PMID- 12682880
OWN - NLM
STAT- MEDLINE
DCOM- 20031107
LR  - 20081121
IS  - 0037-1963 (Print)
IS  - 0037-1963 (Linking)
VI  - 40
IP  - 1 Suppl 1
DP  - 2003 Jan
TI  - Transplant decision-making strategies in the myeloproliferative disorders.
PG  - 30-3
AB  - Myelofibrosis with myeloid metaplasia, also known as idiopathic myelofibrosis 
      (IF) or agnogenic myeloid metaplasia, is one of the characteristic manifestations 
      of polycythemia vera (PV) in the spent phase, and has a particularly adverse 
      prognosis. IF may also present de novo. To date, treatment strategies for both 
      spent-phase PV and IF have frustrated both clinicians and patients, with little 
      clear progress made over the past 50 years. Treatment modalities with some 
      benefit in chronic myeloid leukemia (CML), such as interferon (IFN), have been 
      used to shrink the massive organomegaly seen in these patients and to improve 
      their marrow function, but are not curative, and not all patients respond or can 
      tolerate the agent. A curative approach is allogeneic peripheral hematopoietic 
      stem cell transplantation. The preparative regimens used in fully ablative 
      techniques rule out older patients for consideration, and many younger patients 
      with good prognostic criteria may do sufficiently well on medical treatment or 
      observation to avoid transplantation. Older patients may have the option to 
      undergo a human leukocyte antigen (HLA)-identical sibling transplant using a 
      reduced intensity preparative regimen in order to minimize peritransplant 
      mortality. Thus a prerequisite to the broad use of transplantation is objective 
      determination of candidacy. Several evaluation methods agree that anemia, age, 
      and cytogenetic abnormalities all predict poor survival in IF, suggesting that 
      patients with anemia and an abnormal karyotype are the prime candidates for 
      allogeneic transplantation. Experimental peripheral blood models that may reflect 
      the degree of marrow fibrosis, such as the serum procollagen 3 peptide assay, 
      have been used to determine if they are more informative of patient status than a 
      single, random bone marrow sampling. Marrow fibrosis may be patchy, and thus a 
      marrow biopsy alone without other data about marrow function may be misleading. 
      Considerable long-term success in eradicating fibrosis and restoring normal 
      cytogenetics, normal bone marrow morphology, and normal complete blood cell 
      counts through transplantation has been reported. Many questions remain to be 
      answered, however, before the appropriate role of hematopoietic stem cell 
      transplantation in the setting of both spent-phase PV and IF can be determined.
CI  - Copyright 2003 Elsevier Inc. All rights reserved.
FAU - Fruchtman, Steven M
AU  - Fruchtman SM
AD  - Division of Hematology, Mount Sinai School of Medicine, One Gustave L. Levy 
      Place, Box 1410, New York, NY 10029, USA.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Semin Hematol
JT  - Seminars in hematology
JID - 0404514
SB  - IM
MH  - *Hematopoietic Stem Cell Transplantation
MH  - Humans
MH  - Leukemia, Myeloid, Acute/etiology/therapy
MH  - Male
MH  - Middle Aged
MH  - Myeloproliferative Disorders/*diagnosis/*therapy
MH  - Polycythemia Vera/complications/mortality/therapy
MH  - Primary Myelofibrosis/etiology/therapy
MH  - Risk Factors
MH  - Survival Rate
MH  - Thrombocythemia, Essential/complications/mortality
RF  - 19
EDAT- 2003/04/12 05:00
MHDA- 2003/11/08 05:00
CRDT- 2003/04/12 05:00
PHST- 2003/04/12 05:00 [pubmed]
PHST- 2003/11/08 05:00 [medline]
PHST- 2003/04/12 05:00 [entrez]
AID - S003719630350021X [pii]
AID - 10.1053/shem.2003.50032 [doi]
PST - ppublish
SO  - Semin Hematol. 2003 Jan;40(1 Suppl 1):30-3. doi: 10.1053/shem.2003.50032.