Diagnostic and Differential Criteria of Essential Thrombocythemia and Reactive Thrombocytosis

Abstract

Among the chronic myeloproliferative disorders essential thrombocythemia (ET) is known to be a distinct clinical entity in which an excessive number of morphologically and functionally abnormal platelets are produced. The clonal nature of the disease is well established. Based on a review of the literature the present authors propose the following novel criteria for the diagnosis of ET:

A1. Platelet count in excess of 600 × 10⁹/L.

A2. No increase in red-cell mass (RCM) in the presence of stainable iron in the bone marrow or failure of iron trial (RCM >36mL/kg in males and >32mL/kg in females; or RCM <25% above mean normal predicted value*).

A3. No Philadelphia chromosome.

A4. Megakaryocytic hyperplasia (=increased megakaryocyte number and size) in histological sections of bone marrow and/or increased megakaryocytic ploidy (two-color flow cytometry); no collagen fibrosis.

B1. Splenomegaly on isotopic scan or echogram.

B2. Unstimulated growth of BFU-E and/or CFU-Meg present.

B3. Normal ESR/fibrinogen.

The diagnosis of ET is considered to be established if A1 + A2 + A3 + A4 or A1 + A2 + A3 + two B-criteria are fulfilled. (Br J Haematol 1995;89:748–756.)

Key Words:

• Essential thrombocythemia
• chronic myeloproliberative disorders
• colony forming units
• megakaryocyte ploidy
• spleen size