Myeloproliferative neoplasm classification: Difference between revisions

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==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, [[mastocytosis]].<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>
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==
==Classification==



Revision as of 06:27, 24 June 2018

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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

Disease Cell of origin W.H.O. Diagnostic criteria[1]

Polycythemia vera

Erythroid precursor

Major criteria:

  • Hemoglobin > 16.5 g/dl in men or hemoglobin > 16 g/dl in women
  • Bone marrow biopsy showing hypercellularly for age and trilineage growth (panmyelosis)
  • Presence of JAK2 V617F or exon 12 mutation

Minor criterion:

  • Subnormal erythropoietin level

Diagnosis requires meeting all 3 major criterion or the top 2 major plus the 1 minor criterion

Essential thrombocythemia

Megakaryocyte

Major criteria:

  • Platelet count > 450,000 per microliter
  • Bone marrow biopsy showing mainly proliferation of megakaryocytes with increased number of enlarged and mature megakaryocytes
  • Not meeting criteria for other myeloproliferative neoplasms
  • Presence of JAK2, CALR, or MPL mutation

Minor criterion:

  • 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.

Primary myelofibrosis

Megakaryocyte

Major criteria:

  • Presence of megakaryocyte proliferation and atypia with reticulin fibrosis
  • Not meeting criteria for other myeloproliferative neoplasms
  • Presence of JAK2, CALR, or MPL mutation

Minor criteria:

  • Anemia
  • White blood cell count >11,000 per microliter.
  • Palpable splenomegaly
  • Elevated LDH
  • Leukoerythroblastic smear

Diagnosis requires meeting all major criteria and at least 1 minor criterion.

Chronic myeloid leukemia

Common myeloid progenitor

  • Presence of BCR-ABL translocation (chromosomes 9 and 22)

Chronic neutrophilic leukemia

Neutrophil

  • 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
  • Absence of genetic rearrangements of PDGFRA, PDGFRB, FGFR1, or PCM1-JAK2
  • Presence of CSF3R T618I or other characteristic mutation

Chronic eosinophilic leukemia

Eosinophil

No formal W.H.O. criteria

  • Typically associated with >1,500 eosinophils per microliter in peripheral blood
  • Typically associated with rearrangements of PDGFRA, PDGFRB, FGFR1, JAK2

Myeloproliferative neoplasm, unclassifiable

Variable

Not meeting criteria for other subcategories

Mastocytosis

Mast cell

Major criteria:

  • Dense multifocal aggregates of >15 mast cells in bone marrow or other organ

Minor criteria:

  • Presence of c-kit D816Vmutation
  • Expression of CD2, CD25, or both on mast cells
  • Serum tryptase level >20ng/ml when patient is at baseline health
  • Atypical morphology or spindles in >25% of mast cells in bone marrow or other organ

Diagnosis requires meeting the one major plus one minor criterion, or 3 minor criteria.

References

  1. 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.