Chronic lymphocytic leukemia and myeloproliferative neoplasms concurrently diagnosed: clinical and biological characteristics

Leuk Lymphoma. 2016 May;57(5):1054-9. doi: 10.3109/10428194.2015.1092527. Epub 2015 Oct 19.

Abstract

Chronic lymphocytic leukemia (CLL) and myeloproliferative neoplasms (MPN) may occur concomitantly. However, little is known about the pathobiological characteristics and interaction between the neoplastic clones in these rare cases of coinciding malignancies. We retrospectively examined the clinical and biological characteristics of 13 patients with concomitant CLL and MPN--eight primary myelofibrosis (PMF), three essential thrombocytosis (ET), and two polycythemia vera (PV)--who presented to our institution between 1998 and 2014, and tested all patients for MPN-specific aberrations, such as JAK2, MPL and CALR mutations. Along with epidemiological and molecular characterization of this rare condition, we found that JAK2 mutation can be detected 9 years prior to PMF diagnosis, suggesting that PMF clinical phenotype may require several years to develop and CLL/MPN clinical co-occurrence might be sustained by common molecular events. Some features of these patients suggest that pathobiologies of these diseases might be intertwined.

Keywords: CALR; Chronic lymphocytic leukemia; JAK2; MPL; myeloproliferative neoplasms; primary myelofibrosis.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Female
  • Follow-Up Studies
  • Humans
  • Immunoglobulin Heavy Chains / genetics
  • Janus Kinase 2 / genetics
  • Leukemia, Lymphocytic, Chronic, B-Cell / complications*
  • Leukemia, Lymphocytic, Chronic, B-Cell / diagnosis*
  • Leukemia, Lymphocytic, Chronic, B-Cell / drug therapy
  • Leukemia, Lymphocytic, Chronic, B-Cell / genetics
  • Male
  • Middle Aged
  • Mutation
  • Myeloproliferative Disorders / complications*
  • Myeloproliferative Disorders / diagnosis*
  • Myeloproliferative Disorders / drug therapy
  • Myeloproliferative Disorders / genetics
  • Neoplasm Staging
  • Patient Outcome Assessment
  • Polycythemia Vera
  • Primary Myelofibrosis
  • Receptors, Thrombopoietin / genetics
  • Retrospective Studies
  • Thrombocythemia, Essential

Substances

  • Immunoglobulin Heavy Chains
  • Receptors, Thrombopoietin
  • MPL protein, human
  • JAK2 protein, human
  • Janus Kinase 2