20+ yr without leukemic or fibrotic transformation in essential thrombocythemia or polycythemia vera: predictors at diagnosis

• Published in: European journal of haematology 2008-05, Vol.80 (5), p.386-390
• Main Authors: Tefferi, Ayalew, Gangat, Naseema, Wolanskyj, Alexandra P., Schwager, Susan, Pardanani, Animesh, Lasho, Terra L., Mesa, Ruben, McClure, Rebecca F., Li, Chin-Yang, Hanson, Curtis A.
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; Female; Fibrosis - pathology; Humans; JAK2; JAK2V617F; leukemia; Leukemia - pathology; Male; Middle Aged; Polycythemia Vera - diagnosis; Polycythemia Vera - epidemiology; prognosis; survival; Thrombocythemia, Essential - diagnosis; Thrombocythemia, Essential - epidemiology; Time Factors; V617F
• ISSN: 0902-4441; 1600-0609
• DOI: 10.1111/j.1600-0609.2008.01038.x

Objectives:  The current study identified patients with either essential thrombocythemia (ET) or polycythemia vera (PV) who have survived for at least 20 yr without the development of either acute myeloid leukemia/myelodysplastic syndrome (AML/MDS) or myelofibrosis (MF) and compared their presenting features with those in whom these complications occurred in the first 10 yr of disease. Methods:  The study patients were selected from an institutional database of 1061 patients with either ET (n = 603) or PV (n = 458). In both instances, three distinct groups were delineated and their presenting features compared; group A included patients who have remained AML/MDS/MF free after a minimum follow‐up of 20 yr; groups B and C included patients who developed either AML/MDS or MF, respectively, in the first decade of their disease. Results:  The respective number of patients who fulfilled the above‐mentioned criteria for inclusion in groups A, B and C were 40, 12 and 8 for ET and 23, 18 and 12 for PV. In ET, compared with both groups B and C, group A displayed significantly fewer patients with less than normal hemoglobin level (P