Myelofibrosis diagnostic study of choice: Difference between revisions
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|}<ref name="pmid17210175">{{cite journal |vauthors=Mesa RA, Verstovsek S, Cervantes F, Barosi G, Reilly JT, Dupriez B, Levine R, Le Bousse-Kerdiles MC, Wadleigh M, Campbell PJ, Silver RT, Vannucchi AM, Deeg HJ, Gisslinger H, Thomas D, Odenike O, Solberg LA, Gotlib J, Hexner E, Nimer SD, Kantarjian H, Orazi A, Vardiman JW, Thiele J, Tefferi A |title=Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): Consensus on terminology by the international working group for myelofibrosis research and treatment (IWG-MRT) |journal=Leuk. Res. |volume=31 |issue=6 |pages=737–40 |date=June 2007 |pmid=17210175 |doi=10.1016/j.leukres.2006.12.002 |url=}}</ref><ref name="TefferiThiele2007">{{cite journal|last1=Tefferi|first1=A.|last2=Thiele|first2=J.|last3=Orazi|first3=A.|last4=Kvasnicka|first4=H. M.|last5=Barbui|first5=T.|last6=Hanson|first6=C. A.|last7=Barosi|first7=G.|last8=Verstovsek|first8=S.|last9=Birgegard|first9=G.|last10=Mesa|first10=R.|last11=Reilly|first11=J. T.|last12=Gisslinger|first12=H.|last13=Vannucchi|first13=A. M.|last14=Cervantes|first14=F.|last15=Finazzi|first15=G.|last16=Hoffman|first16=R.|last17=Gilliland|first17=D. G.|last18=Bloomfield|first18=C. D.|last19=Vardiman|first19=J. W.|title=Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel|journal=Blood|volume=110|issue=4|year=2007|pages=1092–1097|issn=0006-4971|doi=10.1182/blood-2007-04-083501}}</ref> | |}<ref name="pmid17210175">{{cite journal |vauthors=Mesa RA, Verstovsek S, Cervantes F, Barosi G, Reilly JT, Dupriez B, Levine R, Le Bousse-Kerdiles MC, Wadleigh M, Campbell PJ, Silver RT, Vannucchi AM, Deeg HJ, Gisslinger H, Thomas D, Odenike O, Solberg LA, Gotlib J, Hexner E, Nimer SD, Kantarjian H, Orazi A, Vardiman JW, Thiele J, Tefferi A |title=Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): Consensus on terminology by the international working group for myelofibrosis research and treatment (IWG-MRT) |journal=Leuk. Res. |volume=31 |issue=6 |pages=737–40 |date=June 2007 |pmid=17210175 |doi=10.1016/j.leukres.2006.12.002 |url=}}</ref><ref name="TefferiThiele2007">{{cite journal|last1=Tefferi|first1=A.|last2=Thiele|first2=J.|last3=Orazi|first3=A.|last4=Kvasnicka|first4=H. M.|last5=Barbui|first5=T.|last6=Hanson|first6=C. A.|last7=Barosi|first7=G.|last8=Verstovsek|first8=S.|last9=Birgegard|first9=G.|last10=Mesa|first10=R.|last11=Reilly|first11=J. T.|last12=Gisslinger|first12=H.|last13=Vannucchi|first13=A. M.|last14=Cervantes|first14=F.|last15=Finazzi|first15=G.|last16=Hoffman|first16=R.|last17=Gilliland|first17=D. G.|last18=Bloomfield|first18=C. D.|last19=Vardiman|first19=J. W.|title=Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel|journal=Blood|volume=110|issue=4|year=2007|pages=1092–1097|issn=0006-4971|doi=10.1182/blood-2007-04-083501}}</ref> | ||
===Proposed revised World Health Organization (WHO) criteria for primary myelofibrosis (PMF)=== | ===Proposed revised 2016 World Health Organization (WHO) criteria for primary myelofibrosis (PMF)=== | ||
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| style="padding: 5px 5px; background: #DCDCDC;" |'''Major criteria''' | | style="padding: 5px 5px; background: #DCDCDC;" |'''Major criteria''' | ||
#Presence of [[megakaryocyte]] proliferation and [[atypia]],* usually accompanied by either [[reticulin]] and/or [[collagen]] [[fibrosis]], or, in the absence of significant [[reticulin]] [[fibrosis]], the [[megakaryocyte]] changes must be accompanied by an increased [[bone marrow]] cellularity characterized by [[Granulocyte|granulocytic]] proliferation and often decreased [[erythropoiesis]] (ie, prefibrotic cellular-phase disease) | #Presence of [[megakaryocyte]] proliferation and [[atypia]],* usually accompanied by either [[reticulin]] and/or [[collagen]] [[fibrosis]], or, in the absence of significant [[reticulin]] [[fibrosis]], the [[megakaryocyte]] changes must be accompanied by an increased [[bone marrow]] cellularity characterized by [[Granulocyte|granulocytic]] proliferation and often decreased [[erythropoiesis]] (ie, prefibrotic cellular-phase disease) | ||
#Not meeting [[World Health Organization|World Health Organization (WHO)]] criteria for [[Polycythemia vera|polycythemia vera (PV)]], † [[Chronic myelogenous leukemia|chronic myelogenous leukemia (CML)]], ‡ [[Myelodysplastic syndrome|myelodysplastic syndrome (MDS)]], § or other [[myeloid]] [[neoplasm]] | #Not meeting [[World Health Organization|World Health Organization (WHO)]] criteria for [[Polycythemia vera|polycythemia vera (PV)]], † [[Chronic myelogenous leukemia|chronic myelogenous leukemia (CML)]], ‡ [[Myelodysplastic syndrome|myelodysplastic syndrome (MDS)]], § [[essential thrombocythemia]] or other [[myeloid]] [[neoplasm]] | ||
#Demonstration of JAK2617V>F or other clonal marker | #Demonstration of JAK2617V>F, CALR mutation, MPL mutation, or other clonal marker, or in the absence of a clonal marker, no evidence of [[bone marrow]] [[fibrosis]] due to underlying [[Inflammation|inflammatory]] or other [[neoplastic diseases]]¶ | ||
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| style="padding: 5px 5px; background: #DCDCDC;" |'''Minor criteria''' | | style="padding: 5px 5px; background: #DCDCDC;" |'''Minor criteria''' | ||
Revision as of 03:45, 22 December 2018
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Myelofibrosis Microchapters |
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Diagnosis |
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Myelofibrosis diagnostic study of choice On the Web |
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American Roentgen Ray Society Images of Myelofibrosis diagnostic study of choice |
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Risk calculators and risk factors for Myelofibrosis diagnostic study of choice |
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
Diagnosis of myelofibrosis may be made based upon a thorough clinical evaluation, detailed patient history, and specialized tests. The World Health Organization (WHO) has set the criteria for diagnosing primary myelofibrosis (PMF). It has determined set rules for distinguishing the prefibrotic/early (pre-primary myelofibrosis) phase and the overtly fibrotic (overt primary myelofibrosis) phase. The World Health Organization (WHO) has also introduced a proposed revised criteria for primary myelofibrosis (PMF).
Diagnostic Criteria
2001 World Health Organization (WHO) criteria for prefibrotic/early (pre-primary myelofibrosis) phase
| Clinical findings | Morphological findings |
|---|---|
Spleen and liver
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Blood
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Hematology (variable)
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Bone marrow
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2001 World Health Organization (WHO) criteria for overtly fibrotic (overt primary myelofibrosis) phase
| Clinical findings | Morphological findings |
|---|---|
Spleen and liver
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Blood
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Hematology (variable)
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Bone marrow
*Clustering of megakaryocytes, abnormally lobulated megakaryocytic nuclei, naked megakaryocytic nuclei |
Proposed revised 2016 World Health Organization (WHO) criteria for primary myelofibrosis (PMF)
| Clinical findings |
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Major criteria
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Minor criteria
*Small to large megakaryocytes with an aberrant nuclear/cytoplasmic ratio and hyperchromatic, bulbous, or irregularly folded nuclei and dense clustering. †Requires the failure of iron replacement therapy to increase hemoglobin level to the polycythemia vera (PV) range in the presence of decreased serum ferritin. Exclusion of polycythemia vera (PV) is based on hemoglobin and hematocrit levels. Red cell mass measurement is not required. ‡Requires the absence of BCR/ABL. §Requires the absence of dyserythropoiesis and dysgranulopoiesis. ¶Secondary to infection, autoimmune disorder or other chronic inflammatory condition, hairy cell leukemia or other lymphoid neoplasm, metastatic malignancy, or toxic (chronic) myelopathies. It should be noted that patients with conditions associated with reactive myelofibrosis are not immune to primary myelofibrosis and the diagnosis should be considered in such cases if other criteria are met. ∥Degree of abnormality could be borderline or marked. |
References
- ↑ 1.0 1.1 1.2 Mesa RA, Verstovsek S, Cervantes F, Barosi G, Reilly JT, Dupriez B, Levine R, Le Bousse-Kerdiles MC, Wadleigh M, Campbell PJ, Silver RT, Vannucchi AM, Deeg HJ, Gisslinger H, Thomas D, Odenike O, Solberg LA, Gotlib J, Hexner E, Nimer SD, Kantarjian H, Orazi A, Vardiman JW, Thiele J, Tefferi A (June 2007). "Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): Consensus on terminology by the international working group for myelofibrosis research and treatment (IWG-MRT)". Leuk. Res. 31 (6): 737–40. doi:10.1016/j.leukres.2006.12.002. PMID 17210175.
- ↑ 2.0 2.1 2.2 Tefferi, A.; Thiele, J.; Orazi, A.; Kvasnicka, H. M.; Barbui, T.; Hanson, C. A.; Barosi, G.; Verstovsek, S.; Birgegard, G.; Mesa, R.; Reilly, J. T.; Gisslinger, H.; Vannucchi, A. M.; Cervantes, F.; Finazzi, G.; Hoffman, R.; Gilliland, D. G.; Bloomfield, C. D.; Vardiman, J. W. (2007). "Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel". Blood. 110 (4): 1092–1097. doi:10.1182/blood-2007-04-083501. ISSN 0006-4971.
- ↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.