[Idiopathic myelofibrosis: clinical course, survival, and causes of death in a series of 60 patients].
The evolutive patterns, actuarial survival and causes of death of idiopathic myelofibrosis were analysed in a series of 60 patients. The median age of the patients was 64 years; 41 were males and 19 females. In the initial bone-marrow biopsy studies 25 patients were in stage MF/C, 17 in MF/O- and 18 in MF/O+. When performing this analysis, 32 patients were dead, 19 were still alive and 9 had been lost after a median follow-up of 6 months. The median survival of the whole series was 57 months. Four major evolutive patterns were recorded: 1) blastic crisis (7 cases), 2) portal vein hypertension (4 cases), 3) liver insufficiency due to massive myeloid metaplasia of the liver without signs of portal vein hypertension (5 cases); in 2 of them this pattern followed splenectomy), and 4) heart failure ascribable, at least partially, to post-transfusion haemochromatosis (3 cases). The blastic crisis appearing in 7 patients presented after a median follow-up of 19 months, and 6 of these patients have died after a median of 5 months since the diagnosis of the blastic crisis. In the 16 patients who died without any characteristic evolutive pattern, the following causes of death could be registered: septic shock (6 cases), intracranial haemorrhage, haemoperitoneum and acute renal failure (1 case each), whereas the cause of the deceased was unclear in the remaining 7 patients.
Escuela de Hematología Farreras Valenti, Servicio de Hematología, Hospital Clínico, Villarroel, Barcelona.
A
A
López-Guillermo
F
F
Cervantes
M
M
Rovira
A
A
Pereira
L
L
Quinteros
C
C
Rozman
D000368:Aged; D001752:Blast Crisis; D002423:Cause of Death; D005260:Female; D006486:Hemosiderosis / Q000209:etiology; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology; D015993:Life Tables; D008107:Liver Diseases / Q000209:etiology; D008297:Male; D008875:Middle Aged; D055728:Primary Myelofibrosis / Q000150:complications / Q000401:mortality* / Q000473:pathology; D013030:Spain / Q000453:epidemiology; D015996:Survival Rate; D065227:Transfusion Reaction
Mielofibrosis idiopática: patrones evolutivos, supervivencia y causas de muerte en una serie de 60 pacientes.