Table 5 Criteria for primary... | American Society of Hematology

Table 5

Criteria for primary myelofibrosis (PMF)

Diagnosis requires meeting all 3 major criteria and 2 minor criteria
Major criteria Presence of megakaryocyte proliferation and atypia,* usually accompanied by either reticulin 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 polycythemia vera,†BCR-ABL1–positive chronic myelogenous leukemia,‡ myelodysplastic syndrome,§ or other myeloid disorders Demonstration of JAK2 V617F or other clonal marker (eg, MPLW515K/L),or,in the absence of the above clonal markers, no evidence that bone marrow fibrosis is secondary to infection, autoimmune disorder or other chronic inflammatory condition, hairy cell leukemia or other lymphoid neoplasm, metastatic malignancy, or toxic (chronic) myelopathies‖
Minor criteria Leukoerythroblastosis¶ Increase in serum lactate dehydrogenase level¶ Anemia¶ Palpable splenomegaly¶

Diagnosis requires meeting all 3 major criteria and 2 minor criteria

Major criteria

Presence of megakaryocyte proliferation and atypia,* usually accompanied by either reticulin 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 polycythemia vera,†BCR-ABL1–positive chronic myelogenous leukemia,‡ myelodysplastic syndrome,§ or other myeloid disorders
Demonstration of JAK2 V617F or other clonal marker (eg, MPLW515K/L),or,in the absence of the above clonal markers, no evidence that bone marrow fibrosis is secondary to infection, autoimmune disorder or other chronic inflammatory condition, hairy cell leukemia or other lymphoid neoplasm, metastatic malignancy, or toxic (chronic) myelopathies‖

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-ABL1.

Requires the absence of dyserythropoiesis and dysgranulopoiesis.

‖

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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