Primary myelofibrosis is the most frequent myeloproliferative neoplasm associated with del(5q): Clinicopathologic comparison of del(5q)-positive and -negative cases

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

Research output: Contribution to journalArticle

13 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1927-1930
Number of pages4
JournalLeukemia Research
Volume32
Issue number12
DOIs
StatePublished - Dec 1 2008

Keywords

  • Deletion 5q (del(5q))
  • Myeloproliferative
  • Primary myelofibrosis

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

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