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| According to the World Health Organization (WHO) diagnostic criteria for primary myelofibrosis, [[polycythemia vera]], and [[essential thrombocythemia]], the diagnosis of primary myelofibrosis is made when all three of the following major diagnostic criteria and at least two minor criteria are met.<ref name=diagnosticcriteriamyelofibrosis1>World Health Organization (WHO) Diagnostic Criteria for Primary Myelofibrosis (PMF), Polycythemia Vera (PV), and Essential Thrombocythemia (ET). MPN Connect 2016. http://www.mpnconnect.com/pdf/who-diagnostic-criteria-myelofibrosis.pdf. Accessed on March 8, 2016</ref><ref name="pmid17488875">{{cite journal| author=Tefferi A, Thiele J, Orazi A, Kvasnicka HM, Barbui T, Hanson CA et al.| 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 | year= 2007 | volume= 110 | issue= 4 | pages= 1092-7 | pmid=17488875 | doi=10.1182/blood-2007-04-083501 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17488875 }} </ref>
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| ! style="background: #4479BA; width: 800px;" | {{fontcolor|#FFF|Major Criteria}}
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| ! style="background: #4479BA; width: 800px;" | {{fontcolor|#FFF|Minor Criteria}}
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| '''I'''. On [[bone marrow biopsy]], proliferation and atypia of [[megakaryocytes]], with or without [[reticulin]] or [[collagen|collagen fibrosis]] and it must be accompanied by hypercellular [[bone marrow]]<br><br>
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| '''II'''. Finding of ''[[JAK2|JAK2-V617F]]'' mutation or other clonal marker<br><br>
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| '''III'''. Not meeting the WHO criteria for [[chronic myeloid leukemia]] (CML), [[polycythemia vera]] (PV), [[myelodysplastic syndrome]] (MDS), or other myeloid neoplasm
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| '''I'''. [[Anemia]]<br><br>
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| '''II'''. [[LDH|Increased serum lactate dehydrogenase (LDH)]]<br><br>
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| '''III'''. Leukoerythroblastosis on blood smear<br><br>
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| '''IV'''. [[splenomegaly|Palpable splenomegaly]]
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| ==References== | | ==References== |
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
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| Clinical findings
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Morphological findings
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Spleen and liver
- No or mild splenomegaly or hepatomegaly
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Blood
- No or mild leukoerythroblastosis
- No or mild red blood cell poikilocytosis
- Few if any dacryocytes
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Hematology (variable)
- Mild anemia
- Mild to moderate leukocytosis
- Mild to marked thrombocytosis
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Bone marrow
- Hypercellularity
- Neutrophilic proliferation
- Megakaryocytic proliferation
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2001 World Health Organization (WHO) criteria for overtly fibrotic (overt primary myelofibrosis) phase
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| Clinical findings
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Morphological findings
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Spleen and liver
- Moderate to marked splenomegaly or hepatomegaly
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Blood
- Leukoerythroblastosis
- Prominent red blood cell poikilocytosis
- Prominent dacryocytosis
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Hematology (variable)
- Leukoerythroblastosis
- White blood cells decreased to elevated
- Platelet count decreased to elevated
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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
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Proposed revised World Health Organization (WHO) criteria for primary myelofibrosis (PMF)
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| Clinical findings
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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)
- 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¶
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Minor criteria
- Leukoerythroblastosis∥
- Increase in serum lactate dehydrogenase level∥
- Anemia∥
- 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.
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- Diagnosis requires meeting all 3 major criteria and 2 minor criteria.
References
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