Myelofibrosis with myeloid metaplasia in adult individuals 30 years old or younger

Presenting features, evolution and survival

Francisco Cervantes, Giovanni Barosi, Juan Carlos Hernández-Boluda, Monia Marchetti, Emilio Montserrat

Research output: Contribution to journalArticle

25 Citations (Scopus)

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 languageEnglish
Pages (from-to)324-327
Number of pages4
JournalEuropean Journal of Haematology
Volume66
Issue number5
DOIs
Publication statusPublished - 2001

Fingerprint

Primary Myelofibrosis
Survival
Budd-Chiari Syndrome
Thrombocytosis
Gastrointestinal Diseases
Lost to Follow-Up
Leukocytosis
Portal Hypertension
Bone Marrow Transplantation
Chromosome Aberrations
Disease Progression
Anemia
Young Adult
Blood Cells
Bone Marrow
Hemorrhage

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 journalArticle

Cervantes, Francisco ; Barosi, Giovanni ; Hernández-Boluda, Juan Carlos ; Marchetti, Monia ; Montserrat, Emilio. / Myelofibrosis with myeloid metaplasia in adult individuals 30 years old or younger : Presenting features, evolution and survival. In: European Journal of Haematology. 2001 ; Vol. 66, No. 5. pp. 324-327.
@article{54a48068dfd34fc1b592804f863173b7,
title = "Myelofibrosis with myeloid metaplasia in adult individuals 30 years old or younger: Presenting features, evolution and survival",
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.",
keywords = "Agnogenic myeloid, Idiopathic myelofibrosis, Metaplasia, Myelofibrosis with myeloid metaplasia, Natural history, Young patients",
author = "Francisco Cervantes and Giovanni Barosi and Hern{\'a}ndez-Boluda, {Juan Carlos} and Monia Marchetti and Emilio Montserrat",
year = "2001",
doi = "10.1034/j.1600-0609.2001.066005324.x",
language = "English",
volume = "66",
pages = "324--327",
journal = "European Journal of Haematology",
issn = "0902-4441",
publisher = "Wiley-Blackwell Publishing Ltd",
number = "5",

}

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 -