Myeloproliferative neoplasm classification: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Myeloproliferative disease}} | {{Myeloproliferative disease}} | ||
{{CMG}}{{AE}} {{MJK}} {{shyam}} | {{CMG}}{{AE}} {{MJK}}, {{shyam}} | ||
==Overview== | ==Overview== | ||
Myeloproliferative neoplasm may be classified according to the World Health Organization into eight subtypes: [[polycythemia vera]], [[essential thrombocythemia]], [[primary myelofibrosis]], [[chronic myelogenous leukemia]], [[chronic neutrophilic leukemia]], [[chronic eosinophilic leukemia]], myeloproliferative neoplasms unclassifiable, and [[mastocytosis]]. Each subtypes is based on a distinct malignant cell, and each subtype has different criteria for diagnosis. | Myeloproliferative neoplasm may be classified according to the World Health Organization into eight subtypes: [[polycythemia vera]], [[essential thrombocythemia]], [[primary myelofibrosis]], [[chronic myelogenous leukemia]], [[chronic neutrophilic leukemia]], [[chronic eosinophilic leukemia]], myeloproliferative neoplasms unclassifiable, and [[mastocytosis]]. Each subtypes is based on a distinct [[Malignant|malignant cell]], and each subtype has different criteria for diagnosis. | ||
==Classification== | ==Classification== | ||
Myeloproliferative neoplasm may be classified according to the World Health Organization into eight subtypes as follows:<ref name="pmid27069254">{{cite journal| author=Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM et al.| title=The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. | journal=Blood | year= 2016 | volume= 127 | issue= 20 | pages= 2391-405 | pmid=27069254 | doi=10.1182/blood-2016-03-643544 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27069254 }} </ref><ref name="pmid19357394">{{cite journal| author=Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A et al.| title=The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. | journal=Blood | year= 2009 | volume= 114 | issue= 5 | pages= 937-51 | pmid=19357394 | doi=10.1182/blood-2009-03-209262 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19357394 }} </ref><ref name="pmid28254862">{{cite journal| author=Valent P, Akin C, Hartmann K, Nilsson G, Reiter A, Hermine O et al.| title=Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future. | journal=Cancer Res | year= 2017 | volume= 77 | issue= 6 | pages= 1261-1270 | pmid=28254862 | doi=10.1158/0008-5472.CAN-16-2234 | pmc=5354959 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28254862 }} </ref> | |||
{| | |||
! style="background: #4479BA; " | {{fontcolor|#FFF|Disease}} | |||
! style="background: #4479BA; " | {{fontcolor|#FFF|Cell of origin}} | |||
! style="background: #4479BA; " | {{fontcolor|#FFF|W.H.O. Diagnostic criteria}} | |||
|- | |- | ||
! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | | |||
Polycythemia vera | [[Polycythemia vera]] | ||
| style=" | | align="center" style="background:#F5F5F5;" + | | ||
Erythroid precursor | Erythroid precursor | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
''Major criteria'': | ''Major criteria'': | ||
*Hemoglobin > 16.5 g/dl in men or hemoglobin > 16 g/dl in women | *[[Hemoglobin]] > 16.5 g/dl in men or [[hemoglobin]] > 16 g/dl in women | ||
*Bone marrow biopsy showing | *[[Bone marrow biopsy]] showing hypercellularity for age and trilineage growth (panmyelosis) | ||
*Presence of ''JAK2'' ''V617F'' or exon 12 mutation | *Presence of ''[[Janus kinase|JAK2]]'' ''V617F'' or exon 12 [[mutation]] | ||
''Minor criterion'': | ''Minor criterion'': | ||
*Subnormal erythropoietin level | *Subnormal [[erythropoietin]] level | ||
Diagnosis requires meeting all 3 major criterion or the top 2 major plus the 1 minor criterion | Diagnosis requires meeting all 3 major criterion or the top 2 major plus the 1 minor criterion | ||
|- | |- | ||
! style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" | | |||
Essential thrombocythemia | [[Essential thrombocythemia]] | ||
| style=" | | align="center" style="background:#F5F5F5;" + | | ||
Megakaryocyte | [[Megakaryocyte]] | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
''Major criteria'': | ''Major criteria'': | ||
*Platelet count > 450,000 per microliter | *[[Platelet]] count > 450,000 per microliter | ||
*Bone marrow biopsy showing mainly proliferation of megakaryocytes with increased number of enlarged and mature megakaryocytes | *[[Bone marrow examination|Bone marrow biopsy]] showing mainly the proliferation of [[Megakaryocyte|megakaryocytes]] with an increased number of enlarged and mature [[Megakaryocyte|megakaryocytes]] | ||
*Not meeting criteria for other myeloproliferative neoplasms | *Not meeting criteria for other myeloproliferative neoplasms | ||
*Presence of ''JAK2'', ''CALR'', or ''MPL'' mutation | *Presence of ''[[JAK2]]'', ''CALR'', or ''MPL'' [[mutation]] | ||
''Minor criterion'': | ''Minor criterion'': | ||
*Presence of a clonal marker or absence of reactive thrombocytosis | *Presence of a clonal marker or absence of reactive [[thrombocytosis]] | ||
Diagnosis requires meeting all 4 major criteria or the first 3 major plus the 1 minor criterion | Diagnosis requires meeting all 4 major criteria or the first 3 major plus the 1 minor criterion | ||
|- | |- | ||
! style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" | | |||
Primary myelofibrosis | [[Primary myelofibrosis]] | ||
| style=" | | align="center" style="background:#F5F5F5;" + | | ||
Megakaryocyte | [[Megakaryocyte]] | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
''Major criteria'': | ''Major criteria'': | ||
*Presence of megakaryocyte proliferation and atypia with reticulin fibrosis | *Presence of [[megakaryocyte]] proliferation and atypia with reticulin fibrosis | ||
*Not meeting criteria for other myeloproliferative neoplasms | *Not meeting criteria for other myeloproliferative neoplasms | ||
*Presence of ''JAK2'', ''CALR'', or ''MPL'' mutation | *Presence of ''[[Janus kinase|JAK2]]'', ''CALR'', or ''MPL'' [[mutation]] | ||
''Minor criteria'': | ''Minor criteria'': | ||
*Anemia | *[[Anemia]] | ||
*White blood cell count >11,000 per microliter | *[[White blood cells|White blood cell]] count >11,000 per microliter | ||
*Palpable splenomegaly | *Palpable [[splenomegaly]] | ||
*Elevated LDH | *Elevated [[Lactate dehydrogenase|LDH]] | ||
*Leukoerythroblastic smear | *Leukoerythroblastic smear | ||
Diagnosis requires meeting all major criteria and at least 1 minor criterion | Diagnosis requires meeting all major criteria and at least 1 minor criterion | ||
|- | |- | ||
! style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" | | |||
Chronic myeloid leukemia | [[Chronic myeloid leukemia]] | ||
| align="center" style="background:#F5F5F5;" + | | |||
Common [[myeloid]] progenitor | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
*Presence of [[BCR/ABL|BCR-ABL translocation]] ([[Chromosome|chromosomes]] 9 and 22) | |||
*Presence of BCR-ABL translocation (chromosomes 9 and 22) | |||
|- | |- | ||
! style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" | | |||
Chronic neutrophilic leukemia | [[Chronic neutrophilic leukemia]] | ||
| align="center" style="background:#F5F5F5;" + |[[Neutrophil]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
*Peripheral blood [[White blood cells|white blood cell]] count > 25,000 per microliter with rare [[Myeloblast|myeloblasts]] and no dysgranulopoiesis | |||
*[[Bone marrow]] hypercellularity with increased [[Granulocyte|granulocytes]] and normal maturation and <5% [[Myeloblast|myeloblasts]] | |||
*Peripheral blood white blood cell count > 25,000 per microliter with rare myeloblasts and no dysgranulopoiesis | |||
*Bone marrow hypercellularity with increased granulocytes and normal maturation and <5% myeloblasts | |||
*Not meeting criteria for other myeloproliferative neoplasms | *Not meeting criteria for other myeloproliferative neoplasms | ||
*Absence of genetic rearrangements of ''PDGFRA'', ''PDGFRB'', ''FGFR1'', or ''PCM1-JAK2'' | *Absence of genetic rearrangements of ''PDGFRA'', ''[[PDGFRB]]'', ''[[Fibroblast growth factor receptor 1|FGFR1]]'', or ''PCM1-[[Janus kinase|JAK2]]'' | ||
*Presence of ''CSF3R'' ''T618I'' or other characteristic mutation | *Presence of ''[[CSF3R]]'' ''T618I'' or other characteristic [[mutation]] | ||
|- | |- | ||
! style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" | | |||
Chronic eosinophilic leukemia | [[Chronic eosinophilic leukemia]] | ||
| style=" | | align="center" style="background:#F5F5F5;" + |[[Eosinophil granulocyte|Eosinophil]] | ||
Eosinophil | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
No formal W.H.O. criteria | No formal W.H.O. criteria | ||
*Typically associated with >1,500 eosinophils per microliter in peripheral blood | *Typically associated with >1,500 [[Eosinophil granulocyte|eosinophils]] per microliter in [[Venous blood|peripheral blood]] | ||
*Typically associated with rearrangements of ''PDGFRA'', ''PDGFRB'', ''FGFR1'', ''JAK2'' | *Typically associated with rearrangements of ''PDGFRA'', ''[[PDGFRB]]'', ''[[Fibroblast growth factor receptor 1|FGFR1]]'', ''[[Janus kinase|JAK2]]'' | ||
|- | |- | ||
! style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" | | |||
Myeloproliferative neoplasm, unclassifiable | [[Myeloproliferative neoplasm, unclassifiable]] | ||
| style=" | | align="center" style="background:#F5F5F5;" + |Variable | ||
Variable | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
Not meeting criteria for other subcategories | Not meeting criteria for other subcategories | ||
|- | |- | ||
! style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" | | |||
Mastocytosis | [[Mastocytosis]] | ||
| style=" | | align="center" style="background:#F5F5F5;" + |[[Mast cell]] | ||
Mast cell | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
''Major criteria'': | ''Major criteria'': | ||
*Dense multifocal aggregates of >15 mast cells in bone marrow or other | *Dense multifocal aggregates of >15 [[Mast cell|mast cells]] in [[bone marrow]] or other organs | ||
''Minor criteria'': | ''Minor criteria'': | ||
*Presence of '' | *Presence of ''[[C-kit]] D816V'' [[mutation]] | ||
*Expression of CD2, CD25, or both on mast cells | *Expression of [[CD2]], [[CD25]], or both on [[Mast cell|mast cells]] | ||
*Serum tryptase level >20ng/ml when patient is at baseline health | *Serum [[tryptase]] level >20ng/ml when a patient is at baseline health | ||
*Atypical morphology or spindles in >25% of mast cells in bone marrow or other | *Atypical morphology or spindles in >25% of [[Mast cell|mast cells]] in [[bone marrow]] or other organs | ||
Diagnosis requires meeting the one major plus one minor criterion, or 3 minor criteria | Diagnosis requires meeting the one major plus one minor criterion, or 3 minor criteria | ||
|} | |} | ||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
[[Category: | |||
[[Category:Medicine]] | |||
[[Category:Hematology]] | [[Category:Hematology]] | ||
[[Category:Oncology]] | [[Category:Oncology]] | ||
[[Category: | [[Category:Up-To-Date]] | ||
Latest revision as of 22:51, 29 July 2020
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Myeloproliferative Neoplasm Microchapters |
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Differentiating myeloproliferative neoplasm from other Diseases |
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Risk calculators and risk factors for Myeloproliferative neoplasm classification |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2], Shyam Patel [3]
Overview
Myeloproliferative neoplasm may be classified according to the World Health Organization into eight subtypes: polycythemia vera, essential thrombocythemia, primary myelofibrosis, chronic myelogenous leukemia, chronic neutrophilic leukemia, chronic eosinophilic leukemia, myeloproliferative neoplasms unclassifiable, and mastocytosis. Each subtypes is based on a distinct malignant cell, and each subtype has different criteria for diagnosis.
Classification
Myeloproliferative neoplasm may be classified according to the World Health Organization into eight subtypes as follows:[1][2][3]
| Disease | Cell of origin | W.H.O. Diagnostic criteria |
|---|---|---|
|
Erythroid precursor |
Major criteria:
Minor criterion:
Diagnosis requires meeting all 3 major criterion or the top 2 major plus the 1 minor criterion | |
|
Major criteria:
Minor criterion:
Diagnosis requires meeting all 4 major criteria or the first 3 major plus the 1 minor criterion | ||
|
Major criteria:
Minor criteria:
Diagnosis requires meeting all major criteria and at least 1 minor criterion | ||
|
Common myeloid progenitor |
| |
| Neutrophil |
| |
| Eosinophil |
No formal W.H.O. criteria
| |
| Variable |
Not meeting criteria for other subcategories | |
| Mast cell |
Major criteria:
Minor criteria:
Diagnosis requires meeting the one major plus one minor criterion, or 3 minor criteria |
References
- ↑ Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM; et al. (2016). "The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia". Blood. 127 (20): 2391–405. doi:10.1182/blood-2016-03-643544. PMID 27069254.
- ↑ Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A; et al. (2009). "The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes". Blood. 114 (5): 937–51. doi:10.1182/blood-2009-03-209262. PMID 19357394.
- ↑ Valent P, Akin C, Hartmann K, Nilsson G, Reiter A, Hermine O; et al. (2017). "Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future". Cancer Res. 77 (6): 1261–1270. doi:10.1158/0008-5472.CAN-16-2234. PMC 5354959. PMID 28254862.