Copyright ©The Author(s) 2017.
World J Hematol. Aug 6, 2017; 6(3): 32-54
Published online Aug 6, 2017. doi: 10.5315/wjh.v6.i3.32
Table 1 The 1980 Rotterdam Clinical and Pathological criteria for essential thrombocythemia and polycythemia vera
The 1980 RCP criteria for prefibrotic ET[37,52]
Major criteria
A1Persistent platelet count in excess of 400 × 109/L
A2Increase and clustering of enlarged megakaryocytes in bone marrow biopsy
A3No or slight increase of reticulin fibers (RF 0 or RF 1)
Confirmative criteria
B1Presence of large platelets in a peripheral blood smear
B3No or slight splenomegaly on ultrasound sonography (length diameter normal value < 12 cm)
B4Increase of LAP-score and no signs of fever or inflammation
Exclusion criterion
Ph+ chromosome and any other cytogenetic abnormality in blood or bone marrow nucleated cells
The 1980 RCP criteria for prefibrotic PV to replace the crude 1975 PVSG criteria for PV
A1The combination of erythrocyte count of > 6 × 1012/L and bone marrow hypercellularity due to EM or EMG hyperproliferation is pathognomonic diagnostic for PV (Dameshek and Henthel[27] 1940, Dameshek[38] 1950, Kurnike et al[39] 1972) obviating the need to measure raised red cell mass
A2Increase in bone marrow biopsy of clustered, enlarged pleomorphic megakaryocytes with hyperlobulated nuclei and moderate to marked increase cellularity of megakaryopoiesis/erythropoiesis or typically trilinear mega-erythro-granulopoiesis (EMG). Such a typical PV bone marrow picture excludes all variant of primary and secondary erythrocytosis[37-39]
B1Thrombocythemia, persistant increase of platelet > 400 × 109/L
B2Leukocytosis, leucocyte count > 109/L and low erythrocyte sedimentation rate
B3Raised leukocyte alkaline phosphatase score > 100, absence of fever or infection
B4Splenomegaly on ultrasound sonography
A1 + A2 establish PV and exclude erythrocytosis. One or more of B confirm PV