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 Table of Contents  
Year : 2014  |  Volume : 39  |  Issue : 4  |  Page : 256-257

Fulminant course of a case of idiopathic hypereosinophilic syndrome

Department of Hematology, All India Institute of Medical Sciences, New Delhi, India

Date of Submission15-Aug-2014
Date of Acceptance21-Nov-2014
Date of Web Publication25-Mar-2015

Correspondence Address:
Abhishek Purohit
Department of Hematology, All India Institute of Medical Sciences, New Delhi 110029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1110-1067.153976

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How to cite this article:
Somasundaram V, Purohit A, Aggarwal M, Seth T, Mahapatra M, Pati HP, Saxena R. Fulminant course of a case of idiopathic hypereosinophilic syndrome. Egypt J Haematol 2014;39:256-7

How to cite this URL:
Somasundaram V, Purohit A, Aggarwal M, Seth T, Mahapatra M, Pati HP, Saxena R. Fulminant course of a case of idiopathic hypereosinophilic syndrome. Egypt J Haematol [serial online] 2014 [cited 2022 Sep 30];39:256-7. Available from: http://www.ehj.eg.net/text.asp?2014/39/4/256/153976

Ever since Chusid et al. [1] proposed the diagnostic criteria for hypereosinophilic syndrome (HES), our understanding of these diseases has drastically changed, along with new classifications that characterize patients with marked eosinophilia. In addition, the availability of targeted therapy has improved the outcome of a subset of these patients, although the prognosis may be dismal at times. In this short communication, we bring to your notice one such patient with idiopathic HES who had a progressive fulminant fatal course.

A 14-year-old boy, resident of Nepal, was symptomatic for nearly a year with history of fever, loss of appetite, and weight. On evaluation, he was found to have persistent eosinophilia and was started on steroids and hydroxyurea. Subsequently, he had an intestinal perforation for which he was operated, and histopathology of tissue from the intestine showed dense eosinophilic infiltrate. Later, he received antitubercular therapy for 2 months followed by imatinib, vincristine, and steroids. At this point, he attended our outpatient department with complaints of fever, cough, respiratory distress, and generalized swelling of the body. On examination, he had edema over both upper and lower limb, tachycardia with pulse rate of 139/min, and respiratory rate of 40/min with normal blood pressure and oxygen saturation. He had right-sided pleural effusion with crepitations bilaterally and hepatomegaly of 2 cm below the right costal margin. His hemogram showed hemoglobin of 9.3 g/dl, total leukocyte count 80 000/mm 3 , and platelet count of 405 000/mm 3 . Peripheral smear examination revealed 86% eosinophils [Figure 1] with an absolute eosinophil count of 68 600/mm 3 ; however, there were no blasts or atypical cells or hemoparasites. Repeated stool examination was negative for ova and cysts. Serum biochemistry profile was within normal limits. Pleural fluid examination was transudative in nature. His bone marrow examination showed increase in eosinophils and their precursors [Figure 2] and blasts were less than 5%. There were no evidence of atypical cells, parasites, or granuloma, and cytogenetic studies showed normal male karyotype. RT-PCR for BCR-ABL 1, JAK2 mutation analysis, and PDGFR A and B rearrangements were negative. Serum tryptase level was not high; however, immunoglobulin G and immunoglobulin E levels were elevated. Work-up for autoimmune diseases including c- and p-ANCA was negative. Echocardiogram showed mild left ventricular dysfunction, and Doppler studies showed bilateral axillary and right subclavian vein thrombosis. Computed tomographic scan of the chest revealed features of bilateral pneumonia. Bronchoalveolar lavage and biopsy of lung lesion were performed, both of which revealed increase in eosinophils; however, there was no evidence of fungal elements, parasites, or malignancy. Patient was started on first-line antibiotics, antifungals, and intravenous albumin, and chest tube was put. For sustained eosinophilia, imatinib and hydroxyurea along with prednisone were given. Clexane and warfarin were started for deep vein thrombosis. The total leukocyte count and absolute eosinophil count reduced; however, the respiratory distress persisted and hence interferon-a and methyl prednisolone were added on alternate days. However, even with these different possible therapeutic approaches, the patient could not be salvaged and he succumbed to respiratory failure following eosinophilic pneumonia after being admitted for nearly 40 days.
Figure 1: Peripheral blood shows increase in eosinophils with some showing abnormal morphology (Jenner Giemsa stain, ×1000).

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Figure 2: Bone marrow aspirate shows myeloid preponderance with increase in eosinophils and its precursors; however, there is no increase in blasts or atypical cells (Jenner Giemsa stain, ×1000).

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HES are a heterogeneous group of uncommon disorders characterized by marked peripheral eosinophilia and end organ manifestations attributable to the eosinophilia [2]. Recent diagnostic advances and the development of novel targeted therapies, including tyrosine kinase inhibitors and humanized monoclonal antibodies, have increased the complexity of therapeutic decisions in HESs. Our patient had a sustained eosinophilia for more than a year; all possible etiologies were ruled out by the investigations listed above and he had end organ damage in the form of eosinophilic pneumonia, eosinophilic enteritis with perforation, mild left ventricular dysfunction, and bilateral axillary, right subclavian vein thrombosis, thus fulfilling the criteria for idiopathic HES.

Although usually diagnosed between the ages of 20 and 50 years, idiopathic hypereosinophilia or chronic eosinophilic leukemia may arise at the extremes of age, with infrequent cases being described in infants and children [3].

The aim of this short communication was to highlight some of the uncommon manifestations of this rare entity in our case, which include the young age of 14 years at presentation and rarer sites of involvement such as the intestine, which in the literature range to a cumulative incidence of 29%.

The common gastrointestinal symptoms in a case of HES include abdominal pain, diarrhea, nausea, and vomiting. Eosinophilic gastritis, enterocolitis, or colitis may be present, and the latter may be associated with ascites, if eosinophilic infiltrates involve deeper layers of the intestinal wall [4]. HES presenting with intestinal perforation is seldom encountered, although sporadic case reports are available in the literature [5]. Venous thrombosis in HES is rare in which only a handful of case reports and case series were published earlier [6],[7],[8], although cardiac and pulmonary involvement noted in this case are seen more often.

In conclusion, hypereosinophilia is potentially harmful regardless of the nature of the underlying condition and requires careful management. Currently, prognosis depends on two major aspects of disease - that is, heart involvement and the increased likelihood of developing hematological malignancies. Appropriate treatment is dependent in part on the ability to make an accurate etiologic diagnosis and to judge the clinical urgency for introducing an eosinophil-lowering agent. Advances in understanding the pathogenesis of HES variants have resulted in improved outcome for these uncommon causes of persistent hypereosinophilia; however, it can be dismal as in our patient who succumbed to the illness even after extensive work-up for etiological diagnosis and management with all available modality.

  Acknowledgements Top

Conflicts of interest

There are no conflicts of interest.

  References Top

Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore) 1975; 54 :1-27.  Back to cited text no. 1
Klion AD. How I treat hypereosinophilic syndromes. Blood 2009; 114 :3736-3741.  Back to cited text no. 2
Gotlib J. World Health Organization-defined eosinophilic disorders: 2014 update on diagnosis, risk stratification, and management. Am J Hematol 2014; 89 :325-337.  Back to cited text no. 3
Roufosse FE, Goldman M, Cogan E. Hypereosinophilic syndromes. Orphanet J Rare Dis 2007; 2 :37.  Back to cited text no. 4
Bosslet GT, Knox KS, Noor A. Severe idiopathic hypereosinophilic syndrome. Respiratory Medicine CME 2008; 1 :100-102.  Back to cited text no. 5
Liao YH, Su YW, Tsay W, Chiu HC. Association of cutaneous necrotizing eosinophilic vasculitis and deep vein thrombosis in hypereosinophilic syndrome. Arch Dermatol 2005; 141 :1051-1053.  Back to cited text no. 6
Terrier B, Piette AM, Kerob D, Cordoliani F, Tancrède E, Hamidou L, et al. Superficial venous thrombophlebitis as the initial manifestation of hypereosinophilic syndrome: study of the first 3 cases. Arch Dermatol 2006; 142 :1606-1610.  Back to cited text no. 7
Sakuta R, Tomita Y, Ohashi M, Nagai T, Murakami N. Idiopathic hypereosinophilic syndrome complicated by central sinovenous thrombosis. Brain Dev 2007; 2:182-184.  Back to cited text no. 8


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