Abstract
Myelofibrosis with myeloid metaplasia (MMM) usually affects older people and is associated with a median survival of 3.5-5 yr. Survival of MMM patients 55 yr old or younger is longer, but there is no information on the youngest 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 including 323 cases. Age ranged from 17 to 30 yr; 3 patients were males and 6 females. Five patients were asymptomatic and none had constitutional symptoms. Anemia was observed in 4 patients, being severe in only one. The WBC count was normal in 7 patients and 2 had mild leukocytosis; moderate thrombocytosis was observed in 7 patients. No patient showed blood blast cells or bone marrow cytogenetic abnormalities. With a median follow-up of 6.8 (range 0.8-28) yr, 2 patients died 10.7 and 9.9 yr from diagnosis, one from complications of bone marrow transplantation performed after progression of the disease and the other from gastrointestinal bleeding secondary to portal hypertension. Among remaining patients, one developed Budd-Chiari syndrome, another was lost to follow-up at 3 yr, and 5 remain asymptomatic and currently without treatment (one had been splenectomized at diagnosis). These results indicate that in most young adults with MMM the disease presents without adverse prognostic factors and may remain stable for years, which may be of interest when considering the treatment of such patients.
| Original language | English |
|---|---|
| Pages (from-to) | 324-327 |
| Number of pages | 4 |
| Journal | European Journal of Haematology |
| Volume | 66 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - 2001 |
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Keywords
- Agnogenic myeloid
- Idiopathic myelofibrosis
- Metaplasia
- Myelofibrosis with myeloid metaplasia
- Natural history
- Young patients
ASJC Scopus subject areas
- Hematology
Cite this
Myelofibrosis with myeloid metaplasia in adult individuals 30 years old or younger : Presenting features, evolution and survival. / Cervantes, Francisco; Barosi, Giovanni; Hernández-Boluda, Juan Carlos; Marchetti, Monia; Montserrat, Emilio.
In: European Journal of Haematology, Vol. 66, No. 5, 2001, p. 324-327.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Myelofibrosis with myeloid metaplasia in adult individuals 30 years old or younger
T2 - Presenting features, evolution and survival
AU - Cervantes, Francisco
AU - Barosi, Giovanni
AU - Hernández-Boluda, Juan Carlos
AU - Marchetti, Monia
AU - Montserrat, Emilio
PY - 2001
Y1 - 2001
N2 - Myelofibrosis with myeloid metaplasia (MMM) usually affects older people and is associated with a median survival of 3.5-5 yr. Survival of MMM patients 55 yr old or younger is longer, but there is no information on the youngest 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 including 323 cases. Age ranged from 17 to 30 yr; 3 patients were males and 6 females. Five patients were asymptomatic and none had constitutional symptoms. Anemia was observed in 4 patients, being severe in only one. The WBC count was normal in 7 patients and 2 had mild leukocytosis; moderate thrombocytosis was observed in 7 patients. No patient showed blood blast cells or bone marrow cytogenetic abnormalities. With a median follow-up of 6.8 (range 0.8-28) yr, 2 patients died 10.7 and 9.9 yr from diagnosis, one from complications of bone marrow transplantation performed after progression of the disease and the other from gastrointestinal bleeding secondary to portal hypertension. Among remaining patients, one developed Budd-Chiari syndrome, another was lost to follow-up at 3 yr, and 5 remain asymptomatic and currently without treatment (one had been splenectomized at diagnosis). These results indicate that in most young adults with MMM the disease presents without adverse prognostic factors and may remain stable for years, which may be of interest when considering the treatment of such patients.
AB - Myelofibrosis with myeloid metaplasia (MMM) usually affects older people and is associated with a median survival of 3.5-5 yr. Survival of MMM patients 55 yr old or younger is longer, but there is no information on the youngest 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 including 323 cases. Age ranged from 17 to 30 yr; 3 patients were males and 6 females. Five patients were asymptomatic and none had constitutional symptoms. Anemia was observed in 4 patients, being severe in only one. The WBC count was normal in 7 patients and 2 had mild leukocytosis; moderate thrombocytosis was observed in 7 patients. No patient showed blood blast cells or bone marrow cytogenetic abnormalities. With a median follow-up of 6.8 (range 0.8-28) yr, 2 patients died 10.7 and 9.9 yr from diagnosis, one from complications of bone marrow transplantation performed after progression of the disease and the other from gastrointestinal bleeding secondary to portal hypertension. Among remaining patients, one developed Budd-Chiari syndrome, another was lost to follow-up at 3 yr, and 5 remain asymptomatic and currently without treatment (one had been splenectomized at diagnosis). These results indicate that in most young adults with MMM the disease presents without adverse prognostic factors and may remain stable for years, which may be of interest when considering the treatment of such patients.
KW - Agnogenic myeloid
KW - Idiopathic myelofibrosis
KW - Metaplasia
KW - Myelofibrosis with myeloid metaplasia
KW - Natural history
KW - Young patients
UR - http://www.scopus.com/inward/record.url?scp=0034971782&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0034971782&partnerID=8YFLogxK
U2 - 10.1034/j.1600-0609.2001.066005324.x
DO - 10.1034/j.1600-0609.2001.066005324.x
M3 - Article
VL - 66
SP - 324
EP - 327
JO - European Journal of Haematology
JF - European Journal of Haematology
SN - 0902-4441
IS - 5
ER -