Myelofibrosis diagnostic study of choice: Difference between revisions

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==Overview==
==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).
[[Diagnosis]] of [[myelofibrosis]] may be made based upon a thorough clinical evaluation, detailed [[patient history]], and specialized [[Test|tests]]. The [[World Health Organization|World Health Organization (WHO)]] has set the criteria for [[Diagnosis|diagnosing]] [[Primary myelofibrosis|primary myelofibrosis (PMF)]]. It has determined set rules for distinguishing the [[Prefibrotic primary myelofibrosis|prefibrotic/early (pre-primary myelofibrosis)]] phase and the overtly fibrotic (overt [[primary myelofibrosis]]) phase. The [[World Health Organization|World Health Organization (WHO)]] has also introduced a proposed revised criteria for [[Primary myelofibrosis|primary myelofibrosis (PMF)]].


==Diagnostic Criteria==
==Diagnostic Criteria==
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|-
|-
| style="padding: 5px 5px; background: #DCDCDC;" |'''Spleen and liver'''
| style="padding: 5px 5px; background: #DCDCDC;" |'''Spleen and liver'''
*No or mild splenomegaly or hepatomegaly
*No or mild [[splenomegaly]] or [[hepatomegaly]]
| style="padding: 5px 5px; background: #DCDCDC;" |'''Blood'''
| style="padding: 5px 5px; background: #DCDCDC;" |'''Blood'''
*No or mild leukoerythroblastosis
*No or mild leukoerythroblastosis
*No or mild red blood cell poikilocytosis
*No or mild [[red blood cell]] [[poikilocytosis]]
*Few if any dacryocytes
*Few if any dacryocytes
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;" |'''Hematology (variable)'''
| style="padding: 5px 5px; background: #DCDCDC;" |'''Hematology (variable)'''
*Mild anemia
*Mild [[anemia]]
*Mild to moderate leukocytosis
*Mild to moderate [[leukocytosis]]
*Mild to marked thrombocytosis
*Mild to marked [[thrombocytosis]]
| style="padding: 5px 5px; background: #DCDCDC;" |'''Bone marrow'''
| style="padding: 5px 5px; background: #DCDCDC;" |'''Bone marrow'''
*Hypercellularity
*Hypercellularity
*Neutrophilic proliferation
*[[Neutrophil|Neutrophilic]] proliferation
*Megakaryocytic proliferation
*[[Megakaryocyte|Megakaryocytic]] proliferation
|}<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>


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|-
|-
| style="padding: 5px 5px; background: #DCDCDC;" |'''Spleen and liver'''
| style="padding: 5px 5px; background: #DCDCDC;" |'''Spleen and liver'''
*Moderate to marked splenomegaly or hepatomegaly
*Moderate to marked [[splenomegaly]] or [[hepatomegaly]]
| style="padding: 5px 5px; background: #DCDCDC;" |'''Blood'''
| style="padding: 5px 5px; background: #DCDCDC;" |'''Blood'''
*Leukoerythroblastosis
*Leukoerythroblastosis
*Prominent red blood cell poikilocytosis
*Prominent [[red blood cell]] [[poikilocytosis]]
*Prominent dacryocytosis
*Prominent dacryocytosis
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;" |'''Hematology (variable)'''
| style="padding: 5px 5px; background: #DCDCDC;" |'''Hematology (variable)'''
*Leukoerythroblastosis
*Leukoerythroblastosis
*White blood cells decreased to elevated
*[[White blood cells]] decreased to elevated
*Platelet count decreased to elevated
*[[Platelet]] count decreased to elevated
| style="padding: 5px 5px; background: #DCDCDC;" |'''Bone marrow'''
| style="padding: 5px 5px; background: #DCDCDC;" |'''Bone marrow'''
*Reticulin and/or collagen fibrosis
*[[Reticulin]] and/or [[collagen]] [[fibrosis]]
*Decreased cellularity
*Decreased cellularity
*Dilated marrow sinuses
*Dilated [[Bone marrow|marrow]] sinuses
*Intraluminal hematopoiesis
*[[Intraluminal]] [[hematopoiesis]]
*Neutrophilic proliferation
*[[Neutrophil|Neutrophilic]] proliferation
*Prominent megakaryocytic proliferation
*Prominent [[Megakaryocyte|megakaryocytic]] proliferation
*Megakaryocytic atypia*
*[[Megakaryocyte|Megakaryocytic]] atypia*
*New bone formation (osteosclerosis)
*New [[bone]] formation ([[osteosclerosis]])
<sub>*Clustering of megakaryocytes, abnormally lobulated megakaryocytic nuclei, naked megakaryocytic nuclei</sub>
<sub>*Clustering of [[Megakaryocyte|megakaryocytes]], abnormally lobulated [[Megakaryocyte|megakaryocytic]] [[Cell nucleus|nuclei]], naked [[Megakaryocyte|megakaryocytic]] [[Cell nucleus|nuclei]]</sub>
|}<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>


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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 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 WHO criteria for PV, †CML, ‡ 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)]], § or other [[myeloid]] [[neoplasm]]
#Demonstration of JAK2617V>F or other clonal marker (eg, MPL515W>L/K), or in the absence of a clonal marker, no evidence of bone marrow fibrosis due to underlying inflammatory or other neoplastic diseases¶
#Demonstration of JAK2617V>F or other clonal marker (eg, MPL515W>L/K), or in the absence of a clonal marker, no evidence of [[bone marrow]] [[fibrosis]] due to underlying [[Inflammation|inflammatory]] or other [[neoplastic diseases]]¶
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;" |'''Minor criteria'''
| style="padding: 5px 5px; background: #DCDCDC;" |'''Minor criteria'''
#Leukoerythroblastosis∥
#Leukoerythroblastosis∥
#Increase in serum lactate dehydrogenase level∥
#Increase in serum [[Lactate dehydrogenase|lactate dehydrogenase (LDH)]] level∥
#Anemia∥
#[[Anemia]]∥
#Palpable splenomegaly∥
#Palpable [[splenomegaly]]∥
<sub>*Small to large megakaryocytes with an aberrant nuclear/cytoplasmic ratio and hyperchromatic, bulbous, or irregularly folded nuclei and dense clustering.</sub>
<sub>*Small to large [[Megakaryocyte|megakaryocytes]] with an aberrant [[Cell nucleus|nuclear]]/[[Cytoplasm|cytoplasmic]] ratio and [[Hyperchromicity|hyperchromatic]], bulbous, or irregularly folded [[Cell nucleus|nuclei]] and dense clustering.</sub>


<sub>†Requires the failure of iron replacement therapy to increase hemoglobin level to the polycythemia vera range in the presence of decreased serum ferritin. Exclusion of polycythemia vera is based on hemoglobin and hematocrit levels. Red cell mass measurement is not required.</sub>
<sub>†Requires the failure of [[iron]] replacement therapy to increase [[hemoglobin]] level to the [[Polycythemia vera|polycythemia vera (PV)]] range in the presence of decreased serum [[ferritin]]. Exclusion of [[Polycythemia vera|polycythemia vera (PV)]] is based on [[hemoglobin]] and [[hematocrit]] levels. [[Red blood cell|Red cell]] mass measurement is not required.</sub>


<sub>‡Requires the absence of BCR-ABL.</sub>
<sub>‡Requires the absence of [[BCR/ABL]].</sub>


<sub>§Requires the absence of dyserythropoiesis and dysgranulopoiesis.</sub>
<sub>§Requires the absence of [[dyserythropoiesis]] and dysgranulopoiesis.</sub>


<sub>¶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.</sub>
<sub>¶Secondary to [[infection]], [[Autoimmunity|autoimmune disorder]] or other [[Chronic (medical)|chronic]] [[Inflammation|inflammatory]] condition, [[hairy cell leukemia]] or other [[lymphoid]] [[neoplasm]], [[Metastasis|metastatic]] [[Cancer|malignancy]], or [[toxic]] ([[Chronic (medical)|chronic]]) [[Myelopathy|myelopathies]]. It should be noted that patients with conditions associated with reactive [[myelofibrosis]] are not [[Immunity (medical)|immune]] to [[primary myelofibrosis]] and the [[diagnosis]] should be considered in such cases if other criteria are met.</sub>


<sub>∥Degree of abnormality could be borderline or marked.</sub>
<sub>∥Degree of abnormality could be borderline or marked.</sub>
|}
|}
*Diagnosis requires meeting all 3 major criteria and 2 minor criteria.<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>{{cite book | last = Hoffman | first = Ronald | title = Hematology : basic principles and practice | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 9780323357623 }}</ref>
*[[Diagnosis]] requires meeting all 3 major criteria and 2 minor criteria.<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>{{cite book | last = Hoffman | first = Ronald | title = Hematology : basic principles and practice | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 9780323357623 }}</ref>


==References==
==References==

Revision as of 20:33, 11 December 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, 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 Blood
Hematology (variable) Bone marrow

[1][2]

2001 World Health Organization (WHO) criteria for overtly fibrotic (overt primary myelofibrosis) phase
Clinical findings Morphological findings
Spleen and liver Blood
Hematology (variable) Bone marrow

*Clustering of megakaryocytes, abnormally lobulated megakaryocytic nuclei, naked megakaryocytic nuclei

[1][2]

Proposed revised World Health Organization (WHO) criteria for primary myelofibrosis (PMF)
Clinical findings
Major criteria
  1. 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 granulocytic proliferation and often decreased erythropoiesis (ie, prefibrotic cellular-phase disease)
  2. Not meeting World Health Organization (WHO) criteria for polycythemia vera (PV), † chronic myelogenous leukemia (CML), ‡ myelodysplastic syndrome (MDS), § or other myeloid neoplasm
  3. Demonstration of JAK2617V>F or other clonal marker (eg, MPL515W>L/K), or in the absence of a clonal marker, no evidence of bone marrow fibrosis due to underlying inflammatory or other neoplastic diseases
Minor criteria
  1. Leukoerythroblastosis∥
  2. Increase in serum lactate dehydrogenase (LDH) level∥
  3. Anemia∥
  4. Palpable splenomegaly∥

*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. ↑ 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. ↑ 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.
  3. ↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.

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