Distinct clustering of symptomatic burden among myeloproliferative neoplasm patients: retrospective assessment in 1470 patients

被引:70
作者
Geyer, Holly L. [1 ]
Scherber, Robyn M. [1 ]
Dueck, Amylou C. [2 ]
Kiladjian, Jean-Jacques [3 ]
Xiao, Zhijian [4 ,5 ,6 ,7 ]
Slot, Stefanie [8 ]
Zweegman, Sonja [8 ]
Sackmann, Federico [9 ]
Kerguelen Fuentes, Ana [10 ]
Hernandez-Maraver, Dolores [10 ]
Doehner, Konstanze [11 ]
Harrison, Claire N. [12 ]
Radia, Deepti [12 ]
Muxi, Pablo [13 ]
Besses, Carlos [14 ]
Cervantes, Francisco [15 ]
Johansson, Peter L. [16 ]
Andreasson, Bjorn [16 ]
Rambaldi, Alessandro [17 ]
Barbui, Tiziano [17 ]
Vannucchi, Alessandro M. [18 ]
Passamonti, Francesco [19 ]
Samuelsson, Jan [20 ]
Birgegard, Gunnar [21 ]
Mesa, Ruben A. [22 ]
机构
[1] Mayo Clin, Div Hosp Internal Med, Scottsdale, AZ 85259 USA
[2] Mayo Clin, Biostat Sect, Scottsdale, AZ 85259 USA
[3] Hosp St Louis, Clin Invest Ctr, Paris, France
[4] Chinese Acad Med Sci, Inst Hematol, Myelodysplast Syndrome & Myeloproliferat Neoplasi, Tianjin, Peoples R China
[5] Chinese Acad Med Sci, Blood Dis Hosp, Tianjin, Peoples R China
[6] Chinese Acad Med Sci, Inst Hematol, State Key Lab Expt Hematol, Tianjin, Peoples R China
[7] Peking Union Med Coll, Tianjin, Peoples R China
[8] Vrije Univ Amsterdam, Med Ctr, Dept Hematol, Amsterdam, Netherlands
[9] Fundaleu, Buenos Aires, DF, Argentina
[10] Univ Hosp La Paz, Dept Haematol, Madrid, Spain
[11] Univ Hosp Ulm, Dept Internal Med 3, Ulm, Germany
[12] Guys & St Thomas Natl Hlth Serv Fdn Trust, Dept Haematol, London, England
[13] Hosp Britan, Unidadde Hematol, Montevideo, Uruguay
[14] Hosp del Mar, Dept Hematol, Barcelona, Spain
[15] Univ Barcelona, Inst Invest Biomed August Pi & Sunyer, Hosp Clin, Dept Hematol, E-08007 Barcelona, Spain
[16] Nephrourol Hosp Org, Uddevalla, Sweden
[17] Azienda Osped Papa Giovanni XXIII, Hematol Unit, Bergamo, Italy
[18] Osped Circolo Varese, Hematol Div Hematol, Varese, Italy
[19] Univ Pavia, Fdn IRCCS Policlin San Matteo, Dept Hematol, I-27100 Pavia, Italy
[20] Stockholm South Hosp, Dept Internal Med, Stockholm, Sweden
[21] Univ Uppsala Hosp, Dept Hematol, Uppsala, Sweden
[22] Mayo Clin, Dept Hematol Oncol, Scottsdale, AZ 85259 USA
关键词
QUALITY-OF-LIFE; ESSENTIAL THROMBOCYTHEMIA; POLYCYTHEMIA-VERA; SCORING SYSTEM; MYELOFIBROSIS; RUXOLITINIB; DISORDERS; MUTATIONS; FATIGUE; TRIAL;
D O I
10.1182/blood-2013-09-527903
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Symptom burden in myeloproliferative neoplasms (MPNs) is heterogeneous even among patients within the same MPN diagnosis. Using cluster analysis from prospectively gathered symptom burden data in 1470 international patients with essential thrombocythemia (ET), polycythemia vera (PV), or myelofibrosis (MF), we assessed for the presence of clusters and relationship to disease features and prognosis. In MF (4 clusters identified), clusters significantly differed by Dynamic International Prognostic Scoring System (DIPSS) risk (P < .001), leukopenia (P = .009), thrombocytopenia (P < .001), and spleen size (P = .02). Although an association existed between clusters and DIPSS risk, high symptom burden was noted in some low and intermediate-1-risk MF patients. In PV (5 clusters identified), total symptom score increased across clusters (P < .001), but clusters did not significantly differ by PV risk or the risk assessment variable of age. Among ET patients (5 clusters identified), clusters differed by gender (P = .04), anemia (P = .01), and prior hemorrhage (P = .047). Total symptom score increased across clusters (P < .001), but clusters did not significantly differ by International Prognostic Score for ET risk including the risk assessment variables. Significant symptom heterogeneity exists within each MPN subtype, sometimes independent of disease features or prognosis.
引用
收藏
页码:3803 / 3810
页数:8
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