Sei sulla pagina 1di 1

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 patients 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).
PRIMARY MYELOFIBROSIS (PMF)
SOURCE

Genetics and other


considerations

Clinical examination

CRITERIA

POLYCYTHEMIA VERA (PV)

ESSENTIAL
THROMBOCYTHEMIA (ET)

Major criteria

Minor criteria

Major criteria

Minor criteria

Major criteria

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

Presence of JAK2V617F
or other clonal markera
Not meeting WHO criteria for CML,
PV, MDS, or other myeloid neoplasm
Palpable splenomegaly
Proliferation and atypia of
megakaryocytes, with or without
b

Bone marrow biopsy

Megakaryocyte proliferation with


large and mature morphologyc
Myeloproliferationd
Anemia

OTHER LAB RESULTS

Complete
blood count

Hemoglobin > 18.5 g/dL in men,


> 16.5 g/dL in women e
Platelet count 450 x 10 9 /L
Subnormal serum EPO level

Biochemistry
Increased serum LDH
Leukoerythroblastosis
Special assay

Endogenous erythroid colony growth

TOTAL NUMBER OF
HIGHLIGHTED CRITERIA

To meet WHO
diagnostic criteria
for this MPN

Must have at least


Or

CML = chronic myelogenous leukemia; EPO = erythropoietin; LDH = lactate dehydrogenase; MDS = myelodysplastic syndrome; MPN = myeloproliferative neoplasm;
WHO = World Health Organization
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 persons baseline value that cannot be attributed
to correction of iron deficiency or elevated red cell mass >25% above mean normal predicted value.
a

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

Potrebbero piacerti anche