The Spectrum of Hypereosinophilia and Associated Clonal Disorders - A Real-World Data Based on Combined Retrospective and Prospective Analysis from a Tropical Setting.

Objective: To determine the frequency, etiological spectrum and treatment outcome of hypereosinophilia (HE) and hypereosinophilic syndromes (HES) in a tropical setting.

Methods: A retrospective analysis of hospital data of five years (January 2009 to December 2013) and a comprehensive prospective evaluation of patients presenting with HE/HES over a period of 33 months (January 2014 to September 2016) was performed.

Results: HE/HES was diagnosed in a total of 125 patients during the study period with an estimated prevalence of 0.5-1 case per 100,000 population in our hospital settings. 41 patients were excluded from the final analysis due to lack of sufficient data. Infections, especially helminths were the commonest cause (34%) followed by primary/clonal HE/HES (24%) and reactive HE/HES secondary to various clonal disorders (14.3%). A lymphocytic variant of HES and FIP1L1-PDGFRA positive HES were diagnosed in 3.6% each. Imatinib-responsive BCR-ABL1 negative HE/HES constitute 7.1% in our patients. None of the clinical or routine laboratory features including the age of patients, duration of HE, presence or absence of organomegaly, hemoglobin levels, eosinophil %, absolute eosinophil count, total leukocyte count, platelet counts, serum IgE levels or presence of myelofibrosis could predict or exclude malignancy in patients with HE/HES. The absence of blasts in peripheral blood or the absence of >5% blasts in bone marrow does not exclude primary/clonal HES.

Conclusions: An underlying malignancy (Primary HE/HES and neoplasms leading to reactive HES; 35.7%) is diagnosed with nearly equal frequency compared to infections (34.5%) in tropical settings. There are no hematological or serological parameters, which can reliably be used to exclude an underlying malignancy, necessitating a thorough follow-up and comprehensive work-up in patients with HE/HES.