Myeloproliferative neoplasm classification: Difference between revisions

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{{Myeloproliferative disease}}
{{Myeloproliferative disease}}
 
{{CMG}}{{AE}} {{MJK}}, {{shyam}}
{{CMG}}
==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|malignant cell]], and each subtype has different criteria for diagnosis.
==Classification==
==Classification==
Although not a [[cancer|malignant neoplasm]], MPDs are classified within the [[Hematological malignancy|hematological neoplasms]].
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>
 
{|
There are four main myeloproliferative diseases, which can be further categorized by the presence of the [[Philadelphia chromosome]]:
! style="background: #4479BA; " | {{fontcolor|#FFF|Disease}}
 
! style="background: #4479BA; " | {{fontcolor|#FFF|Cell of origin}}
{| class="wikitable"
! style="background: #4479BA; " | {{fontcolor|#FFF|W.H.O. Diagnostic criteria}}
|-
! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
[[Polycythemia vera]]
| align="center" style="background:#F5F5F5;" + |
Erythroid precursor
| style="padding: 5px 5px; background: #F5F5F5;" |
''Major criteria'':
*[[Hemoglobin]] > 16.5 g/dl in men or [[hemoglobin]] > 16 g/dl in women
*[[Bone marrow biopsy]] showing hypercellularity for age and trilineage growth (panmyelosis)
*Presence of ''[[Janus kinase|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
|-
! style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
[[Essential thrombocythemia]]
| align="center" style="background:#F5F5F5;" + |
[[Megakaryocyte]]
| style="padding: 5px 5px; background: #F5F5F5;" |
''Major criteria'':
*[[Platelet]] count > 450,000 per microliter
*[[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
*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
|-
! style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
[[Primary myelofibrosis]]
| align="center" style="background:#F5F5F5;" + |
[[Megakaryocyte]]
| style="padding: 5px 5px; background: #F5F5F5;" |
''Major criteria'':
*Presence of [[megakaryocyte]] proliferation and atypia with reticulin fibrosis
*Not meeting criteria for other myeloproliferative neoplasms
*Presence of ''[[Janus kinase|JAK2]]'', ''CALR'', or ''MPL'' [[mutation]]
''Minor criteria'':
*[[Anemia]]
*[[White blood cells|White blood cell]] count >11,000 per microliter
*Palpable [[splenomegaly]]
*Elevated [[Lactate dehydrogenase|LDH]]
*Leukoerythroblastic smear
Diagnosis requires meeting all major criteria and at least 1 minor criterion
|-
! style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
[[Chronic myeloid leukemia]]
| align="center" style="background:#F5F5F5;" + |
Common [[myeloid]] progenitor
| style="padding: 5px 5px; background: #F5F5F5;" |
*Presence of [[BCR/ABL|BCR-ABL translocation]] ([[Chromosome|chromosomes]] 9 and 22)
|-
! style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
[[Chronic neutrophilic leukemia]]
| align="center" style="background:#F5F5F5;" + |[[Neutrophil]]
| 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]]
*Not meeting criteria for other myeloproliferative neoplasms
*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]]
|-
|-
! Philadelphia Chromosome "positive"
! style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
! Philadelphia Chromosome "negative"
[[Chronic eosinophilic leukemia]]
| align="center" style="background:#F5F5F5;" + |[[Eosinophil granulocyte|Eosinophil]]
| style="padding: 5px 5px; background: #F5F5F5;" |
No formal W.H.O. criteria
*Typically associated with >1,500 [[Eosinophil granulocyte|eosinophils]] per microliter in [[Venous blood|peripheral blood]]
*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" |
* [[Chronic myelogenous leukemia]] (CML)
[[Myeloproliferative neoplasm, unclassifiable]]
|  
| align="center" style="background:#F5F5F5;" + |Variable
* [[Polycythemia vera]] (PV)
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Essential thrombocytosis]] (ET)
Not meeting criteria for other subcategories
* [[Myelofibrosis]] (MF)
|-
|-
! style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
[[Mastocytosis]]
| align="center" style="background:#F5F5F5;" + |[[Mast cell]]
| style="padding: 5px 5px; background: #F5F5F5;" |
''Major criteria'':
*Dense multifocal aggregates of >15 [[Mast cell|mast cells]] in [[bone marrow]] or other organs
''Minor criteria'':
*Presence of ''[[C-kit]] D816V'' [[mutation]]
*Expression of [[CD2]], [[CD25]], or both on [[Mast cell|mast cells]]
*Serum [[tryptase]] level >20ng/ml when a patient is at baseline health
*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
|}


|}
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
[[Category:Hematology]]
[[Category:Needs overview]]
[[Category: Disease]]


{{WH}}
[[Category:Medicine]]
{{WS}}
[[Category:Hematology]]
[[Category:Oncology]]
[[Category:Up-To-Date]]

Latest revision as of 22:51, 29 July 2020

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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

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

Polycythemia vera

Erythroid precursor

Major criteria:

Minor criterion:

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

Essential thrombocythemia

Megakaryocyte

Major criteria:

Minor criterion:

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:

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

Chronic myeloid leukemia

Common myeloid progenitor

Chronic neutrophilic leukemia

Neutrophil

Chronic eosinophilic leukemia

Eosinophil

No formal W.H.O. criteria

Myeloproliferative neoplasm, unclassifiable

Variable

Not meeting criteria for other subcategories

Mastocytosis

Mast cell

Major criteria:

Minor criteria:

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.
  2. 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.
  3. 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.