Who Diagnostic Criteria Myelofibrosis

World Health Organization (WHO) Diagnostic Criteria for Primary Myelofibrosis (PMF), Polycythemia Vera (PV), and Essential Thrombocythemia (ET) 1 Check off the major and minor criteria corresponding to a patient’s clinical presentation. Add the number of check marks in the HIGHLIGHTED criteria in each column and compare the result against the totals required to meet WHO guidelines for diagnosis of each myeloproliferative neoplasm (MPN). CML = chronic myelogenous leukemia; EPO = erythropoietin; LDH = lactate dehydrogenase; MDS = myelodysplastic syndrome; MPN = myeloproliferative neoplasm; WHO = World Health Organization a For PV: or JAK2 exon 12 mutation; for ET: or other clonal marker or no evidence of reactive thrombocytosis; for PMF: or other clonal marker or no evidence of reactive marrow fibrosis. b Presence of megakaryocyte proliferation and atypia (small to large megakaryocytes with an aberrant nuclear/cytoplasmic ratio and hyperchromatic, bulbous, or irregularly folded nuclei and dense clustering). 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). c And no or little granulocyte or erythroid proliferation. d For PV: bone marrow trilineage myeloproliferation. e Or other evidence of increased red cell volume as follows: Hemoglobin or hematocrit >99th percentile of method-specific reference range for age, sex, altitude of residence, or hemoglobin >17 g/dL in men, >15 g/dL in women if associated with a documented and sustained increase of at least 2 g/dL from a person’s baseline value that cannot be attributed to correction of iron deficiency or elevated red cell mass >25% above mean normal predicted value. REFERENCES 1. Tefferi A, Vardiman JW. Leukemia. 2008;22:14-22. © 2014, Incyte Corporation. All rights reserved. EDU-1147 04/14 | Learn more at MPNConnect.com PRIMARY MYELOFIBROSIS (PMF) POLYCYTHEMIA VERA (PV) ESSENTIAL THROMBOCYTHEMIA (ET) SOURCE CRITERIA Major criteria Minor criteria Major criteria Minor criteria Major criteria Genetics and other considerations Presence of JAK2V617F or other clonal marker a Not meeting WHO criteria for CML, PV, MDS, or other myeloid neoplasm TOTAL NUMBER OF HIGHLIGHTED CRITERIA To meet WHO diagnostic criteria for this MPN » Must have at least » 3 2 2 1 4 Or » Hemoglobin > 18.5 g/dL in men, > 16.5 g/dL in women 2 Clinical examination Palpable splenomegaly Bone marrow biopsy Proliferation and atypia of megakaryocytes, with or without b Megakaryocyte proliferation with large and mature morphology c Myeloproliferation d Complete blood count Anemia Hemoglobin > 18.5 g/dL in men, > 16.5 g/dL in women e Platelet count ≥450 x 10 9 /L Biochemistry Subnormal serum EPO level Increased serum LDH Leukoerythroblastosis Special assay Endogenous erythroid colony growth OTHER LAB RESULTS

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Transcript of Who Diagnostic Criteria Myelofibrosis

World Health Organization (WHO) Diagnostic Criteria for Primary Myelofibrosis (PMF), Polycythemia Vera (PV), and Essential Thrombocythemia (ET)1

Check off the major and minor criteria corresponding to a patient’s clinical presentation. Add the number of check marks in the HIGHLIGHTED criteria in each column and compare the result against the totals required to meet WHO guidelines for diagnosis of each myeloproliferative neoplasm (MPN).

CML = chronic myelogenous leukemia; EPO = erythropoietin; LDH = lactate dehydrogenase; MDS = myelodysplastic syndrome; MPN = myeloproliferative neoplasm; WHO = World Health Organization

aFor PV: or JAK2 exon 12 mutation; for ET: or other clonal marker or no evidence of reactive thrombocytosis; for PMF: or other clonal marker or no evidence of reactive marrow fibrosis.

bPresence of megakaryocyte proliferation and atypia (small to large megakaryocytes with an aberrant nuclear/cytoplasmic ratio and hyperchromatic, bulbous, or irregularly folded nuclei and dense clustering). 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).

cAnd no or little granulocyte or erythroid proliferation.dFor PV: bone marrow trilineage myeloproliferation.eOr other evidence of increased red cell volume as follows: Hemoglobin or hematocrit >99th percentile of method-specific reference range for age, sex, altitude of residence, or hemoglobin >17 g/dL in men, >15 g/dL in women if associated with a documented and sustained increase of at least 2 g/dL from a person’s baseline value that cannot be attributed to correction of iron deficiency or elevated red cell mass >25% above mean normal predicted value.

REFERENCES 1. Tefferi A, Vardiman JW. Leukemia. 2008;22:14-22.

PRIMARY MYELOFIBROSIS (PMF) POLYCYTHEMIA VERA (PV) ESSENTIALTHROMBOCYTHEMIA (ET)

SOURCE CRITERIA Major criteria Minor criteria Major criteria Minor criteria Major criteria

Genetics and other considerations

Presence of JAK2V617F or other clonal markera

Not meeting WHO criteria for CML, PV, MDS, or other myeloid neoplasm

TOTAL NUMBER OFHIGHLIGHTED CRITERIA

To meet WHO diagnostic criteria

for this MPN »

Must have at least » 3 2 2 1 4

Or »Hemoglobin

> 18.5 g/dL in men,> 16.5 g/dL in women