Chronic myeloproliferative disorders with thrombocythemia: a comparative study of two classification systems (PVSG, WHO) on 839 patients

A multicenter observational study was performned on 839 adult patients with a chronic myeloproliferative disorder and a platelet count in excess of 600x10(9)/l to compare the updated criteria of the Polycythemia Vera Study Group (PVSG) with the recently published WHO classification. Essential thrombocythemia (ET) was diagnosed in 483 patients according to the PVSG; however, when considering histopathology as a major diagnostic feature of the WHO criteria, (true) ET could be established in only 162 patients. The remaining cases were found to represent either initially prefibrotic (184 patients) or early fibrotic (137 patients) chronic idiopathic myelofibrosis (IMF). On the other hand, both classification systems enabled a clear-cut distinction of patients showing overt IMF and polycythemia vera. Follow-up examinations in 140 patients with ET according to the PVSG criteria included also sequential bone marrow biopsies (interval: 38+/-30 months). A transition into mild reticulin fibrosis occurred in only 2 of 49 patients with (true) ET in contrast to 45 of 91 patients with initial and early IMF where a progression into overt myelofibrosis was encountered. Survival patterns for ET displayed significant differences because according to the PVSG a 16.5% disease-specific loss of life expectancy was calculable compared to a value of only 8.9% when following the WHO criteria. Contrasting this finding, initial and early IMF mimicking ET was characterized by a reduction of life expectancy ranging between 21.6% and 32.3 %. In conclusion, a more accurate classification of ET is warranted by regarding the WHO criteria that include histopathology as a major feature for diagnosis.