Circulating Cd34+ cell count differentiates primary myelofibrosis from other Philadelphia-negative myeloproliferative neoplasms: a pragmatic study

Ann Hematol. 2016 Oct;95(11):1819-23. doi: 10.1007/s00277-016-2784-x. Epub 2016 Sep 1.

Abstract

A high number of circulating CD34+ cells has been advocated to distinguish primary myelofibrosis from other Philadelphia-negative myeloproliferative neoplasms. We re-evaluated the diagnostic interest of measuring circulating CD34+ cells in 26 healthy volunteers and 256 consecutive patients at diagnosis for whom a myeloproliferative neoplasm was suspected. The ROC curve analysis showed that a number of CD34+ <10/μl excludes the diagnosis of primary myelofibrosis with a sensitivity of 97 % and a specificity of 90 % (area under the curve: 0.93 [0.89-0.98]; p < 0.001). Patients with PMF harboring a CALR mutation had more circulating CD34+ cells than patients with either a JAK 2 or MPL mutation (p = 0.02 and p < 0.01, respectively). These results suggest that this fast, simple, non-invasive, and standardized test is of particular interest to exclude the diagnosis of primary myelofibrosis.

Keywords: Circulating CD34+ cells; Myeloproliferative neoplasms (MPN); Primary myelofibrosis (PMF).

Publication types

  • Multicenter Study

MeSH terms

  • Antigens, CD34 / analysis
  • Area Under Curve
  • Blood Cell Count*
  • Calreticulin / genetics
  • DNA Mutational Analysis
  • Hematopoietic Stem Cells*
  • Humans
  • Janus Kinase 2 / genetics
  • Mutation
  • Myelodysplastic Syndromes / blood
  • Myelodysplastic Syndromes / diagnosis
  • Myeloproliferative Disorders / blood
  • Myeloproliferative Disorders / diagnosis
  • Primary Myelofibrosis / blood
  • Primary Myelofibrosis / diagnosis*
  • Primary Myelofibrosis / genetics
  • ROC Curve
  • Receptors, Thrombopoietin / genetics
  • Retrospective Studies
  • Sensitivity and Specificity

Substances

  • Antigens, CD34
  • CALR protein, human
  • Calreticulin
  • Receptors, Thrombopoietin
  • MPL protein, human
  • JAK2 protein, human
  • Janus Kinase 2