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 Hoyer, Curtis A. Hanson

Research output: Contribution to journalArticle

12 Citations (Scopus)

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 2008

Fingerprint

Primary Myelofibrosis
Megakaryocytes
Neoplasms
Systemic Mastocytosis
Essential Thrombocythemia
Polycythemia Vera
Cytogenetics

Keywords

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

ASJC Scopus subject areas

  • Cancer Research
  • Hematology
  • Oncology

Cite this

Primary myelofibrosis is the most frequent myeloproliferative neoplasm associated with del(5q) : Clinicopathologic comparison of del(5q)-positive and -negative cases. / Santana-Davila, Rafael; Tefferi, Ayalew; Holtan, Shernan G.; Ketterling, Rhett P.; Dewald, Gordon W.; Knudson, Ryan A.; Steensma, David P.; Chen, Dong; Hoyer, James; Hanson, Curtis A.

In: Leukemia Research, Vol. 32, No. 12, 12.2008, p. 1927-1930.

Research output: Contribution to journalArticle

Santana-Davila, Rafael ; Tefferi, Ayalew ; Holtan, Shernan G. ; Ketterling, Rhett P. ; Dewald, Gordon W. ; Knudson, Ryan A. ; Steensma, David P. ; Chen, Dong ; Hoyer, James ; Hanson, Curtis A. / Primary myelofibrosis is the most frequent myeloproliferative neoplasm associated with del(5q) : Clinicopathologic comparison of del(5q)-positive and -negative cases. In: Leukemia Research. 2008 ; Vol. 32, No. 12. pp. 1927-1930.
@article{500976aa280e429291703fd246ccec8c,
title = "Primary myelofibrosis is the most frequent myeloproliferative neoplasm associated with del(5q): Clinicopathologic comparison of del(5q)-positive and -negative cases",
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.",
keywords = "Deletion 5q (del(5q)), Myeloproliferative, Primary myelofibrosis",
author = "Rafael Santana-Davila and Ayalew Tefferi and Holtan, {Shernan G.} and Ketterling, {Rhett P.} and Dewald, {Gordon W.} and Knudson, {Ryan A.} and Steensma, {David P.} and Dong Chen and James Hoyer and Hanson, {Curtis A.}",
year = "2008",
month = "12",
doi = "10.1016/j.leukres.2008.04.022",
language = "English (US)",
volume = "32",
pages = "1927--1930",
journal = "Leukemia Research",
issn = "0145-2126",
publisher = "Elsevier Limited",
number = "12",

}

TY - JOUR

T1 - Primary myelofibrosis is the most frequent myeloproliferative neoplasm associated with del(5q)

T2 - Clinicopathologic comparison of del(5q)-positive and -negative cases

AU - Santana-Davila, Rafael

AU - Tefferi, Ayalew

AU - Holtan, Shernan G.

AU - Ketterling, Rhett P.

AU - Dewald, Gordon W.

AU - Knudson, Ryan A.

AU - Steensma, David P.

AU - Chen, Dong

AU - Hoyer, James

AU - Hanson, Curtis A.

PY - 2008/12

Y1 - 2008/12

N2 - 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.

AB - 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.

KW - Deletion 5q (del(5q))

KW - Myeloproliferative

KW - Primary myelofibrosis

UR - http://www.scopus.com/inward/record.url?scp=51649121491&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=51649121491&partnerID=8YFLogxK

U2 - 10.1016/j.leukres.2008.04.022

DO - 10.1016/j.leukres.2008.04.022

M3 - Article

C2 - 18538839

AN - SCOPUS:51649121491

VL - 32

SP - 1927

EP - 1930

JO - Leukemia Research

JF - Leukemia Research

SN - 0145-2126

IS - 12

ER -