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IMPACT OF SPLENOMEGALY ON MPN SYMPTOMS AND ASSOCIATION WITH CLINICAL FEATURES: AN ANALYSIS BY THE MPN QUALITY OF LIFE INTERNATIONAL STUDY GROUP
Author(s): ,
Holly Geyer
Affiliations:
Mayo Clinic,Phoenix,United States
,
Robyn Scherber
Affiliations:
OHSU,Portland,United States
,
Heidi Kosiorek
Affiliations:
MAYO CLINIC,Phoenix,United States
,
Amylou Dueck
Affiliations:
MAYO CLINIC,Phoenix,United States
,
Jean-Jacques Kiladjian
Affiliations:
Hospital Saint Louis,Paris,France
,
Stephanie Slot
Affiliations:
VU University Medical Center,Amsterdam,Netherlands
,
Sonja Zweegman
Affiliations:
VU University Medical Center,Amsterdam,Netherlands
,
Peter Boekhorst
Affiliations:
Erasmus Medical Cener,Rotterdam,Netherlands
,
Harry Schouten
Affiliations:
Maastricht University Medical Center,Maastricht,Netherlands
,
Federico Sackmann
Affiliations:
Fundaleu,Buenos Aires,Argentina
,
Ana Fuentes
Affiliations:
University Hospital La Paz,Madrid,Spain
,
Dolores Hernandez-Maraver
Affiliations:
University Hospital La Paz,Madrid,Spain
,
Heike Pahl
Affiliations:
University Hospital Freiburg,Freiburg,Germany
,
Frank Stegelmann
Affiliations:
University Hospital of Ulm,Ulm,Germany
,
Kostanze Doehner
Affiliations:
University Hospital of Ulm,Ulm,Germany
,
Karin Bonatz
Affiliations:
Medizinische Klinik,Mannheim,Germany
,
Andreas Reiter
Affiliations:
Medizinische Klinik,Mannheim,Germany
,
Francoise Boyer
Affiliations:
Centre Hospitalier Universitaire,Angers,France
,
Gabriel Etienne
Affiliations:
Centre Hospitalier Universitaire,Angers,France
,
Jean-Christophe Ianotto
Affiliations:
Hospitalier Universitaire,Brest,France
,
Dana Ranta
Affiliations:
Hospitalier Universitaire,Nancy,France
,
Lydia Roy
Affiliations:
Centre Hospitalier Universitaire,Poitiers,France
,
Jean-Yves Cahn
Affiliations:
Centre Hospitalier Universitaire,Grenoble,France
,
Claire Harrison
Affiliations:
Guy's and St. Thomas NHS Foundation Trust,London,United Kingdom
,
Deepti Radia
Affiliations:
Guy's and St. Thomas NHS Foundation Trust,London,United Kingdom
,
Pablo Muxi
Affiliations:
Hospital Britanico,Montevideo,Uruguay
,
Norman Maldonado
Affiliations:
University of Puerto Rico School of Medicine,San Juan,Puerto Rico
,
Carlos Besses
Affiliations:
Hospital del Mar,Barcelona,Spain
,
Francisco Cervantes
Affiliations:
University of Barcelona,Barcelona,Spain
,
Peter Johansson
Affiliations:
NU Hospital Organization,Uddevalla,Sweden
,
Giovanni Barosi
Affiliations:
IRCCS Policlinico S. Matteo Foundation,Pavia,Italy
,
Alessandro Vannucchi
Affiliations:
Ospedale di Circolo,Varese,Italy
,
Francesco Passamonti
Affiliations:
Fondazione IRCCS Policlinico San Matteo,Pavia,Italy
,
Bjorn Andreasson
Affiliations:
NU Hospital Organization,Uddavalla,Sweden
,
Jan Samuelsson
Affiliations:
Stockholm South Hospital,Stockholm,Sweden
,
Gunnar Birgegard
Affiliations:
University Hospital,Uppsala,Sweden
,
Xiujuan Sun
Affiliations:
Chinese Academy of Medical Sciences and Peking Union Medical College,Tianjin,China
,
Junqing Xu
Affiliations:
Chinese Academy of Medical Sciences and Peking Union Medical College,Tianjin,China
,
Peihong Zhang
Affiliations:
Chinese Academy of Medical Sciences & Peking Union Medical College,Tianjin,China
,
Zefeng Xu
Affiliations:
Chinese Academy of Medical Sciences and Peking Union Medical College,Tianjin,China
,
Tiziano Barbui
Affiliations:
Ospedali Riuniti di Bergamo,Bergamo,Italy
,
Tiziano Barbui
Affiliations:
Ospedali Riuniti di Bergamo,Bergamo,Italy
,
Zhenya Senyak
Affiliations:
MPN Forum,Ashville, North Carolina,United States
,
Martin Grieshammer
Affiliations:
Johannes Wesling Klinikum,Minden,Germany
,
Alessandro Rambaldi
Affiliations:
Ospedali Riuniti di Bergamo,Bergamo,Italy
,
Maria Ferrari
Affiliations:
Ospedali Riuniti di Bergamo,Bergamo,Italy
,
Thomas Lehmann
Affiliations:
University Hospital Basel,Basel,Switzerland
Ruben Mesa
Affiliations:
Mayo Clinic,Phoenix,United States
(Abstract release date: 05/19/16) EHA Library. Geyer H. 06/09/16; 132888; E1339
Dr. Holly L Geyer
Dr. Holly L Geyer
Contributions
Abstract
Abstract: E1339

Type: Eposter Presentation

Background
Splenomegaly (SM) is frequently observed in Myeloproliferative Neoplasms (MPNs).  Many MPN symptoms have been attributed to splenomegaly but studies exploring these associations are lacking.

Aims
We sought to evaluate how the presence of severe splenomegaly (>10cm below costal margin [BCM]) impacts patient symptomatology and relates to other clinical features.

Methods
Data was collected among an international cohort of MPN patients including polycythemia vera (PV), essential thrombocythemia (ET) and myelofibrosis (MF) without splenectomy as reported previously (Emanuel et al., JCO 2013).  Subjects completed BFI and MPN-SAF instruments.  Items were scored on a 0 (absent) to 10 (worst imaginable) scale. For individuals completing at least 6 of the 10 MPN-SAF TSS items, the survey was scored by multiplying the average score across items by 10 to achieve a 0 to 100 scaled score. Demographics, clinical features and symptom scores were compared between splenomegaly subgroups using ANOVA F-tests or chi-squared tests.  Splenomegaly was assessed by clinicians based on physical examination and reported as an estimated value BCM.  Patients were categorized into ‘No-SM’ (non-palpable), ‘Mild-SM’ (1-10cm) and ‘Severe-SM' (>10cm).

Results
Demographics:  A total of 2043 patients were included in the analysis (No-SM, n=1462; Mild-SM, n=404; Severe-SM, n=177) with a mean spleen size of 2.6 cm BCM (range 10.1-31.0 cm). Patients were of expected age (59.7 years) and similar gender distribution (54.6% female).  MPN subtypes included ET (41.9%), PV (35.2%) and MF (22.9%). The most prevalent prognostic risk group for each subtype was as follows: ET, intermediate (46.1%); MF, Int.-1 (54.9%); PV, High (49.2%). Compared to a history of prior thrombosis (20.3%), anemia (8.7%), leukopenia (9.9%), thrombocytopenia (11.0%), red blood cell transfusions (5.8%) and prior hemorrhage (5.0%) were relatively uncommon.Clinical Correlations:  When compared to No-SM populations, patients with Severe-SM were most likely to be >60 years (54.5% vs. 45.5%, p=0.015) and have MF (62.7% vs. 10.8%, [p<0.001]). Severe-SM patients were also most likely to suffer from anemia (29.3% vs. 4.0%), leukopenia (16.4% vs. 7.5%) and thrombocytopenia (34.2% vs. 7.1%, all p<0.001), along with prior hemorrhages (8% vs. 4.2%, p=0.029) and have the highest transfusion requirements (15.3% vs. 2.8%, p<0.001).  In contrast, No-SM patients were most likely to have a history of prior thrombosis (20.1% vs. 14.3%, p=0.046).  SM did not correlate with gender or individual prognostic risk categories.Symptoms:  Severe-SM was associated with the highest Total Symptom Scores (TSS, 28.5 vs. 16.3 in No-SM, p<0.001) and individual symptom scores for all items assessed, with the exception of fever and bone pain (both p>0.05; Figure 1). Highest scores were noted for fatigue (4.9 vs. 4.1, p=0.006), early satiety (3.6 vs. 2.3, p<0.001), inactivity (3.4 vs. 2.2, p<0.001), and impact on overall quality of life (3.8 vs. 2.7, p<0.001).  Abdominal discomfort (2.9 vs. 1.7, p<0.001) and weight loss (1.9 vs. 1.1, p<0.001) scores were of relatively low severity in comparison to other complaints.    

Conclusion
Spleen size directly correlates with symptoms unrelated to the abdominal cavity and overall MPN disease burden. The lack of association between spleen size and prognostic risk scores suggests low risk patients may still suffer from significant, undermanaged symptomatology.



Session topic: E-poster

Keyword(s): Essential Thrombocytemia, Myelofibrosis, Myeloproliferative disorder, Polycythemia vera
Abstract: E1339

Type: Eposter Presentation

Background
Splenomegaly (SM) is frequently observed in Myeloproliferative Neoplasms (MPNs).  Many MPN symptoms have been attributed to splenomegaly but studies exploring these associations are lacking.

Aims
We sought to evaluate how the presence of severe splenomegaly (>10cm below costal margin [BCM]) impacts patient symptomatology and relates to other clinical features.

Methods
Data was collected among an international cohort of MPN patients including polycythemia vera (PV), essential thrombocythemia (ET) and myelofibrosis (MF) without splenectomy as reported previously (Emanuel et al., JCO 2013).  Subjects completed BFI and MPN-SAF instruments.  Items were scored on a 0 (absent) to 10 (worst imaginable) scale. For individuals completing at least 6 of the 10 MPN-SAF TSS items, the survey was scored by multiplying the average score across items by 10 to achieve a 0 to 100 scaled score. Demographics, clinical features and symptom scores were compared between splenomegaly subgroups using ANOVA F-tests or chi-squared tests.  Splenomegaly was assessed by clinicians based on physical examination and reported as an estimated value BCM.  Patients were categorized into ‘No-SM’ (non-palpable), ‘Mild-SM’ (1-10cm) and ‘Severe-SM' (>10cm).

Results
Demographics:  A total of 2043 patients were included in the analysis (No-SM, n=1462; Mild-SM, n=404; Severe-SM, n=177) with a mean spleen size of 2.6 cm BCM (range 10.1-31.0 cm). Patients were of expected age (59.7 years) and similar gender distribution (54.6% female).  MPN subtypes included ET (41.9%), PV (35.2%) and MF (22.9%). The most prevalent prognostic risk group for each subtype was as follows: ET, intermediate (46.1%); MF, Int.-1 (54.9%); PV, High (49.2%). Compared to a history of prior thrombosis (20.3%), anemia (8.7%), leukopenia (9.9%), thrombocytopenia (11.0%), red blood cell transfusions (5.8%) and prior hemorrhage (5.0%) were relatively uncommon.Clinical Correlations:  When compared to No-SM populations, patients with Severe-SM were most likely to be >60 years (54.5% vs. 45.5%, p=0.015) and have MF (62.7% vs. 10.8%, [p<0.001]). Severe-SM patients were also most likely to suffer from anemia (29.3% vs. 4.0%), leukopenia (16.4% vs. 7.5%) and thrombocytopenia (34.2% vs. 7.1%, all p<0.001), along with prior hemorrhages (8% vs. 4.2%, p=0.029) and have the highest transfusion requirements (15.3% vs. 2.8%, p<0.001).  In contrast, No-SM patients were most likely to have a history of prior thrombosis (20.1% vs. 14.3%, p=0.046).  SM did not correlate with gender or individual prognostic risk categories.Symptoms:  Severe-SM was associated with the highest Total Symptom Scores (TSS, 28.5 vs. 16.3 in No-SM, p<0.001) and individual symptom scores for all items assessed, with the exception of fever and bone pain (both p>0.05; Figure 1). Highest scores were noted for fatigue (4.9 vs. 4.1, p=0.006), early satiety (3.6 vs. 2.3, p<0.001), inactivity (3.4 vs. 2.2, p<0.001), and impact on overall quality of life (3.8 vs. 2.7, p<0.001).  Abdominal discomfort (2.9 vs. 1.7, p<0.001) and weight loss (1.9 vs. 1.1, p<0.001) scores were of relatively low severity in comparison to other complaints.    

Conclusion
Spleen size directly correlates with symptoms unrelated to the abdominal cavity and overall MPN disease burden. The lack of association between spleen size and prognostic risk scores suggests low risk patients may still suffer from significant, undermanaged symptomatology.



Session topic: E-poster

Keyword(s): Essential Thrombocytemia, Myelofibrosis, Myeloproliferative disorder, Polycythemia vera

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