Myelofibrosis natural history, complications and prognosis: Difference between revisions

Jump to navigation Jump to search
VisualWikitext
 
(52 intermediate revisions by 5 users not shown)
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{Myelofibrosis}}
{{Myelofibrosis}}
{{CMG}}{{AE}}{{SR}}
{{CMG}}{{AE}}{{Sab}}


==Overview==
==Overview==
==Natural History==
The development of [[myelofibrosis]] is a a slow process and it does not cause early [[Symptom|symptoms]]. A significant proportion of the [[Patient|patients]] can be [[asymptomatic]] and the [[diagnosis]] is usually made in the setting of an unrelated condition. The most overlapping and common findings encountered are [[anemia]] and [[splenomegaly]] presenting as [[weakness]], easy [[fatigability]], [[Palpitation|palpitations]], and [[dyspnea]] in the case of [[anemia]] and [[early satiety]] with possible accompanying left upper quadrant [[discomfort]] if [[splenomegaly]] is present. The [[disease]] has a progressive course and can result in [[pancytopenia]] as the [[bone marrow failure]] ensues. This can result in [[bleeding]] complications, [[easy bruising]], increase in the susceptibility to [[Infection|infections]], and worsening [[anemia]]. The [[bone marrow failure]] paves the way for [[Extramedullary hematopoiesis|extramedullary hematopoiesis (EMH)]] which mainly occurs in the [[Reticuloendothelial system|reticuloendothelial tissues]]. If left untreated, [[myelofibrosis]] can lead to severe [[complications]], the most feared of which are [[acute leukemia]], [[heart failure]], and [[portal hypertension]].
Myelofibrosis has a very indolent course.<ref name=wikidiagn1osismf1>Diagnosis of myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 8, 2016</ref>


==Complications==
==Natural History, Complications, and Prognosis==
Common complications of myelofibrosis include:<ref name=complicatnmf1>Complications of myelofibrosis. US National Library of Medicine 2016. https://www.nlm.nih.gov/medlineplus/ency/article/000531.htm. Accessed on March 7, 2016</ref><ref name=signsandsymptomsofmyelofibrosiswiki1>Signs and symptoms of myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 7, 2016</ref><ref name="KelleYıldız2015">{{cite journal|last1=Kelle|first1=Bayram|last2=Yıldız|first2=Fatih|last3=Paydas|first3=Semra|last4=Bagır|first4=Emine Kılıc|last5=Ergin|first5=Melek|last6=Kozanoglu|first6=Erkan|title=Coexistence of hypertrophic osteoarthropathy and myelofibrosis|journal=Revista Brasileira de Reumatologia (English Edition)|year=2015|issn=22555021|doi=10.1016/j.rbre.2014.11.004}}</ref>
*[[Acute myelogenous leukemia]]
*[[Infections]]
*[[Bleeding]]
*[[Blood clots|Thrombohemorrhagic events]]
*[[Hepatic failure]]
*[[Heart failure]]
*[[Gout]]
*[[Bone marrow failure|Progressive marrow failure]]
*[[Hypertrophic osteoarthropathy]]


==Prognosis==
=== Natural History ===
*Prognosis of myelofibrosis is poor, with slow progression and death usually within 2-3 years.<ref name=rxandprogofmyelofibrosisradiopaedia1>Treatment and prognosis of myelofibrosis. Dr Henry Knipe and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/myelofibrosis. Accessed on March 8, 2016</ref>
 
*The median survival for myelofibrosis is 3.5 years to 5.5 years, but patients younger than 55 years have a median survival of 11 years.<ref name=diseaseoverviewmf1>Disease overview of primary myelofibrosis. National cancer institute 2016. http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_9. Accessed on March 10, 2016</ref>
*[[Myelofibrosis]] is a [[chronic]], [[malignant]] [[hematologic]] disorder which can have a slow progressive course.<ref>{{cite book | last = Hoffman | first = Ronald | title = Hematology : basic principles and practice | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 9780323357623 }}</ref>
*Along the course of the [[disease]], [[myelofibrosis]] most commonly presents with symptoms related to [[hypermetabolic]] state, [[anemia]], and [[splenomegaly]].<ref name="pmid9488952">{{cite journal |vauthors=Cervantes F, Pereira A, Esteve J, Cobo F, Rozman C, Montserrat E |title=[Idiopathic myelofibrosis: initial features, evolutive patterns and survival in a series of 106 patients] |language=Spanish; Castilian |journal=Med Clin (Barc) |volume=109 |issue=17 |pages=651–5 |date=November 1997 |pmid=9488952 |doi= |url=}}</ref>
*Progression of the [[disease]] can vary from [[patient]] to [[patient]] and a significant proportion of [[Patient|patients]] can be [[asymptomatic]].<ref name="pmid29741513">{{cite journal |vauthors=O'Sullivan JM, Harrison CN |title=Myelofibrosis: clinicopathologic features, prognosis, and management |journal=Clin Adv Hematol Oncol |volume=16 |issue=2 |pages=121–131 |date=February 2018 |pmid=29741513 |doi= |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="pmid9488952">{{cite journal |vauthors=Cervantes F, Pereira A, Esteve J, Cobo F, Rozman C, Montserrat E |title=[Idiopathic myelofibrosis: initial features, evolutive patterns and survival in a series of 106 patients] |language=Spanish; Castilian |journal=Med Clin (Barc) |volume=109 |issue=17 |pages=651–5 |date=November 1997 |pmid=9488952 |doi= |url=}}</ref>
*The [[disease]] is characterized by irregularity in the [[Blood cell|blood cells]] as a result of [[Bone marrow|marrow]] [[fibrosis]] and the clinical course correlates with this accordingly.<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><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 manifest as [[anemia]] if the [[pathology]] involves the [[Red blood cells|red blood cells (RBCs)]] as the initiating event and it can present as [[shortness of breath]], [[fatigue]], [[lightheadedness]], [[weakness]], [[Headache|headaches]], [[irritability]], and [[pale skin color]].<ref name="pmid30472746">{{cite journal |vauthors=Birgegard G, Samuelsson J, Ahlstrand E, Ejerblad E, Enevold C, Ghanima W, Hasselbalch H, Nielsen CH, Knutsen H, Pedersen OB, Sørensen A, Andreasson B |title=Inflammatory functional iron deficiency common in myelofibrosis, contributes to anaemia and impairs quality of life. From the Nordic MPN study Group |journal=Eur. J. Haematol. |volume= |issue= |pages= |date=November 2018 |pmid=30472746 |doi=10.1111/ejh.13198 |url=}}</ref><ref name="pmid30100963">{{cite journal |vauthors=Chahdi H, Oukabli M |title=[A special form of pancytopenia] |language=French |journal=Pan Afr Med J |volume=29 |issue= |pages=209 |date=2018 |pmid=30100963 |pmc=6080970 |doi=10.11604/pamj.2018.29.209.14055 |url=}}</ref><ref name="pmid30039550">{{cite journal |vauthors=Tefferi A |title=Primary myelofibrosis: 2019 update on diagnosis, risk-stratification and management |journal=Am. J. Hematol. |volume=93 |issue=12 |pages=1551–1560 |date=December 2018 |pmid=30039550 |doi=10.1002/ajh.25230 |url=}}</ref>
*[[Patient|Patients]] can present with increased susceptibility to [[Infection|infections]] which can be the presenting or an additional [[symptom]]. These [[Infection|infections]] can be [[Virus|viral]], [[Bacteria|bacterial]], or [[Fungus|fungal]] in origin.<ref name="pmid30057341">{{cite journal |vauthors=Karigane D, Kikuchi T, Sakurai M, Kato J, Yamane Y, Hashida R, Abe R, Hatano M, Hasegawa N, Wakayama M, Shibuya K, Okamoto S, Mori T |title=Invasive hepatic mucormycosis: A case report and review of the literature |journal=J. Infect. Chemother. |volume= |issue= |pages= |date=July 2018 |pmid=30057341 |doi=10.1016/j.jiac.2018.06.013 |url=}}</ref>
*The disrupted [[platelet]] production results in [[bleeding]] [[complications]] such as [[easy bruising]] following minimal [[injury]] and [[bleeding]] from the [[Mucous membrane|mucous membranes]].<ref name="pmid30405096">{{cite journal |vauthors=Finazzi G, Vannucchi AM, Barbui T |title=Prefibrotic myelofibrosis: treatment algorithm 2018 |journal=Blood Cancer J |volume=8 |issue=11 |pages=104 |date=November 2018 |pmid=30405096 |pmc=6221891 |doi=10.1038/s41408-018-0142-z |url=}}</ref><ref name="pmid29898956">{{cite journal |vauthors=Hofmann I, Geer MJ, Vögtle T, Crispin A, Campagna DR, Barr A, Calicchio ML, Heising S, van Geffen JP, Kuijpers MJE, Heemskerk JWM, Eble JA, Schmitz-Abe K, Obeng EA, Douglas M, Freson K, Pondarré C, Favier R, Jarvis GE, Markianos K, Turro E, Ouwehand WH, Mazharian A, Fleming MD, Senis YA |title=Congenital macrothrombocytopenia with focal myelofibrosis due to mutations in human G6b-B is rescued in humanized mice |journal=Blood |volume=132 |issue=13 |pages=1399–1412 |date=September 2018 |pmid=29898956 |doi=10.1182/blood-2017-08-802769 |url=}}</ref>
*As a compensation for the lack of efficient [[blood cell]] production in the [[bone marrow]], [[Extramedullary hematopoiesis|extramedullary hematopoiesis (EMH)]] can ensue which will present as [[splenomegaly]], [[hepatomegaly]], [[lymph node]] enlargement, and [[skin]], [[kidney]], or [[lung]] [[pathology]].<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><ref name="pmid26885416">{{cite journal |vauthors=Mohyuddin GR, Yacoub A |title=Primary Myelofibrosis Presenting as Extramedullary Hematopoiesis in a Transplanted Liver Graft: Case Report and Review of the Literature |journal=Case Rep Hematol |volume=2016 |issue= |pages=9515404 |date=2016 |pmid=26885416 |pmc=4739215 |doi=10.1155/2016/9515404 |url=}}</ref><ref name="pmid23619118">{{cite journal |vauthors=Henry M, Chitlur M, Rajpurkar M, Mastropietro CW, Poulik J, Ravindranath Y |title=Myelofibrosis, hepatic extramedullary hematopoiesis and ascites associated with vitamin D deficiency in early infancy |journal=J. Pediatr. Hematol. Oncol. |volume=36 |issue=4 |pages=319–21 |date=May 2014 |pmid=23619118 |doi=10.1097/MPH.0b013e31828e548a |url=}}</ref><ref name="pmid28895103">{{cite journal |vauthors=Imai K, Aoi T, Kitai H, Endo N, Fujino M, Ichida S |title=A case of perirenal extramedullary hematopoiesis in a patient with primary myelofibrosis |journal=CEN Case Rep |volume=6 |issue=2 |pages=194–199 |date=November 2017 |pmid=28895103 |pmc=5694411 |doi=10.1007/s13730-017-0274-1 |url=}}</ref><ref name="pmid10983698">{{cite journal |vauthors=Kwak HS, Lee JM |title=CT findings of extramedullary hematopoiesis in the thorax, liver and kidneys, in a patient with idiopathic myelofibrosis |journal=J. Korean Med. Sci. |volume=15 |issue=4 |pages=460–2 |date=August 2000 |pmid=10983698 |pmc=3054659 |doi=10.3346/jkms.2000.15.4.460 |url=}}</ref><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="pmid2406300">{{cite journal |vauthors=Mizoguchi M, Kawa Y, Minami T, Nakayama H, Mizoguchi H |title=Cutaneous extramedullary hematopoiesis in myelofibrosis |journal=J. Am. Acad. Dermatol. |volume=22 |issue=2 Pt 2 |pages=351–5 |date=February 1990 |pmid=2406300 |doi= |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>
*[[Bone]] or [[joint pain]] can be a late presentation of [[myelofibrosis]].<ref name="pmid28544906">{{cite journal |vauthors=Gwaltney C, Paty J, Kwitkowski VE, Mesa RA, Dueck AC, Papadopoulos EJ, Wang L, Feliciano J, Coons SJ |title=Development of a harmonized patient-reported outcome questionnaire to assess myelofibrosis symptoms in clinical trials |journal=Leuk. Res. |volume=59 |issue= |pages=26–31 |date=August 2017 |pmid=28544906 |doi=10.1016/j.leukres.2017.05.012 |url=}}</ref>
*The progression of [[myelofibrosis]] and its respective presentation can further be aligned with the complications encountered as a result of the disease itself.
 
===Complications===
Common complications of [[myelofibrosis]] include:
 
====== '''Hematologic''' ======
 
*[[Bleeding]]<ref name="pmid28808761">{{cite journal |vauthors=Kc D, Falchi L, Verstovsek S |title=The underappreciated risk of thrombosis and bleeding in patients with myelofibrosis: a review |journal=Ann. Hematol. |volume=96 |issue=10 |pages=1595–1604 |date=October 2017 |pmid=28808761 |pmc=5693670 |doi=10.1007/s00277-017-3099-2 |url=}}</ref><ref name="complmf1radio1">Complications of primary myelofibrosis. Dr Henry Knipe and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/myelofibrosis. Accessed on March 10, 2016</ref>
*[[Blood clots|Thrombohemorrhagic events]]<ref name="pmid29946154">{{cite journal |vauthors=Finazzi G, De Stefano V, Barbui T |title=Splanchnic vein thrombosis in myeloproliferative neoplasms: treatment algorithm 2018 |journal=Blood Cancer J |volume=8 |issue=7 |pages=64 |date=June 2018 |pmid=29946154 |pmc=6018786 |doi=10.1038/s41408-018-0100-9 |url=}}</ref><ref name="pmid29368941">{{cite journal |vauthors=Sández Montagut VM, Giráldez Gallego Á, Ontanilla Clavijo G |title=Regenerative nodular hyperplasia, portal vein thrombosis and primary myelofibrosis: an unusual triple association |journal=Rev Esp Enferm Dig |volume=110 |issue=3 |pages=209–210 |date=March 2018 |pmid=29368941 |doi=10.17235/reed.2018.5349/2017 |url=}}</ref><ref name="pmid29048104">{{cite journal |vauthors=How J, Trinkaus KM, Oh ST |title=Distinct clinical, laboratory and molecular features of myeloproliferative neoplasm patients with splanchnic vein thrombosis |journal=Br. J. Haematol. |volume=183 |issue=2 |pages=310–313 |date=October 2018 |pmid=29048104 |doi=10.1111/bjh.14958 |url=}}</ref><ref name="pmid28991281">{{cite journal |vauthors=Campos-Cabrera G, Campos-Cabrera V, Campos-Cabrera S, Campos-Villagómez JL, Romero-González A |title=Splanchnic vein thrombosis as a first manifestation of Primary myelofibrosis |language=Spanish; Castilian |journal=Gac Med Mex |volume=153 |issue=4 |pages=537–540 |date=2017 |pmid=28991281 |doi=10.24875/GMM.17002822 |url=}}</ref><ref name="pmid28808761">{{cite journal |vauthors=Kc D, Falchi L, Verstovsek S |title=The underappreciated risk of thrombosis and bleeding in patients with myelofibrosis: a review |journal=Ann. Hematol. |volume=96 |issue=10 |pages=1595–1604 |date=October 2017 |pmid=28808761 |pmc=5693670 |doi=10.1007/s00277-017-3099-2 |url=}}</ref>
*[[Bone marrow failure|Progressive marrow failure]]<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>
 
====== '''Gastroentistinal''' ======
 
*[[Hepatic failure]]<ref name="pmid7996922">{{cite journal |vauthors=Escorsell A, Montero A |title=[Severe acute liver insufficiency in a 72 year old male with idiopathic myelofibrosis] |language=Spanish; Castilian |journal=Med Clin (Barc) |volume=103 |issue=16 |pages=628–35 |date=November 1994 |pmid=7996922 |doi= |url=}}</ref><ref name="pmid7446477">{{cite journal |vauthors=McBrine PA, Miller A, Zimelman AP, Koff RS |title=Polycythemia vera with myelofibrosis and myeloid metaplasia. Acute hepatic failure following splenectomy |journal=Am. J. Clin. Pathol. |volume=74 |issue=5 |pages=693–6 |date=November 1980 |pmid=7446477 |doi= |url=}}</ref>
*[[Splenic rupture]]<ref name="pmid22096219">{{cite journal |vauthors=Saboo SS, Krajewski KM, O'Regan KN, Giardino A, Brown JR, Ramaiya N, Jagannathan JP |title=Spleen in haematological malignancies: spectrum of imaging findings |journal=Br J Radiol |volume=85 |issue=1009 |pages=81–92 |date=January 2012 |pmid=22096219 |pmc=3473934 |doi=10.1259/bjr/31542964 |url=}}</ref><ref name="pmid2395215">{{cite journal |vauthors=Baba M, Tanahashi N, Seno K, Nara M, Shinbo T |title=[Myelofibrosis with marked subcapsular bleeding of the spleen--a case report] |language=Japanese |journal=Rinsho Ketsueki |volume=31 |issue=5 |pages=669–73 |date=May 1990 |pmid=2395215 |doi= |url=}}</ref>
*[[Portal hypertension]]<ref name="pmid22851873">{{cite journal |vauthors=Tokai K, Miyatani H, Yoshida Y, Yamada S |title=Multiple esophageal variceal ruptures with massive ascites due to myelofibrosis-induced portal hypertension |journal=World J. Gastroenterol. |volume=18 |issue=28 |pages=3770–4 |date=July 2012 |pmid=22851873 |pmc=3406433 |doi=10.3748/wjg.v18.i28.3770 |url=}}</ref><ref name="pmid11293952">{{cite journal |vauthors=Bĕlohlávek J, Schwarz J, Jirásek A, Krajina A, Polák F, Hrubý M |title=Idiopathic myelofibrosis complicated by portal hypertension treated with a transjugular intrahepatic portosystemic shunt (TIPS) |journal=Wien. Klin. Wochenschr. |volume=113 |issue=5-6 |pages=208–11 |date=March 2001 |pmid=11293952 |doi= |url=}}</ref><ref name="pmid24501543">{{cite journal |vauthors=Mughal TI, Vaddi K, Sarlis NJ, Verstovsek S |title=Myelofibrosis-associated complications: pathogenesis, clinical manifestations, and effects on outcomes |journal=Int J Gen Med |volume=7 |issue= |pages=89–101 |date=2014 |pmid=24501543 |pmc=3912063 |doi=10.2147/IJGM.S51800 |url=}}</ref><ref name="pmid17301514">{{cite journal |vauthors=Doki N, Irisawa H, Takada S, Sakura T, Miyawaki S |title=Transjugular intrahepatic portosystemic shunt for the treatment of portal hypertension due to idiopathic myelofibrosis |journal=Intern. Med. |volume=46 |issue=4 |pages=187–90 |date=2007 |pmid=17301514 |doi= |url=}}</ref>
*[[Peritonitis]]<ref name="pmid21134837">{{cite journal |vauthors=Srinivasaiah N, Zia MK, Muralikrishnan V |title=Peritonitis in myelofibrosis: a cautionary tale |journal=HBPD INT |volume=9 |issue=6 |pages=651–3 |date=December 2010 |pmid=21134837 |doi= |url=}}</ref>
*[[Intestinal obstruction]]<ref name="pmid25206301">{{cite journal |vauthors=Wei XQ, Zheng ZH, Jin Y, Tao J, Abassa KK, Wen ZF, Shao CK, Wei HB, Wu B |title=Intestinal obstruction caused by extramedullary hematopoiesis and ascites in primary myelofibrosis |journal=World J. Gastroenterol. |volume=20 |issue=33 |pages=11921–6 |date=September 2014 |pmid=25206301 |pmc=4155387 |doi=10.3748/wjg.v20.i33.11921 |url=}}</ref>
 
====== '''Urological''' ======
 
*[[Renal amyloidosis]]<ref name="pmid30227762">{{cite journal |vauthors=Babushok DV, Nelson EJ, Morrissette JJD, Joshi S, Palmer MB, Frank D, Cambor CL, Hexner EO |title=Myelofibrosis patients can develop extramedullary complications including renal amyloidosis and sclerosing hematopoietic tumor while otherwise meeting traditional measures of ruxolitinib response |journal=Leuk. Lymphoma |volume= |issue= |pages=1–4 |date=September 2018 |pmid=30227762 |doi=10.1080/10428194.2018.1509319 |url=}}</ref>
*[[Focal segmental glomerulosclerosis|Focal segmental glomerulosclerosis (FSGS)]]<ref name="pmid20199191">{{cite journal |vauthors=Kaygusuz I, Koc M, Arikan H, Adiguzel C, Cakalagaoglu F, Tuglular TF, Akoglu E |title=Focal segmental glomerulosclerosis associated with idiopathic myelofibrosis |journal=Ren Fail |volume=32 |issue=2 |pages=273–6 |date=January 2010 |pmid=20199191 |doi=10.3109/08860220903573286 |url=}}</ref><ref name="pmid29728434">{{cite journal |vauthors=Bohra GK, Meena DS, Bajpai N, Purohit A |title=Focal segmental glomerulosclerosis in a patient with prefibrotic primary myelofibrosis |journal=BMJ Case Rep |volume=2018 |issue= |pages= |date=May 2018 |pmid=29728434 |doi=10.1136/bcr-2017-223803 |url=}}</ref><ref name="pmid26232031">{{cite journal |vauthors=Rajasekaran A, Ngo TT, Abdelrahim M, Glass W, Podoll A, Verstovsek S, Abudayyeh A |title=Primary myelofibrosis associated glomerulopathy: significant improvement after therapy with ruxolitinib |journal=BMC Nephrol |volume=16 |issue= |pages=121 |date=August 2015 |pmid=26232031 |pmc=4521341 |doi=10.1186/s12882-015-0121-6 |url=}}</ref>
*[[Membranous nephropathy]]<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="pmid26232031">{{cite journal |vauthors=Rajasekaran A, Ngo TT, Abdelrahim M, Glass W, Podoll A, Verstovsek S, Abudayyeh A |title=Primary myelofibrosis associated glomerulopathy: significant improvement after therapy with ruxolitinib |journal=BMC Nephrol |volume=16 |issue= |pages=121 |date=August 2015 |pmid=26232031 |pmc=4521341 |doi=10.1186/s12882-015-0121-6 |url=}}</ref>
*[[Chronic kidney disease|Chronic kidney disease (CKD)]]<ref name="pmid24630365">{{cite journal |vauthors=Christensen AS, Møller JB, Hasselbalch HC |title=Chronic kidney disease in patients with the Philadelphia-negative chronic myeloproliferative neoplasms |journal=Leuk. Res. |volume=38 |issue=4 |pages=490–5 |date=April 2014 |pmid=24630365 |doi=10.1016/j.leukres.2014.01.014 |url=}}</ref>
*Bilateral [[ureteral obstruction]]<ref name="pmid20949707">{{cite journal |vauthors=Cvetković ZP, Cvetković BR, Celeketić D, Milenković D, Perunicić-Peković G |title=Bilateral ureteral obstruction due to primary myelofibrosis caused hyperuricaemia |journal=Acta Chir Iugosl |volume=57 |issue=2 |pages=79–83 |date=2010 |pmid=20949707 |doi= |url=}}</ref>
*[[Kidney|Renal]] [[Nephron|tubular]] damage<ref name="pmid25745963">{{cite journal |vauthors=Shimono J, Tsutsumi Y, Ohigashi H |title=[Acute renal tubular damage caused by disseminated Trichosporon infection in primary myelofibrosis] |language=Japanese |journal=Rinsho Ketsueki |volume=56 |issue=1 |pages=21–4 |date=January 2015 |pmid=25745963 |doi=10.11406/rinketsu.56.21 |url=}}</ref>
 
====== '''Cardiopulmonary''' ======
 
*[[Heart failure]]<ref name="pmid18160817">{{cite journal |vauthors=Guilpain P, Montani D, Damaj G, Achouh L, Lefrère F, Le Pavec J, Marfaing-Koka A, Dartevelle P, Simonneau G, Humbert M, Hermine O |title=Pulmonary hypertension associated with myeloproliferative disorders: a retrospective study of ten cases |journal=Respiration |volume=76 |issue=3 |pages=295–302 |date=2008 |pmid=18160817 |doi=10.1159/000112822 |url=}}</ref><ref name="pmid9929105">{{cite journal |vauthors=García-Manero G, Schuster SJ, Patrick H, Martinez J |title=Pulmonary hypertension in patients with myelofibrosis secondary to myeloproliferative diseases |journal=Am. J. Hematol. |volume=60 |issue=2 |pages=130–5 |date=February 1999 |pmid=9929105 |doi= |url=}}</ref>
*[[Pulmonary hypertension]]<ref name="pmid28680586">{{cite journal |vauthors=Singh I, Mikita G, Green D, Risquez C, Sanders A |title=Pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series |journal=Pulm Circ |volume=7 |issue=1 |pages=261–267 |date=March 2017 |pmid=28680586 |pmc=5448544 |doi=10.1177/2045893217702064 |url=}}</ref><ref name="pmid28090305">{{cite journal |vauthors=Faiz SA, Iliescu C, Lopez-Mattei J, Patel B, Bashoura L, Popat U |title=Resolution of myelofibrosis-associated pulmonary arterial hypertension following allogeneic hematopoietic stem cell transplantation |journal=Pulm Circ |volume=6 |issue=4 |pages=611–613 |date=December 2016 |pmid=28090305 |pmc=5210054 |doi=10.1086/687291 |url=}}</ref><ref name="pmid28070238">{{cite journal |vauthors=Mathew R, Huang J, Wu JM, Fallon JT, Gewitz MH |title=Hematological disorders and pulmonary hypertension |journal=World J Cardiol |volume=8 |issue=12 |pages=703–718 |date=December 2016 |pmid=28070238 |pmc=5183970 |doi=10.4330/wjc.v8.i12.703 |url=}}</ref>
*[[Alveolar proteinosis]]<ref name="pmid12854908">{{cite journal |vauthors=Tsutsumi Y, Tanaka J, Saito S, Tanaka Y, Kawamura T, Obara S, Noto S, Shimoyama N, Asaka M, Imamura M, Masauzi N |title=Myelofibrosis after essential thrombocythemia complicated by alveolar proteinosis |journal=Leuk. Lymphoma |volume=44 |issue=6 |pages=1049–52 |date=June 2003 |pmid=12854908 |doi=10.1080/1042819031000063453 |url=}}</ref>
 
====== '''Neurological''' ======
 
*[[Spinal cord compression]]<ref name="pmid28775247">{{cite journal |vauthors=Fareed S, Nashwan AJ, Abu Jarir S, Husain A, Suliman DS, Ibrahim F, Moustafa A, Akhter MS, Yassin MA |title=Spinal Abscess Caused by Salmonella Bacteremia in a Patient with Primary Myelofibrosis |journal=Am J Case Rep |volume=18 |issue= |pages=859–864 |date=August 2017 |pmid=28775247 |pmc=5551928 |doi= |url=}}</ref><ref name="pmid24429645">{{cite journal |vauthors=Hijikata Y, Ando T, Inagaki T, Watanabe H, Ito M, Sobue G |title=[Spinal cord compression due to extramedullary hematopoiesis in a patient with myelofibrosis] |language=Japanese |journal=Rinsho Shinkeigaku |volume=54 |issue=1 |pages=27–31 |date=2014 |pmid=24429645 |doi= |url=}}</ref><ref name="pmid20534957">{{cite journal |vauthors=Kawasaki Y, Nakazora T, Suzukawa M, Tominaga T, Wang ZK, Shinohara K |title=[Neurological disturbance of the lower extremities by an extramedullary hematopoietic mass complicated with primary myelofibrosis] |language=Japanese |journal=Rinsho Ketsueki |volume=51 |issue=5 |pages=349–52 |date=May 2010 |pmid=20534957 |doi= |url=}}</ref><ref name="pmid18441161">{{cite journal |vauthors=Scott IC, Poynton CH |title=Polycythaemia rubra vera and myelofibrosis with spinal cord compression |journal=J. Clin. Pathol. |volume=61 |issue=5 |pages=681–3 |date=May 2008 |pmid=18441161 |doi=10.1136/jcp.2007.053751 |url=}}</ref><ref name="pmid16440779">{{cite journal |vauthors=Ohnishi K, Torimoto Y, Itabashi K, Inamura J, Shindo M, Ikuta K, Sato K, Kohgo Y |title=[Case of intraspinal epidural tumor developing after systemic mastocytosis with marked osteosclerosis and myelofibrosis] |language=Japanese |journal=Rinsho Ketsueki |volume=46 |issue=10 |pages=1146–51 |date=October 2005 |pmid=16440779 |doi= |url=}}</ref><ref name="pmid12365470">{{cite journal |vauthors=de Haas KP, van de Loosdrecht AA, Daenen SM |title=Intraspinal extramedullary haematopoiesis in a patient with myelofibrosis |journal=Neth J Med |volume=60 |issue=6 |pages=256–9 |date=July 2002 |pmid=12365470 |doi= |url=}}</ref><ref name="pmid8027402">{{cite journal |vauthors=Cook G, Sharp RA |title=Spinal cord compression due to extramedullary haemopoiesis in myelofibrosis |journal=J. Clin. Pathol. |volume=47 |issue=5 |pages=464–5 |date=May 1994 |pmid=8027402 |pmc=502028 |doi= |url=}}</ref>
 
====== '''Musculoskeletal''' ======
 
*[[Gout]]<ref name="pmid5216775">{{cite journal |vauthors=Yü TF |title=Secondary gout associated with myeloproliferative diseases |journal=Arthritis Rheum. |volume=8 |issue=5 |pages=765–71 |date=October 1965 |pmid=5216775 |doi= |url=}}</ref><ref name="pmid702809">{{cite journal |vauthors=Nagai Y, Ikebe K, Ito K, Nishizawa T, Akaoka I, Muranaka M, Horiuchi Y |title=[A case of secondary gout associated with myelofibrosis following polycythemia vera (author's transl)] |language=Japanese |journal=Rinsho Ketsueki |volume=19 |issue=3 |pages=226–33 |date=March 1978 |pmid=702809 |doi= |url=}}</ref><ref name="pmid1062009">{{cite journal |vauthors=Yu T, Weinreb N, Wittman R, Wasserman LR |title=Secondary gout associated with chronic myeloproliferative disorders |journal=Semin. Arthritis Rheum. |volume=5 |issue=3 |pages=247–56 |date=February 1976 |pmid=1062009 |doi= |url=}}</ref>
*[[Hypertrophic osteoarthropathy]]<ref name="KelleYıldız2015">{{cite journal|last1=Kelle|first1=Bayram|last2=Yıldız|first2=Fatih|last3=Paydas|first3=Semra|last4=Bagır|first4=Emine Kılıc|last5=Ergin|first5=Melek|last6=Kozanoglu|first6=Erkan|title=Coexistence of hypertrophic osteoarthropathy and myelofibrosis|journal=Revista Brasileira de Reumatologia (English Edition)|year=2015|issn=22555021|doi=10.1016/j.rbre.2014.11.004}}</ref><ref name="pmid29037318">{{cite journal |vauthors=Kelle B, Yıldız F, Paydas S, Bagır EK, Ergin M, Kozanoglu E |title=Coexistence of hypertrophic osteoarthropathy and myelofibrosis |journal=Rev Bras Reumatol Engl Ed |volume=57 |issue=5 |pages=472–474 |date=2017 |pmid=29037318 |doi=10.1016/j.rbre.2014.11.004 |url=}}</ref><ref name="pmid25785156">{{cite journal |vauthors=Li S, Li Q, Wang Q, Chen D, Li J |title=Primary hypertrophic osteoarthropathy with myelofibrosis and anemia: a case report and review of literature |journal=Int J Clin Exp Med |volume=8 |issue=1 |pages=1467–71 |date=2015 |pmid=25785156 |pmc=4358611 |doi= |url=}}</ref><ref name="pmid18038138">{{cite journal |vauthors=Saghafi M, Azarian A, Nohesara N |title=Primary hypertrophic osteoarthropathy with myelofibrosis |journal=Rheumatol. Int. |volume=28 |issue=6 |pages=597–600 |date=April 2008 |pmid=18038138 |doi=10.1007/s00296-007-0477-4 |url=}}</ref><ref name="pmid15107873">{{cite journal |vauthors=John B, Subhash H, Thomas K |title=Case of myelofibrosis with hypertrophic osteoarthropathy: the role of platelet-derived growth factor in pathogenesis |journal=N. Z. Med. J. |volume=117 |issue=1192 |pages=U853 |date=April 2004 |pmid=15107873 |doi= |url=}}</ref>
 
====== '''Other''' ======
 
*[[Acute myelogenous leukemia]]<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="pmid6930986">{{cite journal |vauthors=Amjad H, Gezer S, Inoue S, Bollinger RO, Kaplan J, Carson S, Bishop CR |title=Acute myelofibrosis terminating in an acute lymphoblastic leukemia: a case report |journal=Cancer |volume=46 |issue=3 |pages=615–8 |date=August 1980 |pmid=6930986 |doi= |url=}}</ref><ref name="pmid22170483">{{cite journal |vauthors=Kundranda MN, Tibes R, Mesa RA |title=Transformation of a chronic myeloproliferative neoplasm to acute myelogenous leukemia: does anything work? |journal=Curr Hematol Malig Rep |volume=7 |issue=1 |pages=78–86 |date=March 2012 |pmid=22170483 |doi=10.1007/s11899-011-0107-9 |url=}}</ref>
*[[Infections]]<ref name="pmid28775247">{{cite journal |vauthors=Fareed S, Nashwan AJ, Abu Jarir S, Husain A, Suliman DS, Ibrahim F, Moustafa A, Akhter MS, Yassin MA |title=Spinal Abscess Caused by Salmonella Bacteremia in a Patient with Primary Myelofibrosis |journal=Am J Case Rep |volume=18 |issue= |pages=859–864 |date=August 2017 |pmid=28775247 |pmc=5551928 |doi= |url=}}</ref><ref name="pmid30057341">{{cite journal |vauthors=Karigane D, Kikuchi T, Sakurai M, Kato J, Yamane Y, Hashida R, Abe R, Hatano M, Hasegawa N, Wakayama M, Shibuya K, Okamoto S, Mori T |title=Invasive hepatic mucormycosis: A case report and review of the literature |journal=J. Infect. Chemother. |volume= |issue= |pages= |date=July 2018 |pmid=30057341 |doi=10.1016/j.jiac.2018.06.013 |url=}}</ref>
*[[Myeloperoxidase deficiency|Acquired myeloperoxidase deficiency]]<ref name="pmid27013444">{{cite journal |vauthors=Theocharides AP, Lundberg P, Lakkaraju AK, Lysenko V, Myburgh R, Aguzzi A, Skoda RC, Manz MG |title=Homozygous calreticulin mutations in patients with myelofibrosis lead to acquired myeloperoxidase deficiency |journal=Blood |volume=127 |issue=25 |pages=3253–9 |date=June 2016 |pmid=27013444 |doi=10.1182/blood-2016-02-696310 |url=}}</ref>
 
===Prognosis===
 
*The Dynamic International Prognostic Scoring System (DIPSS)‐plus currently provides the most comprehensive [[Prognosis|prognostic]] tool for [[Primary myelofibrosis|primary myelofibrosis (PMF)]]. This scoring system comprises of eight risk variables:<ref name="pmid21149668">{{cite journal |vauthors=Gangat N, Caramazza D, Vaidya R, George G, Begna K, Schwager S, Van Dyke D, Hanson C, Wu W, Pardanani A, Cervantes F, Passamonti F, Tefferi A |title=DIPSS plus: a refined Dynamic International Prognostic Scoring System for primary myelofibrosis that incorporates prognostic information from karyotype, platelet count, and transfusion status |journal=J. Clin. Oncol. |volume=29 |issue=4 |pages=392–7 |date=February 2011 |pmid=21149668 |doi=10.1200/JCO.2010.32.2446 |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="pmid27521306">{{cite journal |vauthors=Bose P, Verstovsek S |title=Prognosis of Primary Myelofibrosis in the Genomic Era |journal=Clin Lymphoma Myeloma Leuk |volume=16 Suppl |issue= |pages=S105–13 |date=August 2016 |pmid=27521306 |pmc=4987499 |doi=10.1016/j.clml.2016.02.031 |url=}}</ref>
**Age >65 years
**[[Hemoglobin]] <10 g/dl
**[[Leucocyte]] count >25 × 10<sup>9</sup>/l
**Circulating [[Blast|blasts]] ≥1%
**Constitutional [[Symptom|symptoms]]
**Unfavourable [[karyotype]] (i.e., complex [[karyotype]] or sole or two abnormalities that include +8, -7/7q-, i(17q), inv(3), 5/5q-, 12p-, or 11q23 rearrangement)
**[[Transfusion]] dependency
**[[Platelet]] count <100 × 10<sup>9</sup>/l
*The presence of 0, 1, 2 or 3, and ≥4 adverse factors defines low, intermediate-1, intermediate-2 and high-risk [[disease]] with [[median]] survivals of approximately 15.4, 6.5, 2.9 and 1.3 years, respectively.
 
====== '''[[DIPSS Plus Score|DIPSS Plus Score Calculator for Prognosis in Myelofibrosis]]''' ======
 
*A [[genetically]] inspired [[Prognosis|prognostic]] scoring system (GIPSS) that stratifies [[Primary myelofibrosis|primary myelofibrosis (PMF)]] [[Patient|patients]] by [[genetic]] variants alone has recently been proposed but the lack of overlapping [[Prognosis|prognostic]] variables between the dynamic international prognostic scoring system (DIPSS) and the genetically inspired prognostic scoring system (GIPSS) has also increased the risk for disagreement between the two valid [[Prognosis|prognostic]] models.<ref name="pmid30390311">{{cite journal |vauthors=Kuykendall AT, Talati C, Padron E, Sweet K, Sallman D, List AF, Lancet JE, Komrokji RS |title=Genetically inspired prognostic scoring system (GIPSS) outperforms dynamic international prognostic scoring system (DIPSS) in myelofibrosis patients |journal=Am. J. Hematol. |volume= |issue= |pages= |date=November 2018 |pmid=30390311 |doi=10.1002/ajh.25335 |url=}}</ref>
*The [[Genetics|genetically]] inspired prognostic scoring system (GIPSS) performs equally well for both primary and secondary [[myelofibrosis]] and outperforms the dynamic international prognostic scoring system (DIPSS) in [[Patient|patients]] where the two models disagree.
*[[Monocytosis]] is also a powerful and can be an independent predictor of inferior survival in [[Primary myelofibrosis|primary myelofibrosis (PMF)]].<ref name="pmid29265333">{{cite journal |vauthors=Tefferi A, Shah S, Mudireddy M, Lasho TL, Barraco D, Hanson CA, Ketterling RP, Elliott MA, Patnaik MS, Pardanani A, Gangat N |title=Monocytosis is a powerful and independent predictor of inferior survival in primary myelofibrosis |journal=Br. J. Haematol. |volume= |issue= |pages= |date=December 2017 |pmid=29265333 |doi=10.1111/bjh.15061 |url=}}</ref>
*Marked elevation of serum [[Lactate dehydrogenase|lactate dehydrogenase (LDH)]] can independently predict shorter overall and [[leukemia]]-free survival in [[Primary myelofibrosis|primary myelofibrosis (PMF)]] [[Patient|patients]].<ref name="pmid29249804">{{cite journal |vauthors=Shah S, Mudireddy M, Hanson CA, Ketterling RP, Gangat N, Pardanani A, Tefferi A |title=Marked elevation of serum lactate dehydrogenase in primary myelofibrosis: clinical and prognostic correlates |journal=Blood Cancer J |volume=7 |issue=12 |pages=657 |date=December 2017 |pmid=29249804 |pmc=5802557 |doi=10.1038/s41408-017-0024-9 |url=}}</ref>
*[[Endogenous]] [[erythroid]] colony (EEC) growth, a higher JAK2<sup>V617F</sup> [[allele]] burden, and [[Calreticulin|calreticulin (CALR)]] [[Mutation|mutations]] can also act as independent predictors for better outcomes in [[Primary myelofibrosis|primary myelofibrosis (PMF)]].<ref name="pmid29203554">{{cite journal |vauthors=Kuo MC, Lin TH, Sun CF, Lin TL, Wu JH, Wang PN, Huang YJ, Chang H, Huang TY, Shih LY |title=The clinical and prognostic relevance of driver mutations in 203 Taiwanese patients with primary myelofibrosis |journal=J. Clin. Pathol. |volume=71 |issue=6 |pages=514–521 |date=June 2018 |pmid=29203554 |doi=10.1136/jclinpath-2017-204829 |url=}}</ref><ref name="pmid29164670">{{cite journal |vauthors=Tefferi A, Nicolosi M, Mudireddy M, Szuber N, Finke CM, Lasho TL, Hanson CA, Ketterling RP, Pardanani A, Gangat N, Mannarelli C, Fanelli T, Guglielmelli P, Vannucchi AM |title=Driver mutations and prognosis in primary myelofibrosis: Mayo-Careggi MPN alliance study of 1,095 patients |journal=Am. J. Hematol. |volume=93 |issue=3 |pages=348–355 |date=March 2018 |pmid=29164670 |doi=10.1002/ajh.24978 |url=}}</ref>


==References==
==References==
Line 32: Line 98:
{{WikiDoc Help Menu}}
{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
{{WikiDoc Sources}}
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Neurology]]
[[Category:Neurosurgery]]

Latest revision as of 15:23, 12 November 2019

Myelofibrosis Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Myelofibrosis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Myelofibrosis natural history, complications and prognosis On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Myelofibrosis natural history, complications and prognosis

All Images
X-rays
Echo and Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Myelofibrosis natural history, complications and prognosis

CDC on Myelofibrosis natural history, complications and prognosis

Myelofibrosis natural history, complications and prognosis in the news

Blogs on Myelofibrosis natural history, complications and prognosis

Directions to Hospitals Treating Myelofibrosis

Risk calculators and risk factors for Myelofibrosis natural history, complications and prognosis

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

The development of myelofibrosis is a a slow process and it does not cause early symptoms. A significant proportion of the patients can be asymptomatic and the diagnosis is usually made in the setting of an unrelated condition. The most overlapping and common findings encountered are anemia and splenomegaly presenting as weakness, easy fatigability, palpitations, and dyspnea in the case of anemia and early satiety with possible accompanying left upper quadrant discomfort if splenomegaly is present. The disease has a progressive course and can result in pancytopenia as the bone marrow failure ensues. This can result in bleeding complications, easy bruising, increase in the susceptibility to infections, and worsening anemia. The bone marrow failure paves the way for extramedullary hematopoiesis (EMH) which mainly occurs in the reticuloendothelial tissues. If left untreated, myelofibrosis can lead to severe complications, the most feared of which are acute leukemia, heart failure, and portal hypertension.

Natural History, Complications, and Prognosis

Natural History

Complications

Common complications of myelofibrosis include:

Hematologic
Gastroentistinal
Urological
Cardiopulmonary
Neurological
Musculoskeletal
Other

Prognosis

  • The Dynamic International Prognostic Scoring System (DIPSS)‐plus currently provides the most comprehensive prognostic tool for primary myelofibrosis (PMF). This scoring system comprises of eight risk variables:[75][4][76]
  • The presence of 0, 1, 2 or 3, and ≥4 adverse factors defines low, intermediate-1, intermediate-2 and high-risk disease with median survivals of approximately 15.4, 6.5, 2.9 and 1.3 years, respectively.
DIPSS Plus Score Calculator for Prognosis in Myelofibrosis

References

  1. ↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
  2. ↑ 2.0 2.1 Cervantes F, Pereira A, Esteve J, Cobo F, Rozman C, Montserrat E (November 1997). "[Idiopathic myelofibrosis: initial features, evolutive patterns and survival in a series of 106 patients]". Med Clin (Barc) (in Spanish; Castilian). 109 (17): 651–5. PMID 9488952.
  3. ↑ O'Sullivan JM, Harrison CN (February 2018). "Myelofibrosis: clinicopathologic features, prognosis, and management". Clin Adv Hematol Oncol. 16 (2): 121–131. PMID 29741513.
  4. ↑ 4.0 4.1 4.2 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.
  5. ↑ 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.
  6. ↑ 6.0 6.1 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.
  7. ↑ 7.0 7.1 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.
  8. ↑ 8.0 8.1 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.
  9. ↑ 9.0 9.1 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.
  10. ↑ 10.0 10.1 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.
  11. ↑ 11.0 11.1 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.
  12. ↑ Birgegard G, Samuelsson J, Ahlstrand E, Ejerblad E, Enevold C, Ghanima W, Hasselbalch H, Nielsen CH, Knutsen H, Pedersen OB, Sørensen A, Andreasson B (November 2018). "Inflammatory functional iron deficiency common in myelofibrosis, contributes to anaemia and impairs quality of life. From the Nordic MPN study Group". Eur. J. Haematol. doi:10.1111/ejh.13198. PMID 30472746.
  13. ↑ Chahdi H, Oukabli M (2018). "[A special form of pancytopenia]". Pan Afr Med J (in French). 29: 209. doi:10.11604/pamj.2018.29.209.14055. PMC 6080970. PMID 30100963.
  14. ↑ Tefferi A (December 2018). "Primary myelofibrosis: 2019 update on diagnosis, risk-stratification and management". Am. J. Hematol. 93 (12): 1551–1560. doi:10.1002/ajh.25230. PMID 30039550.
  15. ↑ 15.0 15.1 Karigane D, Kikuchi T, Sakurai M, Kato J, Yamane Y, Hashida R, Abe R, Hatano M, Hasegawa N, Wakayama M, Shibuya K, Okamoto S, Mori T (July 2018). "Invasive hepatic mucormycosis: A case report and review of the literature". J. Infect. Chemother. doi:10.1016/j.jiac.2018.06.013. PMID 30057341.
  16. ↑ Finazzi G, Vannucchi AM, Barbui T (November 2018). "Prefibrotic myelofibrosis: treatment algorithm 2018". Blood Cancer J. 8 (11): 104. doi:10.1038/s41408-018-0142-z. PMC 6221891. PMID 30405096.
  17. ↑ Hofmann I, Geer MJ, Vögtle T, Crispin A, Campagna DR, Barr A, Calicchio ML, Heising S, van Geffen JP, Kuijpers M, Heemskerk J, Eble JA, Schmitz-Abe K, Obeng EA, Douglas M, Freson K, Pondarré C, Favier R, Jarvis GE, Markianos K, Turro E, Ouwehand WH, Mazharian A, Fleming MD, Senis YA (September 2018). "Congenital macrothrombocytopenia with focal myelofibrosis due to mutations in human G6b-B is rescued in humanized mice". Blood. 132 (13): 1399–1412. doi:10.1182/blood-2017-08-802769. PMID 29898956. Vancouver style error: initials (help)
  18. ↑ 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.
  19. ↑ 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.
  20. ↑ 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.
  21. ↑ 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.
  22. ↑ 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.
  23. ↑ 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.
  24. ↑ 24.0 24.1 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.
  25. ↑ 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.
  26. ↑ 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.
  27. ↑ Gwaltney C, Paty J, Kwitkowski VE, Mesa RA, Dueck AC, Papadopoulos EJ, Wang L, Feliciano J, Coons SJ (August 2017). "Development of a harmonized patient-reported outcome questionnaire to assess myelofibrosis symptoms in clinical trials". Leuk. Res. 59: 26–31. doi:10.1016/j.leukres.2017.05.012. PMID 28544906.
  28. ↑ 28.0 28.1 Kc D, Falchi L, Verstovsek S (October 2017). "The underappreciated risk of thrombosis and bleeding in patients with myelofibrosis: a review". Ann. Hematol. 96 (10): 1595–1604. doi:10.1007/s00277-017-3099-2. PMC 5693670. PMID 28808761.
  29. ↑ Complications of primary myelofibrosis. Dr Henry Knipe and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/myelofibrosis. Accessed on March 10, 2016
  30. ↑ Finazzi G, De Stefano V, Barbui T (June 2018). "Splanchnic vein thrombosis in myeloproliferative neoplasms: treatment algorithm 2018". Blood Cancer J. 8 (7): 64. doi:10.1038/s41408-018-0100-9. PMC 6018786. PMID 29946154.
  31. ↑ Sández Montagut VM, Giráldez Gallego Á, Ontanilla Clavijo G (March 2018). "Regenerative nodular hyperplasia, portal vein thrombosis and primary myelofibrosis: an unusual triple association". Rev Esp Enferm Dig. 110 (3): 209–210. doi:10.17235/reed.2018.5349/2017. PMID 29368941.
  32. ↑ How J, Trinkaus KM, Oh ST (October 2018). "Distinct clinical, laboratory and molecular features of myeloproliferative neoplasm patients with splanchnic vein thrombosis". Br. J. Haematol. 183 (2): 310–313. doi:10.1111/bjh.14958. PMID 29048104.
  33. ↑ Campos-Cabrera G, Campos-Cabrera V, Campos-Cabrera S, Campos-Villagómez JL, Romero-González A (2017). "Splanchnic vein thrombosis as a first manifestation of Primary myelofibrosis". Gac Med Mex (in Spanish; Castilian). 153 (4): 537–540. doi:10.24875/GMM.17002822. PMID 28991281.
  34. ↑ Escorsell A, Montero A (November 1994). "[Severe acute liver insufficiency in a 72 year old male with idiopathic myelofibrosis]". Med Clin (Barc) (in Spanish; Castilian). 103 (16): 628–35. PMID 7996922.
  35. ↑ McBrine PA, Miller A, Zimelman AP, Koff RS (November 1980). "Polycythemia vera with myelofibrosis and myeloid metaplasia. Acute hepatic failure following splenectomy". Am. J. Clin. Pathol. 74 (5): 693–6. PMID 7446477.
  36. ↑ Saboo SS, Krajewski KM, O'Regan KN, Giardino A, Brown JR, Ramaiya N, Jagannathan JP (January 2012). "Spleen in haematological malignancies: spectrum of imaging findings". Br J Radiol. 85 (1009): 81–92. doi:10.1259/bjr/31542964. PMC 3473934. PMID 22096219.
  37. ↑ Baba M, Tanahashi N, Seno K, Nara M, Shinbo T (May 1990). "[Myelofibrosis with marked subcapsular bleeding of the spleen--a case report]". Rinsho Ketsueki (in Japanese). 31 (5): 669–73. PMID 2395215.
  38. ↑ Tokai K, Miyatani H, Yoshida Y, Yamada S (July 2012). "Multiple esophageal variceal ruptures with massive ascites due to myelofibrosis-induced portal hypertension". World J. Gastroenterol. 18 (28): 3770–4. doi:10.3748/wjg.v18.i28.3770. PMC 3406433. PMID 22851873.
  39. ↑ Bĕlohlávek J, Schwarz J, Jirásek A, Krajina A, Polák F, Hrubý M (March 2001). "Idiopathic myelofibrosis complicated by portal hypertension treated with a transjugular intrahepatic portosystemic shunt (TIPS)". Wien. Klin. Wochenschr. 113 (5–6): 208–11. PMID 11293952.
  40. ↑ Mughal TI, Vaddi K, Sarlis NJ, Verstovsek S (2014). "Myelofibrosis-associated complications: pathogenesis, clinical manifestations, and effects on outcomes". Int J Gen Med. 7: 89–101. doi:10.2147/IJGM.S51800. PMC 3912063. PMID 24501543.
  41. ↑ Doki N, Irisawa H, Takada S, Sakura T, Miyawaki S (2007). "Transjugular intrahepatic portosystemic shunt for the treatment of portal hypertension due to idiopathic myelofibrosis". Intern. Med. 46 (4): 187–90. PMID 17301514.
  42. ↑ Srinivasaiah N, Zia MK, Muralikrishnan V (December 2010). "Peritonitis in myelofibrosis: a cautionary tale". HBPD INT. 9 (6): 651–3. PMID 21134837.
  43. ↑ Wei XQ, Zheng ZH, Jin Y, Tao J, Abassa KK, Wen ZF, Shao CK, Wei HB, Wu B (September 2014). "Intestinal obstruction caused by extramedullary hematopoiesis and ascites in primary myelofibrosis". World J. Gastroenterol. 20 (33): 11921–6. doi:10.3748/wjg.v20.i33.11921. PMC 4155387. PMID 25206301.
  44. ↑ Babushok DV, Nelson EJ, Morrissette J, Joshi S, Palmer MB, Frank D, Cambor CL, Hexner EO (September 2018). "Myelofibrosis patients can develop extramedullary complications including renal amyloidosis and sclerosing hematopoietic tumor while otherwise meeting traditional measures of ruxolitinib response". Leuk. Lymphoma: 1–4. doi:10.1080/10428194.2018.1509319. PMID 30227762. Vancouver style error: initials (help)
  45. ↑ Kaygusuz I, Koc M, Arikan H, Adiguzel C, Cakalagaoglu F, Tuglular TF, Akoglu E (January 2010). "Focal segmental glomerulosclerosis associated with idiopathic myelofibrosis". Ren Fail. 32 (2): 273–6. doi:10.3109/08860220903573286. PMID 20199191.
  46. ↑ Bohra GK, Meena DS, Bajpai N, Purohit A (May 2018). "Focal segmental glomerulosclerosis in a patient with prefibrotic primary myelofibrosis". BMJ Case Rep. 2018. doi:10.1136/bcr-2017-223803. PMID 29728434.
  47. ↑ 47.0 47.1 Rajasekaran A, Ngo TT, Abdelrahim M, Glass W, Podoll A, Verstovsek S, Abudayyeh A (August 2015). "Primary myelofibrosis associated glomerulopathy: significant improvement after therapy with ruxolitinib". BMC Nephrol. 16: 121. doi:10.1186/s12882-015-0121-6. PMC 4521341. PMID 26232031.
  48. ↑ Christensen AS, Møller JB, Hasselbalch HC (April 2014). "Chronic kidney disease in patients with the Philadelphia-negative chronic myeloproliferative neoplasms". Leuk. Res. 38 (4): 490–5. doi:10.1016/j.leukres.2014.01.014. PMID 24630365.
  49. ↑ Cvetković ZP, Cvetković BR, Celeketić D, Milenković D, Perunicić-Peković G (2010). "Bilateral ureteral obstruction due to primary myelofibrosis caused hyperuricaemia". Acta Chir Iugosl. 57 (2): 79–83. PMID 20949707.
  50. ↑ Shimono J, Tsutsumi Y, Ohigashi H (January 2015). "[Acute renal tubular damage caused by disseminated Trichosporon infection in primary myelofibrosis]". Rinsho Ketsueki (in Japanese). 56 (1): 21–4. doi:10.11406/rinketsu.56.21. PMID 25745963.
  51. ↑ Guilpain P, Montani D, Damaj G, Achouh L, Lefrère F, Le Pavec J, Marfaing-Koka A, Dartevelle P, Simonneau G, Humbert M, Hermine O (2008). "Pulmonary hypertension associated with myeloproliferative disorders: a retrospective study of ten cases". Respiration. 76 (3): 295–302. doi:10.1159/000112822. PMID 18160817.
  52. ↑ García-Manero G, Schuster SJ, Patrick H, Martinez J (February 1999). "Pulmonary hypertension in patients with myelofibrosis secondary to myeloproliferative diseases". Am. J. Hematol. 60 (2): 130–5. PMID 9929105.
  53. ↑ Singh I, Mikita G, Green D, Risquez C, Sanders A (March 2017). "Pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series". Pulm Circ. 7 (1): 261–267. doi:10.1177/2045893217702064. PMC 5448544. PMID 28680586.
  54. ↑ Faiz SA, Iliescu C, Lopez-Mattei J, Patel B, Bashoura L, Popat U (December 2016). "Resolution of myelofibrosis-associated pulmonary arterial hypertension following allogeneic hematopoietic stem cell transplantation". Pulm Circ. 6 (4): 611–613. doi:10.1086/687291. PMC 5210054. PMID 28090305.
  55. ↑ Mathew R, Huang J, Wu JM, Fallon JT, Gewitz MH (December 2016). "Hematological disorders and pulmonary hypertension". World J Cardiol. 8 (12): 703–718. doi:10.4330/wjc.v8.i12.703. PMC 5183970. PMID 28070238.
  56. ↑ Tsutsumi Y, Tanaka J, Saito S, Tanaka Y, Kawamura T, Obara S, Noto S, Shimoyama N, Asaka M, Imamura M, Masauzi N (June 2003). "Myelofibrosis after essential thrombocythemia complicated by alveolar proteinosis". Leuk. Lymphoma. 44 (6): 1049–52. doi:10.1080/1042819031000063453. PMID 12854908.
  57. ↑ 57.0 57.1 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.
  58. ↑ 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.
  59. ↑ Kawasaki Y, Nakazora T, Suzukawa M, Tominaga T, Wang ZK, Shinohara K (May 2010). "[Neurological disturbance of the lower extremities by an extramedullary hematopoietic mass complicated with primary myelofibrosis]". Rinsho Ketsueki (in Japanese). 51 (5): 349–52. PMID 20534957.
  60. ↑ Scott IC, Poynton CH (May 2008). "Polycythaemia rubra vera and myelofibrosis with spinal cord compression". J. Clin. Pathol. 61 (5): 681–3. doi:10.1136/jcp.2007.053751. PMID 18441161.
  61. ↑ Ohnishi K, Torimoto Y, Itabashi K, Inamura J, Shindo M, Ikuta K, Sato K, Kohgo Y (October 2005). "[Case of intraspinal epidural tumor developing after systemic mastocytosis with marked osteosclerosis and myelofibrosis]". Rinsho Ketsueki (in Japanese). 46 (10): 1146–51. PMID 16440779.
  62. ↑ de Haas KP, van de Loosdrecht AA, Daenen SM (July 2002). "Intraspinal extramedullary haematopoiesis in a patient with myelofibrosis". Neth J Med. 60 (6): 256–9. PMID 12365470.
  63. ↑ Cook G, Sharp RA (May 1994). "Spinal cord compression due to extramedullary haemopoiesis in myelofibrosis". J. Clin. Pathol. 47 (5): 464–5. PMC 502028. PMID 8027402.
  64. ↑ Yü TF (October 1965). "Secondary gout associated with myeloproliferative diseases". Arthritis Rheum. 8 (5): 765–71. PMID 5216775.
  65. ↑ Nagai Y, Ikebe K, Ito K, Nishizawa T, Akaoka I, Muranaka M, Horiuchi Y (March 1978). "[A case of secondary gout associated with myelofibrosis following polycythemia vera (author's transl)]". Rinsho Ketsueki (in Japanese). 19 (3): 226–33. PMID 702809.
  66. ↑ Yu T, Weinreb N, Wittman R, Wasserman LR (February 1976). "Secondary gout associated with chronic myeloproliferative disorders". Semin. Arthritis Rheum. 5 (3): 247–56. PMID 1062009.
  67. ↑ Kelle, Bayram; Yıldız, Fatih; Paydas, Semra; Bagır, Emine Kılıc; Ergin, Melek; Kozanoglu, Erkan (2015). "Coexistence of hypertrophic osteoarthropathy and myelofibrosis". Revista Brasileira de Reumatologia (English Edition). doi:10.1016/j.rbre.2014.11.004. ISSN 2255-5021.
  68. ↑ Kelle B, Yıldız F, Paydas S, Bagır EK, Ergin M, Kozanoglu E (2017). "Coexistence of hypertrophic osteoarthropathy and myelofibrosis". Rev Bras Reumatol Engl Ed. 57 (5): 472–474. doi:10.1016/j.rbre.2014.11.004. PMID 29037318.
  69. ↑ Li S, Li Q, Wang Q, Chen D, Li J (2015). "Primary hypertrophic osteoarthropathy with myelofibrosis and anemia: a case report and review of literature". Int J Clin Exp Med. 8 (1): 1467–71. PMC 4358611. PMID 25785156.
  70. ↑ Saghafi M, Azarian A, Nohesara N (April 2008). "Primary hypertrophic osteoarthropathy with myelofibrosis". Rheumatol. Int. 28 (6): 597–600. doi:10.1007/s00296-007-0477-4. PMID 18038138.
  71. ↑ John B, Subhash H, Thomas K (April 2004). "Case of myelofibrosis with hypertrophic osteoarthropathy: the role of platelet-derived growth factor in pathogenesis". N. Z. Med. J. 117 (1192): U853. PMID 15107873.
  72. ↑ Amjad H, Gezer S, Inoue S, Bollinger RO, Kaplan J, Carson S, Bishop CR (August 1980). "Acute myelofibrosis terminating in an acute lymphoblastic leukemia: a case report". Cancer. 46 (3): 615–8. PMID 6930986.
  73. ↑ Kundranda MN, Tibes R, Mesa RA (March 2012). "Transformation of a chronic myeloproliferative neoplasm to acute myelogenous leukemia: does anything work?". Curr Hematol Malig Rep. 7 (1): 78–86. doi:10.1007/s11899-011-0107-9. PMID 22170483.
  74. ↑ Theocharides AP, Lundberg P, Lakkaraju AK, Lysenko V, Myburgh R, Aguzzi A, Skoda RC, Manz MG (June 2016). "Homozygous calreticulin mutations in patients with myelofibrosis lead to acquired myeloperoxidase deficiency". Blood. 127 (25): 3253–9. doi:10.1182/blood-2016-02-696310. PMID 27013444.
  75. ↑ Gangat N, Caramazza D, Vaidya R, George G, Begna K, Schwager S, Van Dyke D, Hanson C, Wu W, Pardanani A, Cervantes F, Passamonti F, Tefferi A (February 2011). "DIPSS plus: a refined Dynamic International Prognostic Scoring System for primary myelofibrosis that incorporates prognostic information from karyotype, platelet count, and transfusion status". J. Clin. Oncol. 29 (4): 392–7. doi:10.1200/JCO.2010.32.2446. PMID 21149668.
  76. ↑ Bose P, Verstovsek S (August 2016). "Prognosis of Primary Myelofibrosis in the Genomic Era". Clin Lymphoma Myeloma Leuk. 16 Suppl: S105–13. doi:10.1016/j.clml.2016.02.031. PMC 4987499. PMID 27521306.
  77. ↑ Kuykendall AT, Talati C, Padron E, Sweet K, Sallman D, List AF, Lancet JE, Komrokji RS (November 2018). "Genetically inspired prognostic scoring system (GIPSS) outperforms dynamic international prognostic scoring system (DIPSS) in myelofibrosis patients". Am. J. Hematol. doi:10.1002/ajh.25335. PMID 30390311.
  78. ↑ Tefferi A, Shah S, Mudireddy M, Lasho TL, Barraco D, Hanson CA, Ketterling RP, Elliott MA, Patnaik MS, Pardanani A, Gangat N (December 2017). "Monocytosis is a powerful and independent predictor of inferior survival in primary myelofibrosis". Br. J. Haematol. doi:10.1111/bjh.15061. PMID 29265333.
  79. ↑ Shah S, Mudireddy M, Hanson CA, Ketterling RP, Gangat N, Pardanani A, Tefferi A (December 2017). "Marked elevation of serum lactate dehydrogenase in primary myelofibrosis: clinical and prognostic correlates". Blood Cancer J. 7 (12): 657. doi:10.1038/s41408-017-0024-9. PMC 5802557. PMID 29249804.
  80. ↑ Kuo MC, Lin TH, Sun CF, Lin TL, Wu JH, Wang PN, Huang YJ, Chang H, Huang TY, Shih LY (June 2018). "The clinical and prognostic relevance of driver mutations in 203 Taiwanese patients with primary myelofibrosis". J. Clin. Pathol. 71 (6): 514–521. doi:10.1136/jclinpath-2017-204829. PMID 29203554.
  81. ↑ Tefferi A, Nicolosi M, Mudireddy M, Szuber N, Finke CM, Lasho TL, Hanson CA, Ketterling RP, Pardanani A, Gangat N, Mannarelli C, Fanelli T, Guglielmelli P, Vannucchi AM (March 2018). "Driver mutations and prognosis in primary myelofibrosis: Mayo-Careggi MPN alliance study of 1,095 patients". Am. J. Hematol. 93 (3): 348–355. doi:10.1002/ajh.24978. PMID 29164670.


Template:WikiDoc Sources

Retrieved from "https://www.wikidoc.org/index.php?title=Myelofibrosis_natural_history,_complications_and_prognosis&oldid=1588927"