Symptomatic Profiles of Patients With Polycythemia Vera: Implications of Inadequately Controlled Disease.
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Geyer Holly, Scherber Robyn, Kosiorek Heidi, Dueck Amylou C, Kiladjian Jean-Jacques, Xiao Zhijian, Slot Stefanie, Zweegman Sonja, Sackmann Federico, Fuentes Ana Kerguelen, Hernández-Maraver Dolores, Döhner Konstanze, Harrison Claire N, Radia Deepti, Muxi Pablo, Besses Carlos, Cervantes Francisco, Johansson Peter L, Andreasson Bjorn, Rambaldi Alessandro, Barbui Tiziano, Bonatz Karin, Reiter Andreas, Boyer Francoise, Etienne Gabriel, Ianotto Jean-Christophe, Ranta Dana, Roy Lydia, Cahn Jean-Yves, Maldonado Norman, Barosi Giovanni, Ferrari Maria L, Gale Robert Peter, Birgegard Gunnar, Xu Zefeng, Zhang Yue, Sun Xiujuan, Xu Junqing, Zhang Peihong, te Boekhorst Peter A W, Commandeur Suzan, Schouten Harry, Pahl Heike L, Griesshammer Martin, Stegelmann Frank, Lehmann Thomas, Senyak Zhenya, Vannucchi Alessandro M, Passamonti Francesco, Samuelsson Jan, Mesa Ruben A
Аннотация
PATIENTS AND METHODS: Through prospective evaluation of 1,334 patients with PV who had characterized symptom burden, we assessed patient demographics, laboratory data, and the presence of splenomegaly by disease feature (ie, known HU use, known phlebotomy requirements, splenomegaly).
RESULTS: The presence of each feature in itself is associated with a moderately high symptom burden (MPN symptom assessment form [SAF] total symptom score [TSS] range, 27.7 to 29.2) that persists independent of PV risk category. In addition, symptoms incrementally increase in severity with the addition of other features. Patients with PV who had all three features (PV-HUPS) faced the highest total score (MPN-SAF TSS, 32.5) but had similar individual symptom scores to patients with known HU use (PV-HU), known phlebotomy (PV-P), and splenomegaly (PV-S).
CONCLUSION: The results of this study suggest that patients with PV who have any one of the features in question (known HU use, known phlebotomy, or splenomegaly) have significant PV-associated symptoms. Furthermore, it demonstrates that many PV symptoms remain severe independent of the number of features present.