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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 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==
==Diagnostic Criteria==
===2001 World Health Organization (WHO) criteria for prefibrotic/early (pre-primary myelofibrosis) phase===
===2001 World Health Organization (WHO) criteria for prefibrotic/early (pre-primary myelofibrosis) phase===
{| style="border: 0px; font-size: 90%; margin: 3px; width:650px"
{| style="border: 0px; font-size: 90%; margin: 3px; width:650px"
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| valign="top" |
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|+
! style="background: #4479BA; width: 340px;" | {{fontcolor|#FFF|Clinical findings}}
! style="background: #4479BA; width: 340px;" | {{fontcolor|#FFF|Clinical findings}}
! style="background: #4479BA; width: 340px;" | {{fontcolor|#FFF|Morphological findings}}
! style="background: #4479BA; width: 340px;" | {{fontcolor|#FFF|Morphological findings}}
|-
|-
| 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'''
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*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
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===2001 World Health Organization (WHO) criteria for overtly fibrotic (overt primary myelofibrosis) phase===
===2001 World Health Organization (WHO) criteria for overtly fibrotic (overt primary myelofibrosis) phase===
{| style="border: 0px; font-size: 90%; margin: 3px; width:650px"
{| style="border: 0px; font-size: 90%; margin: 3px; width:650px"
|valign=top|
| valign="top" |
|+
|+
! style="background: #4479BA; width: 340px;" | {{fontcolor|#FFF|Clinical findings}}
! style="background: #4479BA; width: 340px;" | {{fontcolor|#FFF|Clinical findings}}
! style="background: #4479BA; width: 340px;" | {{fontcolor|#FFF|Morphological findings}}
! style="background: #4479BA; width: 340px;" | {{fontcolor|#FFF|Morphological findings}}
|-
|-
| 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'''
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*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
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===Proposed revised World Health Organization (WHO) criteria for primary myelofibrosis (PMF)===  
===Proposed revised World Health Organization (WHO) criteria for primary myelofibrosis (PMF)===  
{| style="border: 0px; font-size: 90%; margin: 3px; width:650px"
{| style="border: 0px; font-size: 90%; margin: 3px; width:650px"
|valign=top|
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|+
! style="background: #4479BA; width: 340px;" | {{fontcolor|#FFF|Clinical findings}}
! style="background: #4479BA; width: 340px;" | {{fontcolor|#FFF|Clinical findings}}
|-
|-
| 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 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 WHO criteria for PV, †CML, ‡ 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 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 level∥

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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
  • No or mild splenomegaly or hepatomegaly
 Blood
  • No or mild leukoerythroblastosis
  • No or mild red blood cell poikilocytosis
  • Few if any dacryocytes
Hematology (variable)
  • Mild anemia
  • Mild to moderate leukocytosis
  • Mild to marked thrombocytosis
 Bone marrow
  • Hypercellularity
  • Neutrophilic proliferation
  • Megakaryocytic proliferation

[1][2]

2001 World Health Organization (WHO) criteria for overtly fibrotic (overt primary myelofibrosis) phase
Clinical findings Morphological findings
Spleen and liver
  • Moderate to marked splenomegaly or hepatomegaly
 Blood
  • Leukoerythroblastosis
  • Prominent red blood cell poikilocytosis
  • Prominent dacryocytosis
Hematology (variable)
  • Leukoerythroblastosis
  • White blood cells decreased to elevated
  • Platelet count decreased to elevated
 Bone marrow
  • Reticulin and/or collagen fibrosis
  • Decreased cellularity
  • Dilated marrow sinuses
  • Intraluminal hematopoiesis
  • Neutrophilic proliferation
  • Prominent megakaryocytic proliferation
  • Megakaryocytic atypia*
  • New bone formation (osteosclerosis)

*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 WHO criteria for PV, †CML, ‡ 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 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 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.

‡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.

  • Diagnosis requires meeting all 3 major criteria and 2 minor criteria.[1][2][3]

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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