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. 2018 Sep 1;10(1):e2018052.
doi: 10.4084/MJHID.2018.052. eCollection 2018.

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

Sreejesh Sreedharanunni  1 , Neelam Varma  1 , Man Updesh Singh Sachdeva  1 , Shano Naseem  1 , Pankaj Malhotra  2 , Deepak Bansal  3 , Amita Trehan  3 , Subhash Varma  2
Affiliations

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

Sreejesh Sreedharanunni et al. Mediterr J Hematol Infect Dis. .

Abstract

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.

Keywords: FIP1L1-PDGFRA; Hypereosinophilia; Hypereosinophilic syndromes; Imatinib responsive hypereosinophilia; clonal hypereosinophilia; lymphocytic variant of hypereosinophilia.

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Conflict of interest statement

Competing interests: The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1
Spectrum of causes of hypereosinophilia/hypereosinophilic syndrome (EGPA - Eosinophilic granulomatosis with polyangiitis; DRESS - drug reaction with eosinophilia and systemic symptoms; L-HES – lymphocytic variant of HES; HE/HES-N – Neoplastic HE/HES; HE-US – HE of undetermined significance; I-HES – idiopathic HES).
Figure 2
Figure 2
(A) Peripheral blood smear of a patient with Imatinib responsive hypereosinophilia showing bilobed and trilobed eosinophils and myelocytes. (40x, May Grunwald Giemsa stain). (B) Bone marrow trephine biopsy showing hypercellular marrow spaces with marked increase in eosinophils and precursors (Hematoxylin and eosin, 40x). (C). Reticulin stain shows myelofibrosis (Grade 2/3; WHO). (D). Interphase FISH using tricolor FIP1L1-PDGFRA rearrangement probe showing deletion of CHIC2 gene (orange signals) in a case of FIP1L1-PDGFRA positive HES.
Figure 3
Figure 3
The spectrum of therapeutic agents used in the treatment of hypereosinophilic syndromes (n=67) (DEC – diethylcarbamazine).
See this image and copyright information in PMC

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