Leukemia

TABLE 2

FROM:

Classification and diagnosis of myeloproliferative neoplasms: The 2008 World Health Organization criteria and point-of-care diagnostic algorithms

A Tefferi and J W Vardiman

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Table 2. The 2008 World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis

2008 WHO diagnostic criteria
   Polycythemia vera a   Essential thrombocythemia a   Primary myelofibrosis a
Major criteria1Hgb >18.5 g dl-1 (men)
>16.5 g dl-1 (women)
or
Hgb or Hct >99th percentile
of reference range for age,
sex or altitude of residence
or
Hgb>17 g dl-1 (men),
or>15 g dl-1 (women)
if associated with a sustained
increase of greater than or equal to2 g dl-1 from baseline
that cannot be attributed to
correction of iron deficiency
or
Elevated red cell mass
>25% above mean normal
predicted value
1Platelet count greater than or equal to450 times 109 l-1 1Megakaryocyte proliferation
and atypiab accompanied by either
reticulin and/or collagen fibrosis,
or
In the absence of reticulin fibrosis,
the megakaryocyte changes must
be accompanied by increased
marrow cellularity, granulocytic
proliferation and often decreased
erythropoiesis (i.e. pre-fibrotic PMF).
 2Presence of JAK2V617F
or similar mutation
2Megakaryocyte proliferation with
large and mature morphology.
No or little granulocyte or erythroid
Proliferation.
2Not meeting WHO criteria for CML,
PV, MDS, or other myeloid neoplasm
   3Not meeting WHO criteria for CML, PV,
PMF, MDS or other myeloid neoplasm
3Demonstration of JAK2V617F
or other clonal marker
or
no evidence of reactive marrow fibrosis
   4Demonstration of JAK2V617F
or other clonal marker
or
no evidence of reactive thrombocytosis
  
       
Minor criteria1BM trilineage myeloproliferation  1Leukoerythroblastosis
 2Subnormal serum Epo level  2Increased serum LDH
 3EEC growth  3Anemia
     4Palpable splenomegaly

 Abbreviations: CML, chronic myelogenous leukemia; EEC, endogenous erythroid colony; Epo, erythropoietin; Hct, hematocrit; Hgb, hemoglobin; LDH, lactate dehydrogenase; MDS, myelodysplastic syndrome; WHO, World Health Organization.

a Diagnosis of polycythemia vera (PV) requires meeting either both major criteria and one minor criterion or the first major criterion and 2 minor criteria. Diagnosis of essential thrombocythemia requires meeting all four major criteria. Diagnosis of primary myelofibrosis (PMF) requires meeting all three major criteria and two minor criteria.

b Small to large megakaryocytes with an aberrant nuclear/cytoplasmic ratio and hyperchromatic and irregularly folded nuclei and dense clustering.

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