Myelofibrosis with myeloid metaplasia in adult individuals 30 years old oryounger: presenting features, evolution and survival

Myelofibrosis with myeloid metaplasia (MMM) usually affects older people an d is associated with a median survival of 3.5-5 yr. Survival of MMM patient s 55 yr old or younger is longer, but there is no information on the younge st subset. The presenting features, evolution and survival were analyzed in 9 patients with MMM aged 30 yr or less, representing 2.8% of two series in cluding 323 cases. Age ranged from 17 to 30 yr; 3 patients were males and 6 females. Five patients were asymptomatic and none had constitutional sympt oms. Anemia was observed in 4 patients, being severe in only one. The WBC c ount was normal in 7 patients and 2 had mild leukocytosis; moderate thrombo cytosis was observed in 7 patients. No patient showed blood blast cells or bone marrow cytogenetic abnormalities. With a median follow-up of 6.8 (rang e 0.8-28) yr, 2 patients died 10.7 and 9.9 yr from diagnosis, one from comp lications of bone marrow transplantation performed after progression of the disease and the other from gastrointestinal bleeding secondary to portal h ypertension. Among remaining patients, one developed Budd-Chiari syndrome, another was lost to follow-up at 3 yr, and 5 remain asymptomatic and curren tly without treatment tone had been splenectomized at diagnosis). These res ults indicate that in most young adults with MMM the disease presents witho ut adverse prognostic factors and may remain stable for years, which may be of interest when considering the treatment of such patients.