Hypereosinophilic Syndrome Masquerading as Complicated Pneumonia
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SCIENTIFIC LETTER
Hypereosinophilic Syndrome Masquerading as Complicated Pneumonia Sachin Singh 1 & Jagdish Prasad Goyal 1 & Prawin Kumar 1 Received: 9 December 2019 / Accepted: 10 January 2020 # Dr. K C Chaudhuri Foundation 2020
To the Editor: HypereosinophilIic syndrome (HES) is a rare multisystem disorder characterized by increased peripheral eosinophil count with end-organ damage [1]. It is uncommon in children. Its clinical presentation varies from asymptomatic to life-threatening conditions [2]. A 3-y-old boy was admitted for evaluation of fever, cough, and fast breathing for ten days. On examination, there was decreased air entry in the bilateral infrascapular and infra-axillary region. The radiograph showed bilateral infiltrate in the lower zone with pleural and pericardial effusion. A provisional diagnosis of pneumonia with complication was kept and intravenous antibiotic was started. Because of poor response to antibiotics after 72 h, he was further evaluated. In hemograms, hemoglobin – 7 g/dl, total leukocyte count – 45,300 cells/mm3 (polymorphs 05%, lymphocytes 33%, and eosinophils 60%) and platelet count of 8.7 lakh/mm3 were noted. In peripheral smear, there was marked eosinophilia. The bone marrow examination showed myeloid hyperplasia with increased eosinophils and its precursors. Because of multisystem involvement (hematological, pulmonary, and cardiac) and no cause attributable to hypereosinophilia, a final diagnosis of HES was made and he was started on oral prednisolone (1 mg/kg/d). He showed clinical improvement within a week, and prednisolone was gradually tapered and stopped after 12 wk. There was no recurrence of symptoms, and eosinophil count reached within the normal limit at 6-mo of follow-up. Classically, the diagnosis of HES is considered in the presence of following three criteria (1) absolute eosinophils count (AEC) more than 1500 cells/μl, for more than 6 mo (2) absence of other conditions to explain the
underlying eosinophilia and (3) presence of end-organ damage. However, the recent revision (2010) of HES suggests that AEC should be greater than 1500 cells/μl on two consecutive occasions, which persist for a minimum of 1 mo instead of 6 mo [3]. Clinically, HES is classified into five groups; myeloproliferative (or classical), lymphoproliferative, familial, undefined (or idiopathic) and associated [4]. The common clinical features in children are fever, cough, dyspnea, myalgia, and rash. The common organs involved are pulmonary, cardiac, neurological, skin, and gastrointestinal systems. Cardiac involvement is associated with significant mortality and morbidity [2, 5]. Diagnosis of HES is established by meticulous clinical history, physical examination to look for underlying organ damage, relevant investigations to rule out reactive HES, exclusion of malignancy by bone marrow examination, imaging and genetic testing (FIP1L1-PDGFRA) [6]. In symptomatic cases, prednisolone is the drug of choice. In steroid resistant cases, hydroxyurea, cyclosporine, chlorambucil or imatinib (interfer
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