IDIOPATHIC HYPEREOSINOPHILIC SYNDROME (HES)
Dr Ira Shah
Medical Sciences Department, Pediatric Oncall, Mumbai, India
Address for Correspondence
Dr Ira Shah, 1/B Saguna, 271/B St. Francis Road, Vile Parle (W), Mumbai 400056.
Abstract
Idiopathic hypereosinophilic syndrome (HES) is characterised by overproduction of eosinophils due to an undetectable cause that results in organ damage. We report a one and a half years old boy with HES and hepatosplenomegaly.
Introduction
Idiopathic hypereosinophilic syndrome (HES) represents a group of leukoproliferative disorders characterized by overproduction of eosinophils due to an undetectable cause that results in organ damage. (1). HES has been reported in women between 20 to 50 years and is extremely rare in children. (2). We report a one and a half years old boy with HES and hepatosplenomegaly.
Case Report
A one and a half years old boy presented with fever, and abdominal distension since 15 days. On examination, he had pallor with hepatosplenomegaly. His investigations revealed anemia (hemoglobin = 7.4 gm%), leucocytosis with eosinophilia (WBC count = 45,000/cu mm, absolute eosinophil count = 20,250/cu mm) with thrombocytosis (platelet count = 9,22,000/cu mm. His peripheral smear revealed several multinucleated eosinophils with heavy granules. His urine and stool examination was normal. His X-ray chest was normal. There was no history of passing worms in stools, breathlessness, cough or allergic tendencies. A history of contact with stray dogs was subsequently elicited. He was thus suspected to have eosinophilia due to parasitic infestation or malignancy. His bone marrow examination showed no evidence of blast cells with hyper cellular marrow and presence of binucleated / trinucleated eosinophils with heavy granules. His bone marrow cytogenetics was also normal. A liver biopsy for toxocara was also negative. Toxocara IgG & IgM could not be done due to unavailability of facility. He was treated with Albendazole (15 mg/kg/d) for 5 days but his hemogram remained the same (hemoglobin = 8.2 gm%, WBC = 64,400/cu mm, absolute eosinophil count = 49,588/cu mm, Platelet count = 7,50,000/cu mm). He was then given a course of Diethyl Carbamazine for 21 days but the eosinophilia persisted. He was thus diagnosed as a case of Idiopathic eosinophilia and advised oral steroids.
Discussion
Chusid et al have defined 3 features required for diagnosis of HES: 1) The eosinophil count exceeds 1500 cells/cu mm and persists for at least 6 months, 2) No etiology for eosinophilia is present and 3) It leads to organ damage. (1) Organ damage occurs due to increased survival of eosinophils in inflamed tissue for a prolonged time leading to release of toxic cationic proteins from its granules such as major basic protein, eosinophil peroxidase, eosinophil-derived neurotoxin and eosinophilic cationic protein (3). A study by Fauci et al has found hematologic involvement in all, neurological in 64%, skin in 56%, splenomegaly in 45%, pulmonary in 40%, cardiovascular in 54%, hepatomegaly in 35% and ocular involvement in 18% of patients with HES (3). In our patient, bone marrow, liver and spleen were involved with no peripheral organ damage. Clinical features may also include cardiac symptoms in form of thrombosis, endomyocardial fibrosis; hematological manifestations in form of anemia, thrombosis, hypercoagulability; neurological manifestations in form of embolic episodes, encephalopathy, peripheral neuropathy; pulmonary manifestations in form of eosinophilic infiltrates in the lungs, dyspnea, pleural effusion, pulmonary fibrosis, rhinitis; dermatologic manifestations in form of pruritis and other features such as arthralgia, myalgia, diarrhea, fever, weight loss and night sweats (3).

Peripheral eosinophilia is associated with other disorders such as allergic diseases, parasitic infections, eosinophilia-myalgia syndrome, Churg-Strauss syndrome and malignancy which need to be excluded before a diagnosis of HES is made. Patients without organ involvement may have benign eosinophilia which may exist for years with no associated pathology. Eosinophilic leukemia needs to be excluded as both conditions tend to overlap. However, patients with eosinophilic leukemia have clonal abnormalities, >25% immature eosinophils in peripheral blood or more than 5% myeloblasts in bone marrow (1). Bone marrow cytogenetics in our patient was normal.

Medical treatment is needed for symptomatic disease. Patients with no organ dysfunction despite eosinophilia need no treatment except close follow up at 3-6 intervals. Symptomatic patients should be treated with prednisolone therapy (1 mg/kg/d) till clinical improvement occurs followed by alternate day therapy and gradual tapering. Those with non-response to steroids can be treated with hydroxyurea, vincristine, 6-mercaptopurine, busulphan, and chlorambucil.(3)
Funding
None
Conflict of Interest
None
References :
  1. 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
  2. Bhatnagar S et al. Idiopathic Hypereosinophilic Syndrome. Indian Pediatr. 1999;36:824-827.
  3. Fauci AS, Harley JB, Roberts WC, Ferrans VJ, Granlnick HR, Bjornson BH, et al. The idiopathic hypereosinophilic syndrome: Clinical, pathologic and therapeutic considerations. NIH conference. Ann Int Med 1982; 97: 78-92.
Last Updated : Thursday, September 01, 2005 Vol 2 Issue 9 Art #41
How to Cite URL :
Shah I. IDIOPATHIC HYPEREOSINOPHILIC SYNDROME (HES). Pediatric Oncall [serial online] 2005[cited 2005 September 1];2. Art #41. Available From : http://www.pediatriconcall.com/Journal/Article/FullText.aspx?artid=733&type=J&tid=&imgid=&reportid=180&tbltype=
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