title: Associations between gender, disease features and symptom burden in patients with myeloproliferative neoplasms: an analysis by the MPN QOL International Working Group.
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.
authors.affiliation: Department of Internal Medicine, Mayo Clinic, Scottsdale, AZ, USA mesa.ruben@mayo.edu geyer.holly@mayo.edu.
authors.forename: Holly L
authors.initials: HL
authors.lastname: Geyer
authors.affiliation: Section of Biostatistics, Mayo Clinic, Scottsdale, AZ, USA.
authors.forename: Heidi
authors.initials: H
authors.lastname: Kosiorek
authors.affiliation: Section of Biostatistics, Mayo Clinic, Scottsdale, AZ, USA.
authors.forename: Amylou C
authors.initials: AC
authors.lastname: Dueck
authors.affiliation: Oregon Health Sciences University, Portland, OR, USA.
authors.forename: Robyn
authors.initials: R
authors.lastname: Scherber
authors.affiliation: Department of Hematology, VU University Medical Center, Amsterdam, the Netherlands.
authors.forename: Stefanie
authors.initials: S
authors.lastname: Slot
authors.affiliation: Department of Hematology, VU University Medical Center, Amsterdam, the Netherlands.
authors.forename: Sonja
authors.initials: S
authors.lastname: Zweegman
authors.affiliation: Department of Hematology, Erasmus MC, Rotterdam, the Netherlands.
authors.forename: Peter Aw
authors.initials: PA
authors.lastname: Te Boekhorst
authors.affiliation: MPN Forum, Ashville, NC, USA.
authors.forename: Zhenya
authors.initials: Z
authors.lastname: Senyak
authors.affiliation: Department of Hematology, MUMC, Maastricht, the Netherlands.
authors.forename: Harry C
authors.initials: HC
authors.lastname: Schouten
authors.affiliation: Fundaleu, Buenos Aires, Argentina.
authors.forename: Federico
authors.initials: F
authors.lastname: Sackmann
authors.affiliation: Department of Haematology, University Hospital La Paz, Madrid, Spain.
authors.forename: Ana Kerguelen
authors.initials: AK
authors.lastname: Fuentes
authors.affiliation: Department of Haematology, University Hospital La Paz, Madrid, Spain.
authors.forename: Dolores
authors.initials: D
authors.lastname: Hernández-Maraver
authors.affiliation: Department of Molecular Hematology, University Hospital Freiburg, Germany.
authors.forename: Heike L
authors.initials: HL
authors.lastname: Pahl
authors.affiliation: Johannes Wesling Klinikum, Minden, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Griesshammer
authors.affiliation: Department of Internal Medicine III, University Hospital of Ulm, Germany.
authors.forename: Frank
authors.initials: F
authors.lastname: Stegelmann
authors.affiliation: Department of Internal Medicine III, University Hospital of Ulm, Germany.
authors.forename: Konstanze
authors.initials: K
authors.lastname: Döhner
authors.affiliation: Hematology Department, University Hospital, Basel, Switzerland.
authors.forename: Thomas
authors.initials: T
authors.lastname: Lehmann
authors.affiliation: Medizinische Klinik, Universitätsmedizin, Mannheim, Germany.
authors.forename: Karin
authors.initials: K
authors.lastname: Bonatz
authors.affiliation: Medizinische Klinik, Universitätsmedizin, Mannheim, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Reiter
authors.affiliation: Centre Hospitalier Universitaire, Angers, France.
authors.forename: Francoise
authors.initials: F
authors.lastname: Boyer
authors.affiliation: Institut Bergonie, Bordeaux, France.
authors.forename: Gabriel
authors.initials: G
authors.lastname: Etienne
authors.affiliation: Centre Hospitalier Universitaire, Brest, France.
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Ianotto
authors.affiliation: Hospitalier Universitaire, Nancy, France.
authors.forename: Dana
authors.initials: D
authors.lastname: Ranta
authors.affiliation: Centre Hospitalier Universitaire, Poitiers, France.
authors.forename: Lydia
authors.initials: L
authors.lastname: Roy
authors.affiliation: Centre Hospitalier Universitaire, Grenoble, France.
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Cahn
authors.affiliation: Department of Haematology, Guy's and St. Thomas NHS Foundation Trust, London, UK.
authors.forename: Claire N
authors.initials: CN
authors.lastname: Harrison
authors.affiliation: Department of Haematology, Guy's and St. Thomas NHS Foundation Trust, London, UK.
authors.forename: Deepti
authors.initials: D
authors.lastname: Radia
authors.affiliation: Unidadde Hematología, Hospital Británico, Montevideo, Uruguay.
authors.forename: Pablo
authors.initials: P
authors.lastname: Muxi
authors.affiliation: University of Puerto Rico School of Medicine, San Juan, Puerto Rico.
authors.forename: Norman
authors.initials: N
authors.lastname: Maldonado
authors.affiliation: Hematology Department, Hospital del Mar, Barcelona, Spain.
authors.forename: Carlos
authors.initials: C
authors.lastname: Besses
authors.affiliation: Hematology Department, Hospital Clínic, IDIBAPS, University of Barcelona, Spain.
authors.forename: Francisco
authors.initials: F
authors.lastname: Cervantes
authors.affiliation: Internal Medicine, NU Hospital Organization, Uddevalla, Sweden.
authors.forename: Peter L
authors.initials: PL
authors.lastname: Johansson
authors.affiliation: Research Foundation (FROM), Hospital Papa Giovanni XXIII, Bergamo, Italy.
authors.forename: Tiziano
authors.initials: T
authors.lastname: Barbui
authors.affiliation: Laboratory of Clinical Epidemiology, IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Barosi
authors.affiliation: Center for Research and Innovation of Myeloproliferative Neoplasms, University of Florence, Italy.
authors.forename: Alessandro M
authors.initials: AM
authors.lastname: Vannucchi
authors.affiliation: Center for Research and Innovation of Myeloproliferative Neoplasms, University of Florence, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Paoli
authors.affiliation: Ematologia, Dipartimento di Medicina Clinica e Sperimentale, University of Insubria, Varese, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Passamonti
authors.affiliation: Internal Medicine, NU Hospital Organization, Uddevalla, Sweden.
authors.forename: Bjorn
authors.initials: B
authors.lastname: Andreasson
authors.affiliation: Biol. Sci., Ospedali Riuniti di Bergamo, Italy.
authors.forename: Maria L
authors.initials: ML
authors.lastname: Ferrari
authors.affiliation: Research Foundation (FROM), Hospital Papa Giovanni XXIII, Bergamo, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Rambaldi
authors.affiliation: Department of Internal Medicine, Stockholm South Hospital, Sweden.
authors.forename: Jan
authors.initials: J
authors.lastname: Samuelsson
authors.affiliation: Department of Internal Medicine, Mayo Clinic, Scottsdale, AZ, USA.
authors.forename: Keith
authors.initials: K
authors.lastname: Cannon
authors.affiliation: Department of Hematology, University Hospital, Uppsala, Sweden.
authors.forename: Gunnar
authors.initials: G
authors.lastname: Birgegard
authors.affiliation: MDS and MPN Center, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin, China.
authors.forename: Zhijian
authors.initials: Z
authors.lastname: Xiao
authors.affiliation: MDS and MPN Center, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin, China.
authors.forename: Zefeng
authors.initials: Z
authors.lastname: Xu
authors.affiliation: MDS and MPN Center, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin, China.
authors.forename: Yue
authors.initials: Y
authors.lastname: Zhang
authors.affiliation: MDS and MPN Center, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin, China.
authors.forename: Xiujuan
authors.initials: X
authors.lastname: Sun
authors.affiliation: MDS and MPN Center, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin, China.
authors.forename: Junqing
authors.initials: J
authors.lastname: Xu
authors.affiliation: Clinical Investigation Center, Hospital Saint-Louis, Paris, France.
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Kiladjian
authors.affiliation: Department of Pathology, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China.
authors.forename: Peihong
authors.initials: P
authors.lastname: Zhang
authors.affiliation: Imperial College, London, UK.
authors.forename: Robert Peter
authors.initials: RP
authors.lastname: Gale
authors.affiliation: Department of Hematology and Oncology, Mayo Clinic, Scottsdale, AZ, USA mesa.ruben@mayo.edu geyer.holly@mayo.edu.
authors.forename: Ruben A
authors.initials: RA
authors.lastname: Mesa
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000175:diagnosis / Q000453:epidemiology* / Q000401:mortality; D010641:Phenotype; D011379:Prognosis; D011788:Quality of Life; D012737:Sex Factors; D011795:Surveys and Questionnaires; D055815:Young Adult