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Patient-Reported Symptoms from a Diverse Group of Subjects with Hypereosinophilic Syndrome
Sunday, March 6, 2016
South Exhibit Hall H (Convention Center)
Nicholas C. Kovacs, Linda Nelsen, Suyong Yun Kirby, Katy Benjamin, Olga Moshkovich, Nicole Holland-Thomas, MSN, RN, Amy D. Klion, MD, Paneez Khoury, MD, Jonathan Steinfeld
Rationale: Hypereosinophilic Syndromes (HES) are rare heterogeneous disorders with diverse clinical manifestations. Although specific organ involvement in HES may influence patients’ disease-related experiences, the patient-reported symptom profile has not been systematically described.  

Methods: Literature reviews and expert opinion were used to compile a 60-item HES symptom checklist. Subjects with diverse organ system involvement and flares requiring a change in therapy in the preceding year were consented on an IRB-approved protocol. Subjects completed the checklist and a semi-structured interview addressing HES-related symptoms, medication side effects, and symptom impact. 

Results: 19 HES subjects (median age 52 (range 28-76), 53% female, 79% Caucasian) from 13 US states were interviewed, including subjects with myeloproliferative and lymphocytic variant HES. Subjects had dermatologic (58%), gastrointestinal (53%), pulmonary (37%), sinus (37%), neurologic (32%) and cardiac (16%) involvement. On average, subjects had 2.5 organ systems involved (median 2 (range 1-4), and reported 13.8 symptoms (SD = 8.5, median 12 (range 2-33)). The most common symptoms were non-organ specific symptoms including fatigue (73%), weight changes (63%), itching (63%), and malaise (58%). Notably, only 32% of subjects had presumed neurologic involvement; however, 79% of subjects reported at least 1 neurological or cognitive symptom including numbness, extremity-related weakness, and cognitive problems. Similarly 58% of subjects complained of at least one pulmonary symptom including wheezing, coughing or shortness of breath, whereas physician-diagnosed pulmonary involvement was only present in 37% of subjects. 

Conclusions: Despite variable organ involvement, many HES subjects reported similar symptoms. Additionally, patient-reported symptoms and documented organ involvement were not always concordant.