Associations between gender, disease features and symptom burden in patients with myeloproliferative neoplasms: an analysis by the MPN QOL International Working Group

Holly L Geyer, Heidi Kosiorek, Amylou C Dueck, Robyn Scherber, Stefanie Slot, Sonja Zweegman, Peter Aw Te Boekhorst, Zhenya Senyak, Harry C Schouten, Federico Sackmann, Ana Kerguelen Fuentes, Dolores Hernández-Maraver, Heike L Pahl, Martin Griesshammer, Frank Stegelmann, Konstanze Döhner, Thomas Lehmann, Karin Bonatz, Andreas Reiter, Francoise BoyerGabriel Etienne, Jean-Christophe Ianotto, Dana Ranta, Lydia Roy, Jean-Yves Cahn, Claire N Harrison, Deepti Radia, Pablo Muxi, Norman Maldonado, Carlos Besses, Francisco Cervantes, Peter L Johansson, Tiziano Barbui, Giovanni Barosi, Alessandro M Vannucchi, Chiara Paoli, Francesco Passamonti, Bjorn Andreasson, Maria L Ferrari, Alessandro Rambaldi, Jan Samuelsson, Keith Cannon, Gunnar Birgegard, Zhijian Xiao, Zefeng Xu, Yue Zhang, Xiujuan Sun, Junqing Xu, Jean-Jacques Kiladjian, Peihong Zhang, Robert Peter Gale, Ruben A Mesa

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

The myeloproliferative neoplasms, including polycythemia vera, essential thrombocythemia and myelofibrosis, are distinguished by their debilitating symptom profiles, life-threatening complications and profound impact on quality of life. The role gender plays in the symptomatology of myeloproliferative neoplasms remains under-investigated. In this study we evaluated how gender relates to patients' characteristics, disease complications and overall symptom expression. A total of 2,006 patients (polycythemia vera=711, essential thrombocythemia=830, myelofibrosis=460, unknown=5) were prospectively evaluated, with patients completing the Myeloproliferative Neoplasm-Symptom Assessment Form and Brief Fatigue Inventory Patient Reported Outcome tools. Information on the individual patients' characteristics, disease complications and laboratory data was collected. Consistent with known literature, most female patients were more likely to have essential thrombocythemia (48.6% versus 33.0%; P<0.001) and most male patients were more likely to have polycythemia vera (41.8% versus 30.3%; P<0.001). The rate of thrombocytopenia was higher among males than females (13.9% versus 8.2%; P<0.001) and males also had greater red-blood cell transfusion requirements (7.3% versus 4.9%; P=0.02) with shorter mean disease duration (6.4 versus 7.2 years, P=0.03). Despite there being no statistical differences in risk scores, receipt of most therapies or prior complications (hemorrhage, thrombosis), females had more severe and more frequent symptoms for most individual symptoms, along with overall total symptom score (22.8 versus 20.3; P<0.001). Females had particularly high scores for abdominal-related symptoms (abdominal pain/discomfort) and microvascular symptoms (headache, fatigue, insomnia, concentration difficulties, dizziness; all P<0.01). Despite complaining of more severe symptom burden, females had similar quality of life scores to those of males. The results of this study suggest that gender contributes to the heterogeneity of myeloproliferative neoplasms by influencing phenotypic profiles and symptom expression.

Original languageEnglish
Pages (from-to)85-93
Number of pages9
JournalHaematologica-the Hematology Journal
Volume102
Issue number1
DOIs
Publication statusPublished - Jan 2017

Keywords

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Female
  • Humans
  • Male
  • Middle Aged
  • Myeloproliferative Disorders
  • Phenotype
  • Prognosis
  • Quality of Life
  • Sex Factors
  • Surveys and Questionnaires
  • Young Adult
  • Journal Article

Cite this

Geyer, H. L., Kosiorek, H., Dueck, A. C., Scherber, R., Slot, S., Zweegman, S., Te Boekhorst, P. A., Senyak, Z., Schouten, H. C., Sackmann, F., Fuentes, A. K., Hernández-Maraver, D., Pahl, H. L., Griesshammer, M., Stegelmann, F., Döhner, K., Lehmann, T., Bonatz, K., Reiter, A., ... Mesa, R. A. (2017). Associations between gender, disease features and symptom burden in patients with myeloproliferative neoplasms: an analysis by the MPN QOL International Working Group. Haematologica-the Hematology Journal, 102(1), 85-93. https://doi.org/10.3324/haematol.2016.149559