Constitutive STAT5 phosphorylation in CD34+ cells of patients with primary myelofibrosis: Correlation with driver mutation status and disease severity

PLoS One. 2019 Aug 1;14(8):e0220189. doi: 10.1371/journal.pone.0220189. eCollection 2019.

Abstract

Primary Myelofibrosis (PMF) is a myeloproliferative disorder associated with JAK2V617F, Calreticulin (CALR) indels, and MPLW515L/K mutations activating the tyrosine kinase JAK2 and its downstream signaling pathway. The nature of signaling abnormalities in primary cells from PMF patients is poorly understood, since most of the work has been performed in cell lines or animal models. By flow cytometry we measured constitutive and cytokine induced phosphorylation of STAT5, STAT3, and ERK1/2 in circulating CD34+ cells from 57 patients with PMF (20 with prefibrotic-PMF) and 13 healthy controls (CTRLs). Levels of constitutive and TPO induced p-STAT5, and IL6 induced p-STAT3 were higher in patients than in CTRLs. Constitutive p-STAT5 values were lower in CALR than homozygous JAK2V617F mutated CD34+ cells from PMF patients. Moreover, constitutive p-STAT5 and IL6 induced p-STAT3 values correlated directly with circulating CD34+ cell number/L, and inversely with the frequency of circulating CD34+ cells expressing CXCR4. Constitutive p-STAT5 values of CD34+ cells were also inversely correlated with hemoglobin levels. When the patients were divided according with presence/absence of JAK2V617F mutation, all the correlations described characterized the JAK2V617F+ patients with prefibrotic-PMF (P-PMF). In conclusion, increased constitutive p-STAT5 and IL6 induced p-STAT3 values in circulating CD34+ cells characterize patients with PMF. Constitutive p-STAT5 and IL6 induced p-STAT3 values correlate with circulating CD34+ cell number/L, the frequency of circulating CD34+ cells expressing CXCR4 and hemoglobin levels within the prefibrotic JAK2V617F+ patient population. Our data point toward a complex activation of STAT5-dependent pathways in the stem/progenitor cell compartment, that characterize the phenotypic diversity of PMF.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Antigens, CD34 / metabolism*
  • Calreticulin / genetics
  • Case-Control Studies
  • Female
  • Follow-Up Studies
  • Humans
  • Janus Kinase 2 / genetics
  • Male
  • Middle Aged
  • Mutation*
  • Phosphorylation
  • Primary Myelofibrosis / genetics
  • Primary Myelofibrosis / metabolism
  • Primary Myelofibrosis / pathology*
  • Prognosis
  • STAT5 Transcription Factor / genetics*
  • STAT5 Transcription Factor / metabolism*
  • Severity of Illness Index*
  • Tumor Suppressor Proteins / genetics*
  • Tumor Suppressor Proteins / metabolism*

Substances

  • Antigens, CD34
  • CALR protein, human
  • Calreticulin
  • STAT5 Transcription Factor
  • STAT5A protein, human
  • Tumor Suppressor Proteins
  • JAK2 protein, human
  • Janus Kinase 2

Grants and funding

This work was supported by: Associazione Italiana Ricerca sul Cancro (AIRC) project number 10005 “Special Program Molecular Clinical Oncology 5x1000” to Associazione Italiana per la Ricerca sul Cancro Gruppo Italiano Malattie Mieloproliferative (AGIMM) (A detailed description of the AGIMM project is available at http://www.progettoagimm.it), received by GB; by Ricerca Corrente Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo, project number 874, code number 08054517, received by VR (Vittorio Rosti) (www.sanmatteo.org); and by Ricerca Finalizzata Italian Ministry of Health, project number GR-2016-02363136, received by VA (Vittorio Abbonante). The funders had no role in study design, data collection and analysis, preparation of the manuscript or decision to publish.