Symptomatic profiles of patients with polycythemia vera: Implications of inadequately controlled disease

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

Research output: Contribution to journalArticle

28 Citations (Scopus)

Abstract

Purpose. Polycythemia vera (PV) is amyeloproliferative neoplasm (MPN) associatedwith disabling symptoms and a heightened risk of life-threatening complications. Recent studies have demonstrated the effectiveness of JAK inhibitor therapy in patients with PV patients who have a history of prior hydroxyurea (HU) use (including resistance or intolerance), phlebotomy requirements, and palpable splenomegaly. We aimed to determine how these features contribute alone and in aggregate to the PV symptom burden. 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 patientswith PVwho 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.

Original languageEnglish
Pages (from-to)151-159
Number of pages9
JournalJournal of Clinical Oncology
Volume34
Issue number2
DOIs
Publication statusPublished - Jan 10 2016

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Polycythemia Vera
Hydroxyurea
Splenomegaly
Phlebotomy
Symptom Assessment
Demography

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

Geyer, H., Scherber, R., Kosiorek, H., Dueck, A. C., Kiladjian, J. J., Xiao, Z., ... Mesa, R. A. (2016). Symptomatic profiles of patients with polycythemia vera: Implications of inadequately controlled disease. Journal of Clinical Oncology, 34(2), 151-159. https://doi.org/10.1200/JCO.2015.62.9337

Symptomatic profiles of patients with polycythemia vera : Implications of inadequately controlled disease. / 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.

In: Journal of Clinical Oncology, Vol. 34, No. 2, 10.01.2016, p. 151-159.

Research output: Contribution to journalArticle

Geyer, H, Scherber, R, Kosiorek, H, Dueck, AC, Kiladjian, JJ, Xiao, Z, Slot, S, Zweegman, S, Sackmann, F, Fuentes, AK, Hernández-Maraver, D, Döhner, K, Harrison, CN, Radia, D, Muxi, P, Besses, C, Cervantes, F, Johansson, PL, Andreasson, B, Rambaldi, A, Barbui, T, Bonatz, K, Reiter, A, Boyer, F, Etienne, G, Ianotto, JC, Ranta, D, Roy, L, Cahn, JY, Maldonado, N, Barosi, G, Ferrari, ML, Gale, RP, Birgegard, G, Xu, Z, Zhang, Y, Sun, X, Xu, J, Zhang, P, Te Boekhorst, PAW, Commandeur, S, Schouten, H, Pahl, HL, Griesshammer, M, Stegelmann, F, Lehmann, T, Senyak, Z, Vannucchi, AM, Passamonti, F, Samuelsson, J & Mesa, RA 2016, 'Symptomatic profiles of patients with polycythemia vera: Implications of inadequately controlled disease', Journal of Clinical Oncology, vol. 34, no. 2, pp. 151-159. https://doi.org/10.1200/JCO.2015.62.9337
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. / Symptomatic profiles of patients with polycythemia vera : Implications of inadequately controlled disease. In: Journal of Clinical Oncology. 2016 ; Vol. 34, No. 2. pp. 151-159.
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abstract = "Purpose. Polycythemia vera (PV) is amyeloproliferative neoplasm (MPN) associatedwith disabling symptoms and a heightened risk of life-threatening complications. Recent studies have demonstrated the effectiveness of JAK inhibitor therapy in patients with PV patients who have a history of prior hydroxyurea (HU) use (including resistance or intolerance), phlebotomy requirements, and palpable splenomegaly. We aimed to determine how these features contribute alone and in aggregate to the PV symptom burden. 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 patientswith PVwho 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.",
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T1 - Symptomatic profiles of patients with polycythemia vera

T2 - Implications of inadequately controlled disease

AU - Geyer, Holly

AU - Scherber, Robyn

AU - Kosiorek, Heidi

AU - Dueck, Amylou C.

AU - Kiladjian, Jean Jacques

AU - Xiao, Zhijian

AU - Slot, Stefanie

AU - Zweegman, Sonja

AU - Sackmann, Federico

AU - Fuentes, Ana Kerguelen

AU - Hernández-Maraver, Dolores

AU - Döhner, Konstanze

AU - Harrison, Claire N.

AU - Radia, Deepti

AU - Muxi, Pablo

AU - Besses, Carlos

AU - Cervantes, Francisco

AU - Johansson, Peter L.

AU - Andreasson, Bjorn

AU - Rambaldi, Alessandro

AU - Barbui, Tiziano

AU - Bonatz, Karin

AU - Reiter, Andreas

AU - Boyer, Francoise

AU - Etienne, Gabriel

AU - Ianotto, Jean Christophe

AU - Ranta, Dana

AU - Roy, Lydia

AU - Cahn, Jean Yves

AU - Maldonado, Norman

AU - Barosi, Giovanni

AU - Ferrari, Maria L.

AU - Gale, Robert Peter

AU - Birgegard, Gunnar

AU - Xu, Zefeng

AU - Zhang, Yue

AU - Sun, Xiujuan

AU - Xu, Junqing

AU - Zhang, Peihong

AU - Te Boekhorst, Peter A W

AU - Commandeur, Suzan

AU - Schouten, Harry

AU - Pahl, Heike L.

AU - Griesshammer, Martin

AU - Stegelmann, Frank

AU - Lehmann, Thomas

AU - Senyak, Zhenya

AU - Vannucchi, Alessandro M.

AU - Passamonti, Francesco

AU - Samuelsson, Jan

AU - Mesa, Ruben A.

PY - 2016/1/10

Y1 - 2016/1/10

N2 - Purpose. Polycythemia vera (PV) is amyeloproliferative neoplasm (MPN) associatedwith disabling symptoms and a heightened risk of life-threatening complications. Recent studies have demonstrated the effectiveness of JAK inhibitor therapy in patients with PV patients who have a history of prior hydroxyurea (HU) use (including resistance or intolerance), phlebotomy requirements, and palpable splenomegaly. We aimed to determine how these features contribute alone and in aggregate to the PV symptom burden. 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 patientswith PVwho 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.

AB - Purpose. Polycythemia vera (PV) is amyeloproliferative neoplasm (MPN) associatedwith disabling symptoms and a heightened risk of life-threatening complications. Recent studies have demonstrated the effectiveness of JAK inhibitor therapy in patients with PV patients who have a history of prior hydroxyurea (HU) use (including resistance or intolerance), phlebotomy requirements, and palpable splenomegaly. We aimed to determine how these features contribute alone and in aggregate to the PV symptom burden. 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 patientswith PVwho 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.

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