46–53 |date=March 2017 |pmid=27874969 |doi=10.1111/vcp.12430 |url=}}</ref><ref name="pmid9210912">{{cite journal |vauthors=Takimoto Y, Imanaka F, Hayashi Y, Shindo H |title=A patient with basophilic-eosinophilic myeloproliferative disorder showing monosomy 7 and hyperhistaminemia |journal=Acta Haematol. |volume=98 |issue=1 |pages=37–41 |date=1997 |pmid=9210912 |doi=10.1159/000203559 |url=}}</ref>
*[[Basophilia]]<ref name="pmid25900789">{{cite journal |vauthors=Koumas S, Prokopiou C, Lerni M, Seimeni O, Neokleous N |title=Isochromosome 17q10 associated with basophilia in primary myelofibrosis while with JAK2 inhibitor |journal=Ann. Hematol. |volume=94 |issue=8 |pages=1421–2 |date=August 2015 |pmid=25900789 |doi=10.1007/s00277-015-2380-5 |url=}}</ref><ref name="pmid27874969">{{cite journal |vauthors=Rautenbach Y, Goddard A, Clift SJ |title=Idiopathic myelofibrosis accompanied by peritoneal extramedullary hematopoiesis presenting as refractory ascites in a dog |journal=Vet Clin Pathol |volume=46 |issue=1 |pages=46–53 |date=March 2017 |pmid=27874969 |doi=10.1111/vcp.12430 |url=}}</ref><ref name="pmid9210912">{{cite journal |vauthors=Takimoto Y, Imanaka F, Hayashi Y, Shindo H |title=A patient with basophilic-eosinophilic myeloproliferative disorder showing monosomy 7 and hyperhistaminemia |journal=Acta Haematol. |volume=98 |issue=1 |pages=37–41 |date=1997 |pmid=9210912 |doi=10.1159/000203559 |url=}}</ref>


===On Confocal Microscopy===
===On Confocal Microscopy===
*Proplatelet (pseudopodia of [[megakaryocyte]] which extend into [[bone marrow]] sinuses to release [[Platelet|platelets]]) formation<ref name="pmid20430444">{{cite journal |vauthors=Muth M, Büsche G, Bock O, Hussein K, Kreipe H |title=Aberrant proplatelet formation in chronic myeloproliferative neoplasms |journal=Leuk. Res. |volume=34 |issue=11 |pages=1424–9 |date=November 2010 |pmid=20430444 |doi=10.1016/j.leukres.2010.03.040 |url=}}</ref>
*Proplatelet (pseudopodia of [[megakaryocyte]] which extend into [[bone marrow]] sinuses to release [[Platelet|platelets]]) formation<ref name="pmid20430444">{{cite journal |vauthors=Muth M, Büsche G, Bock O, Hussein K, Kreipe H |title=Aberrant proplatelet formation in chronic myeloproliferative neoplasms |journal=Leuk. Res. |volume=34 |issue=11 |pages=1424–9 |date=November 2010 |pmid=20430444 |doi=10.1016/j.leukres.2010.03.040 |url=}}</ref>
*[[CD14|CD14+ cells]] and [[Monocyte|monocytes]] expressing the angiopoietin-2 receptor (Tie2) lie close to the [[Blood vessel|vessels]] in the [[spleen]] [[tissue]].<ref name="pmid27281335">{{cite journal |vauthors=Campanelli R, Fois G, Catarsi P, Poletto V, Villani L, Erba BG, Maddaluno L, Jemos B, Salmoiraghi S, Guglielmelli P, Abbonante V, Di Buduo CA, Balduini A, Iurlo A, Barosi G, Rosti V, Massa M |title=Tie2 Expressing Monocytes in the Spleen of Patients with Primary Myelofibrosis |journal=PLoS ONE |volume=11 |issue=6 |pages=e0156990 |date=2016 |pmid=27281335 |pmc=4900622 |doi=10.1371/journal.pone.0156990 |url=}}</ref>
*[[CD14|CD14+ cells]] and [[Monocyte|monocytes]] expressing the angiopoietin-2 receptor (Tie2) lie close to the [[Blood vessel|vessels]] in the [[spleen]] [[tissue]]<ref name="pmid27281335">{{cite journal |vauthors=Campanelli R, Fois G, Catarsi P, Poletto V, Villani L, Erba BG, Maddaluno L, Jemos B, Salmoiraghi S, Guglielmelli P, Abbonante V, Di Buduo CA, Balduini A, Iurlo A, Barosi G, Rosti V, Massa M |title=Tie2 Expressing Monocytes in the Spleen of Patients with Primary Myelofibrosis |journal=PLoS ONE |volume=11 |issue=6 |pages=e0156990 |date=2016 |pmid=27281335 |pmc=4900622 |doi=10.1371/journal.pone.0156990 |url=}}</ref>
*[[Vessels]] show increased [[density]], [[tortuous]] architecture, and increased branching on [[bone marrow]] study.<ref name="pmid10880370">{{cite journal |vauthors=Lundberg LG, Lerner R, Sundelin P, Rogers R, Folkman J, Palmblad J |title=Bone marrow in polycythemia vera, chronic myelocytic leukemia, and myelofibrosis has an increased vascularity |journal=Am. J. Pathol. |volume=157 |issue=1 |pages=15–9 |date=July 2000 |pmid=10880370 |pmc=1850191 |doi=10.1016/S0002-9440(10)64511-7 |url=}}</ref>
*[[Vessels]] with increased [[density]], [[tortuous]] architecture, and increased branching on [[bone marrow]] study<ref name="pmid10880370">{{cite journal |vauthors=Lundberg LG, Lerner R, Sundelin P, Rogers R, Folkman J, Palmblad J |title=Bone marrow in polycythemia vera, chronic myelocytic leukemia, and myelofibrosis has an increased vascularity |journal=Am. J. Pathol. |volume=157 |issue=1 |pages=15–9 |date=July 2000 |pmid=10880370 |pmc=1850191 |doi=10.1016/S0002-9440(10)64511-7 |url=}}</ref>
*Thrombospondins (TSP) overexpression<ref name="pmid23259436">{{cite journal |vauthors=Kreipe H, Büsche G, Bock O, Hussein K |title=Myelofibrosis: molecular and cell biological aspects |journal=Fibrogenesis Tissue Repair |volume=5 |issue=Suppl 1 |pages=S21 |date=2012 |pmid=23259436 |pmc=3368793 |doi=10.1186/1755-1536-5-S1-S21 |url=}}</ref>
*Thrombospondins (TSP) overexpression<ref name="pmid23259436">{{cite journal |vauthors=Kreipe H, Büsche G, Bock O, Hussein K |title=Myelofibrosis: molecular and cell biological aspects |journal=Fibrogenesis Tissue Repair |volume=5 |issue=Suppl 1 |pages=S21 |date=2012 |pmid=23259436 |pmc=3368793 |doi=10.1186/1755-1536-5-S1-S21 |url=}}</ref>
===On Electron Microscopy===
===On Electron Microscopy===
*Micromegakaryocytes with mature [[cytoplasm]] containing [[alpha granules]] and the associated [[Protein|proteins]].<ref name="pmid8435338">{{cite journal |vauthors=Reilly JT, Barnett D, Dolan G, Forrest P, Eastham J, Smith A |title=Characterization of an acute micromegakaryocytic leukaemia: evidence for the pathogenesis of myelofibrosis |journal=Br. J. Haematol. |volume=83 |issue=1 |pages=58–62 |date=January 1993 |pmid=8435338 |doi= |url=}}</ref>
*Micromegakaryocytes with mature [[cytoplasm]] containing [[alpha granules]] and the associated [[Protein|proteins]]<ref name="pmid8435338">{{cite journal |vauthors=Reilly JT, Barnett D, Dolan G, Forrest P, Eastham J, Smith A |title=Characterization of an acute micromegakaryocytic leukaemia: evidence for the pathogenesis of myelofibrosis |journal=Br. J. Haematol. |volume=83 |issue=1 |pages=58–62 |date=January 1993 |pmid=8435338 |doi= |url=}}</ref>
*[[Megakaryocyte|Megakaryocytes]] in the [[peripheral blood]] with leukoerythroblastosis studied on the vertically cut section of the buffy coat of [[blood]]<ref name="pmid2621794">{{cite journal |vauthors=Shimizu S, Onodera Y, Nakamura Y, Ide K, Ayabe T, Isobe H, Nagoshi H, Someya K |title=[Megakaryocyte proportion versus nucleated cells in the peripheral blood showing leukoerythroblastosis] |language=Japanese |journal=Rinsho Ketsueki |volume=30 |issue=12 |pages=2141–7 |date=December 1989 |pmid=2621794 |doi= |url=}}</ref>
*[[Megakaryocyte|Megakaryocytes]] in the [[peripheral blood]] with leukoerythroblastosis<ref name="pmid2621794">{{cite journal |vauthors=Shimizu S, Onodera Y, Nakamura Y, Ide K, Ayabe T, Isobe H, Nagoshi H, Someya K |title=[Megakaryocyte proportion versus nucleated cells in the peripheral blood showing leukoerythroblastosis] |language=Japanese |journal=Rinsho Ketsueki |volume=30 |issue=12 |pages=2141–7 |date=December 1989 |pmid=2621794 |doi= |url=}}</ref>
*Significant atypicalities of the [[Neutrophil|neutrophilic]], [[Basophil|basophilic]] and [[Megakaryocyte|megakaryocytic]] [[cell]] lines such as:
*Significant atypicalities of the [[Neutrophil|neutrophilic]], [[Basophil|basophilic]], and [[Megakaryocyte|megakaryocytic]] [[cell]] lines such as:
:*[[Nuclear]]-[[cytoplasmic]] asynchrony<ref name="pmid6935866">{{cite journal |vauthors=Thiele J, Vykoupil KF, Georgii A |title=Ultrastructure of blastic crisis in osteomyelofibrosis. A report of 2 cases with some unusual features |journal=Virchows Arch A Pathol Anat Histol |volume=389 |issue=3 |pages=287–305 |date=1980 |pmid=6935866 |doi= |url=}}</ref>
:*[[Nuclear]]-[[cytoplasmic]] asynchrony<ref name="pmid6935866">{{cite journal |vauthors=Thiele J, Vykoupil KF, Georgii A |title=Ultrastructure of blastic crisis in osteomyelofibrosis. A report of 2 cases with some unusual features |journal=Virchows Arch A Pathol Anat Histol |volume=389 |issue=3 |pages=287–305 |date=1980 |pmid=6935866 |doi= |url=}}</ref>
:*Partial arrest of [[maturation]]
:*Partial arrest of [[maturation]]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]

Overview

Myelofibrosis, a myeloproliferative disorder, is characterized by the proliferation of megakaryocytes in the bone marrow, disrupted cytokine production, and reactive fibrosis resulting in bone marrow failure. The fibrosed and scarred bone marrow produces fewer and fewer normal functioning blood cells leading to pancytopenia and extramedullary hematopoiesis (EMH). It can mainly be associated with somatic mutation of the myeloproliferative leukemia virus (MPL) oncogene, the calreticulin (CALR) gene, or Janus kinase 2 (JAK2) gene but other genes can also be involved and it can also result in the setting of another primary insult.

Pathogenesis

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Microscopic Pathology

On Light Microscopy

On Confocal Microscopy

On Electron Microscopy

  • Ultrastructural abnormalities in platelets such as:
  • Splenic findings can consist of the following:

References

  1. ↑ 1.0 1.1 Bedekovics J, Méhes G (March 2014). "[Pathomechanism and clinical impact of myelofibrosis in neoplastic diseases of the bone marrow]". Orv Hetil (in Hungarian). 155 (10): 367–75. doi:10.1556/OH.2014.29823. PMID 24583557.
  2. ↑ Vainchenker W, Kralovics R (February 2017). "Genetic basis and molecular pathophysiology of classical myeloproliferative neoplasms". Blood. 129 (6): 667–679. doi:10.1182/blood-2016-10-695940. PMID 28028029.
  3. ↑ 3.0 3.1 3.2 Alshemmari SH, Rajan R, Emadi A (2016). "Molecular Pathogenesis and Clinical Significance of Driver Mutations in Primary Myelofibrosis: A Review". Med Princ Pract. 25 (6): 501–509. doi:10.1159/000450956. PMC 5588514. PMID 27756071.
  4. ↑ de Freitas RM, da Costa Maranduba CM (2015). "Myeloproliferative neoplasms and the JAK/STAT signaling pathway: an overview". Rev Bras Hematol Hemoter. 37 (5): 348–53. doi:10.1016/j.bjhh.2014.10.001. PMC 4685044. PMID 26408371.
  5. ↑ Le Bousse-Kerdilès MC, Martyré MC (October 1999). "Dual implication of fibrogenic cytokines in the pathogenesis of fibrosis and myeloproliferation in myeloid metaplasia with myelofibrosis". Ann. Hematol. 78 (10): 437–44. PMID 10550553.
  6. ↑ Kuter DJ, Bain B, Mufti G, Bagg A, Hasserjian RP (November 2007). "Bone marrow fibrosis: pathophysiology and clinical significance of increased bone marrow stromal fibres". Br. J. Haematol. 139 (3): 351–62. doi:10.1111/j.1365-2141.2007.06807.x. PMID 17910625.
  7. ↑ 7.0 7.1 7.2 Reilly JT, Barnett D, Dolan G, Forrest P, Eastham J, Smith A (January 1993). "Characterization of an acute micromegakaryocytic leukaemia: evidence for the pathogenesis of myelofibrosis". Br. J. Haematol. 83 (1): 58–62. PMID 8435338.
  8. ↑ Schmitt A, Drouin A, Massé JM, Guichard J, Shagraoui H, Cramer EM (April 2002). "Polymorphonuclear neutrophil and megakaryocyte mutual involvement in myelofibrosis pathogenesis". Leuk. Lymphoma. 43 (4): 719–24. doi:10.1080/10428190290016809. PMID 12153156.
  9. ↑ Schmitt A, Jouault H, Guichard J, Wendling F, Drouin A, Cramer EM (August 2000). "Pathologic interaction between megakaryocytes and polymorphonuclear leukocytes in myelofibrosis". Blood. 96 (4): 1342–7. PMID 10942376.
  10. ↑ Zahr AA, Salama ME, Carreau N, Tremblay D, Verstovsek S, Mesa R, Hoffman R, Mascarenhas J (June 2016). "Bone marrow fibrosis in myelofibrosis: pathogenesis, prognosis and targeted strategies". Haematologica. 101 (6): 660–71. doi:10.3324/haematol.2015.141283. PMC 5013940. PMID 27252511.
  11. ↑ Klampfl T, Gisslinger H, Harutyunyan AS, Nivarthi H, Rumi E, Milosevic JD, Them NC, Berg T, Gisslinger B, Pietra D, Chen D, Vladimer GI, Bagienski K, Milanesi C, Casetti IC, Sant'Antonio E, Ferretti V, Elena C, Schischlik F, Cleary C, Six M, Schalling M, Schönegger A, Bock C, Malcovati L, Pascutto C, Superti-Furga G, Cazzola M, Kralovics R (December 2013). "Somatic mutations of calreticulin in myeloproliferative neoplasms". N. Engl. J. Med. 369 (25): 2379–90. doi:10.1056/NEJMoa1311347. PMID 24325356.
  12. ↑ 12.0 12.1 12.2 12.3 12.4 12.5 Mak YK, Chan CH, So CC, Chan MK, Chu YC (February 2002). "Idiopathic myelofibrosis with extramedullary haemopoiesis involving the urinary bladder in a Chinese lady". Clin Lab Haematol. 24 (1): 55–9. PMID 11843900.
  13. ↑ 13.0 13.1 Philipponnet C, Ronco P, Aniort J, Kemeny JL, Heng AE (December 2017). "Membranous Nephropathy and Intrarenal Extramedullary Hematopoiesis in a Patient With Myelofibrosis". Am. J. Kidney Dis. 70 (6): 874–877. doi:10.1053/j.ajkd.2017.06.022. PMID 28821362.
  14. ↑ 14.0 14.1 14.2 Yang M, Roarke M (March 2017). "Diffuse pulmonary extramedullary hematopoiesis in myelofibrosis diagnosed with technetium-99m sulfur colloid bone marrow scintigraphy and single photon emission computerized tomography/CT". Am. J. Hematol. 92 (3): 323–324. doi:10.1002/ajh.24616. PMID 27883206.
  15. ↑ 15.0 15.1 Pizzi M, Gergis U, Chaviano F, Orazi A (September 2016). "The effects of hematopoietic stem cell transplant on splenic extramedullary hematopoiesis in patients with myeloproliferative neoplasm-associated myelofibrosis". Hematol Oncol Stem Cell Ther. 9 (3): 96–104. doi:10.1016/j.hemonc.2016.07.002. PMID 27521149.
  16. ↑ 16.0 16.1 Campanelli R, Fois G, Catarsi P, Poletto V, Villani L, Erba BG, Maddaluno L, Jemos B, Salmoiraghi S, Guglielmelli P, Abbonante V, Di Buduo CA, Balduini A, Iurlo A, Barosi G, Rosti V, Massa M (2016). "Tie2 Expressing Monocytes in the Spleen of Patients with Primary Myelofibrosis". PLoS ONE. 11 (6): e0156990. doi:10.1371/journal.pone.0156990. PMC 4900622. PMID 27281335.
  17. ↑ Mohyuddin GR, Yacoub A (2016). "Primary Myelofibrosis Presenting as Extramedullary Hematopoiesis in a Transplanted Liver Graft: Case Report and Review of the Literature". Case Rep Hematol. 2016: 9515404. doi:10.1155/2016/9515404. PMC 4739215. PMID 26885416.
  18. ↑ Henry M, Chitlur M, Rajpurkar M, Mastropietro CW, Poulik J, Ravindranath Y (May 2014). "Myelofibrosis, hepatic extramedullary hematopoiesis and ascites associated with vitamin D deficiency in early infancy". J. Pediatr. Hematol. Oncol. 36 (4): 319–21. doi:10.1097/MPH.0b013e31828e548a. PMID 23619118.
  19. ↑ 19.0 19.1 Imai K, Aoi T, Kitai H, Endo N, Fujino M, Ichida S (November 2017). "A case of perirenal extramedullary hematopoiesis in a patient with primary myelofibrosis". CEN Case Rep. 6 (2): 194–199. doi:10.1007/s13730-017-0274-1. PMC 5694411. PMID 28895103.
  20. ↑ 20.0 20.1 20.2 Kwak HS, Lee JM (August 2000). "CT findings of extramedullary hematopoiesis in the thorax, liver and kidneys, in a patient with idiopathic myelofibrosis". J. Korean Med. Sci. 15 (4): 460–2. doi:10.3346/jkms.2000.15.4.460. PMC 3054659. PMID 10983698.
  21. ↑ Singer A, Quencer R (2014). "Intracranial extramedullary hematopoiesis: a rare cause of headaches". J Neuroimaging. 24 (5): 524–7. doi:10.1111/jon.12029. PMID 23621819.
  22. ↑ Fareed S, Nashwan AJ, Abu Jarir S, Husain A, Suliman DS, Ibrahim F, Moustafa A, Akhter MS, Yassin MA (August 2017). "Spinal Abscess Caused by Salmonella Bacteremia in a Patient with Primary Myelofibrosis". Am J Case Rep. 18: 859–864. PMC 5551928. PMID 28775247.
  23. ↑ Hijikata Y, Ando T, Inagaki T, Watanabe H, Ito M, Sobue G (2014). "[Spinal cord compression due to extramedullary hematopoiesis in a patient with myelofibrosis]". Rinsho Shinkeigaku (in Japanese). 54 (1): 27–31. PMID 24429645.
  24. ↑ Mizoguchi M, Kawa Y, Minami T, Nakayama H, Mizoguchi H (February 1990). "Cutaneous extramedullary hematopoiesis in myelofibrosis". J. Am. Acad. Dermatol. 22 (2 Pt 2): 351–5. PMID 2406300.
  25. ↑ Patel KP, Newberry KJ, Luthra R, Jabbour E, Pierce S, Cortes J, Singh R, Mehrotra M, Routbort MJ, Luthra M, Manshouri T, Santos FP, Kantarjian H, Verstovsek S (August 2015). "Correlation of mutation profile and response in patients with myelofibrosis treated with ruxolitinib". Blood. 126 (6): 790–7. doi:10.1182/blood-2015-03-633404. PMC 4528066. PMID 26124496.
  26. ↑ Tefferi, A; Lasho, T L; Finke, C M; Knudson, R A; Ketterling, R; Hanson, C H; Maffioli, M; Caramazza, D; Passamonti, F; Pardanani, A (2014). "CALR vs JAK2 vs MPL-mutated or triple-negative myelofibrosis: clinical, cytogenetic and molecular comparisons". Leukemia. 28 (7): 1472–1477. doi:10.1038/leu.2014.3. ISSN 0887-6924.
  27. ↑ Baxter EJ, Scott LM, Campbell PJ; et al. (2005). "Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders". Lancet. 365 (9464): 1054–61. doi:10.1016/S0140-6736(05)71142-9. PMID 15781101.
  28. ↑ Pikman Y, Lee BH, Mercher T; et al. (2006). "MPLW515L is a novel somatic activating mutation in myelofibrosis with myeloid metaplasia". PLoS Med. 3 (7): e270. doi:10.1371/journal.pmed.0030270. PMC 1502153. PMID 16834459. Unknown parameter |month= ignored (help)
  29. ↑ Shammo JM, Stein BL (December 2016). "Mutations in MPNs: prognostic implications, window to biology, and impact on treatment decisions". Hematology Am Soc Hematol Educ Program. 2016 (1): 552–560. doi:10.1182/asheducation-2016.1.552. PMC 6142495. PMID 27913528.
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  31. ↑ Rotunno G, Pacilli A, Artusi V, Rumi E, Maffioli M, Delaini F, Brogi G, Fanelli T, Pancrazzi A, Pietra D, Bernardis I, Belotti C, Pieri L, Sant'Antonio E, Salmoiraghi S, Cilloni D, Rambaldi A, Passamonti F, Barbui T, Manfredini R, Cazzola M, Tagliafico E, Vannucchi AM, Guglielmelli P (July 2016). "Epidemiology and clinical relevance of mutations in postpolycythemia vera and postessential thrombocythemia myelofibrosis: A study on 359 patients of the AGIMM group". Am. J. Hematol. 91 (7): 681–6. doi:10.1002/ajh.24377. PMID 27037840.
  32. ↑ Song J, Hussaini M, Zhang H, Shao H, Qin D, Zhang X, Ma Z, Hussnain Naqvi SM, Zhang L, Moscinski LC (May 2017). "Comparison of the Mutational Profiles of Primary Myelofibrosis, Polycythemia Vera, and Essential Thrombocytosis". Am. J. Clin. Pathol. 147 (5): 444–452. doi:10.1093/ajcp/aqw222. PMC 5402718. PMID 28419183.
  33. ↑ 33.0 33.1 Tefferi A (December 2016). "Primary myelofibrosis: 2017 update on diagnosis, risk-stratification, and management". Am. J. Hematol. 91 (12): 1262–1271. doi:10.1002/ajh.24592. PMID 27870387.
  34. ↑ Vannucchi AM, Lasho TL, Guglielmelli P, Biamonte F, Pardanani A, Pereira A, Finke C, Score J, Gangat N, Mannarelli C, Ketterling RP, Rotunno G, Knudson RA, Susini MC, Laborde RR, Spolverini A, Pancrazzi A, Pieri L, Manfredini R, Tagliafico E, Zini R, Jones A, Zoi K, Reiter A, Duncombe A, Pietra D, Rumi E, Cervantes F, Barosi G, Cazzola M, Cross NC, Tefferi A (September 2013). "Mutations and prognosis in primary myelofibrosis". Leukemia. 27 (9): 1861–9. doi:10.1038/leu.2013.119. PMID 23619563.
  35. ↑ Tefferi A, Pardanani A (April 2015). "Myeloproliferative Neoplasms: A Contemporary Review". JAMA Oncol. 1 (1): 97–105. doi:10.1001/jamaoncol.2015.89. PMID 26182311.
  36. ↑ Boiocchi L, Mathew S, Gianelli U, Iurlo A, Radice T, Barouk-Fox S, Knowles DM, Orazi A (December 2013). "Morphologic and cytogenetic differences between post-polycythemic myelofibrosis and primary myelofibrosis in fibrotic stage". Mod. Pathol. 26 (12): 1577–85. doi:10.1038/modpathol.2013.109. PMID 23787440.
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  38. ↑ Sakatoku K, Takeoka Y, Araki T, Miura A, Fujitani Y, Yamamura R, Miyagi Y, Senzaki H, Ohta K (2017). "Lymphocyte-depleted classical Hodgkin lymphoma accompanied by myelofibrosis". Rinsho Ketsueki (in Japanese). 58 (7): 772–775. doi:10.11406/rinketsu.58.772. PMID 28781273.
  39. ↑ Fu R, Yu H, Wu YH, Liu H, Shao ZH (September 2015). "Hodgkin's lymphoma associated with myelofibrosis: A case report". Oncol Lett. 10 (3): 1551–1554. doi:10.3892/ol.2015.3438. PMC 4533276. PMID 26622707.
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  54. ↑ Cansu DÜ, Teke HÜ, Korkmaz C (March 2017). "A rare cause of cytopenia in a patient with systemic lupus erythematosus: Autoimmune myelofibrosis". Eur J Rheumatol. 4 (1): 76–78. doi:10.5152/eurjrheum.2016.011. PMC 5335895. PMID 28293461.
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  57. ↑ el Mouzan MI, Ahmad MA, al Fadel Saleh M, al Sohaibani MO, al Gindan YM (1988). "Myelofibrosis and pancytopenia in systemic lupus erythematosus". Acta Haematol. 80 (4): 219–21. doi:10.1159/000205641. PMID 3146200.
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  59. ↑ Mouly S, Youssefian T, Souni F, Cramer E, Lefrere F, Varet B, Hermine O (May 2000). "Acquired delta-storage pool deficiency associated with idiopathic myelofibrosis". Leuk. Lymphoma. 37 (5–6): 623–7. doi:10.3109/10428190009058516. PMID 11042524.
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  87. ↑ Wassie E, Finke C, Gangat N, Lasho TL, Pardanani A, Hanson CA, Ketterling RP, Tefferi A (April 2015). "A compendium of cytogenetic abnormalities in myelofibrosis: molecular and phenotypic correlates in 826 patients". Br. J. Haematol. 169 (1): 71–6. doi:10.1111/bjh.13260. PMID 25521305.
  88. ↑ Guglielmelli P, Rotunno G, Pacilli A, Rumi E, Rosti V, Delaini F, Maffioli M, Fanelli T, Pancrazzi A, Pieri L, Fjerza R, Pietra D, Cilloni D, Sant'Antonio E, Salmoiraghi S, Passamonti F, Rambaldi A, Barosi G, Barbui T, Cazzola M, Vannucchi AM (September 2016). "Prognostic impact of bone marrow fibrosis in primary myelofibrosis. A study of the AGIMM group on 490 patients". Am. J. Hematol. 91 (9): 918–22. doi:10.1002/ajh.24442. PMID 27264006.
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