Document Detail

Primary myelofibrosis is the most frequent myeloproliferative neoplasm associated with del(5q): clinicopathologic comparison of del(5q)-positive and -negative cases.
MedLine Citation:
PMID:  18538839     Owner:  NLM     Status:  MEDLINE    
Among 23 cases of myeloproliferative neoplasms (MPNs) with an associated del(5q) seen at our institution, 14 (61%) fulfilled diagnostic criteria for primary myelofibrosis (PMF). Other diagnoses included polycythemia vera (PV; n=2), essential thrombocythemia (ET; n=1), post-ET myelofibrosis (n=1), systemic mastocytosis (SM; n=1), and MPN, unclassifiable (n=4). Compared to their del(5q)-negative counterparts, del(5q)-positive PMF cases were significantly more anemic (p<0.001) and thrombocytopenic (p<0.001). However, survival and leukemic transformation rates appear to be similar between the two groups. del(5q)-positive PMF was histologically characterized by a mixture of both small and monolobated megakaryocytes as well as large and bizarre megakaryocytes. When used, lenalidomide therapy induced hematological and cytogenetic remissions in del(5q)-positive PMF. The current study identifies PMF as another del(5q)-associated myeloid malignancy with characteristic megakaryocyte morphology.
Rafael Santana-Davila; Ayalew Tefferi; Shernan G Holtan; Rhett P Ketterling; Gordon W Dewald; Ryan A Knudson; David P Steensma; Dong Chen; James D Hoyer; Curtis A Hanson
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Publication Detail:
Type:  Journal Article     Date:  2008-06-09
Journal Detail:
Title:  Leukemia research     Volume:  32     ISSN:  0145-2126     ISO Abbreviation:  Leuk. Res.     Publication Date:  2008 Dec 
Date Detail:
Created Date:  2008-09-22     Completed Date:  2009-01-09     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7706787     Medline TA:  Leuk Res     Country:  England    
Other Details:
Languages:  eng     Pagination:  1927-30     Citation Subset:  IM    
Division of Hematology, Mayo Clinic, Rochester, MN, USA.
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MeSH Terms
Bone Marrow / pathology
Chromosomes, Human, Pair 5*
Megakaryocytes / pathology
Myeloproliferative Disorders / genetics*,  pathology
Primary Myelofibrosis / etiology,  genetics*,  mortality,  pathology
Sequence Deletion*
Survival Analysis

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