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Clinical Brief
Hypereosinophilic Syndrome C. Venkatesh, E. Mahender, S. Janani, S. Malathi, M. Vijayakumar and B.R. Nammalwar Departments of Pediatrics, Pediatric Gastroenterology and Pediatric Nephrology, Kanchi Kamakoti CHILDS Trust Hospital, Chennai.
Abstract. Hypereosinophilic syndrome is a leukoproliferative disease characterised by sustained overproduction of eosinophils. The three diagnostic criteria for this disorder are (1) Eosinophilia of greater than 1500 cells/ml, persisting for longer than 6 months, (2) lack of another diagnosis to explain the eosinophilia and (3) signs and symptoms of organ involvement. We report a 15-year-old boy who was diagnosed as Hypereosinophilic syndrome based on these criteria. [Indian J Pediatr 2006; 73 (3) : 237-239] E-mail :
[email protected]. Key words : Eosinophilia; End organ damage
Hypereosinophilic syndrome, (HES) first described by Hardy and Anderson in 1968, is characterized by sustained overproduction of eosinophils. It is a rare disorder in children. The term Idiopathic Hypereosinophilic syndrome as defined by Chusid et al characterized by 3 criteria- eosinophil count greater than 1500 cells/mL persisting longer than 6 months and single or multiple organ system dysfunction attributable to cytotoxic injury by eosinophils, without an identifiable etiology to explain the eosinophilia. 1 The mortality in untreated patients 3years after diagnosis, can be as high as 75%.2 Cardiac involvement is the most common cause of increased morbidity and mortality. 3 Early identification and aggressive therapy is of paramount importance in decreasing the morbidity and mortality associated with this condition. CASE REPORT A 15-year-old previously healthy boy was brought for fever and malaise of two months duration, itchy papular skin rashes with scaling for one month, progressively increasing dyspnoea, with jaundice and decreased urine output of 1-week duration prior to admission. Earlier there was no puffiness of face, abdominal distension or pain, pedal edema, seizures, altered sensorium, hematemesis, malaena, arthralgia, arthritis or mucosal ulceration. His chest X-ray showed right basal pneumonitis, sputum culture had grown Staphylococcus
Correspondence and Reprint requests : Dr. Janani Sankar, Senior Consultant, Kanchi Kmakoti Childs Trust Hospital Chennai-600034. Fax : 044-28259633
Indian Journal of Pediatrics, Volume 73—March, 2006
aureus and was diagnosed to have staphylococcal pneumonia .He was treated with parenteral vancomycin, meropenem, netilmicin, oral cephalexin and doxycycline. Clinically he was well built, afebrile with marked pallor, icterus and extensive exfoliative skin rashes with hyper pigmentation over his entire body. He had bilateral cervical lymphadenopathy of size 1-1.5 cm. His cardiovascular system examination was normal with blood pressure of 100/60 mm of Hg. The respiratory rate was 42/min with fine crackles and wheeze. There was a soft hepato-splenomegaly. The neurological examination was normal. His investigations were as follows: Hemoglobin 6 gm%, PCV 18 %, total leukocyte count 27,600/mm3 with shift to left (stab forms 20%), eosinophils 14%, absolute eosinophil count (AEC) 3864/mm 3, platelet count 1.2 lakhs/mm3, ESR 150 mm in the 1st hour, reticulocyte count 4.7%, Direct Coomb‘s test was negative. Serum total bilirubin was 4.4mg/dL with direct fraction 3.8 mg/dl, SGOT 113 IU/L (5-45IU/L), SGPT 68 IU/L (5-45IU/L), serum alkaline phosphatase 1454 IU/L (130-525 IU/L), serum total protein 7.6 gm/dL, albumin 2.8gm/dL, increased globulin 4.8gm/dL, blood urea 75mg/dL, serum creatinine 3.1 mg/dL, and normal serum electrolytes. Urine showed 4+ proteinuria by dipstick and spot protein/creatinine ratio of more than 3.0 without cellular casts or erythrocyturia. C-reactive protein was 48mg/L, and cultures of blood and urine were sterile. Serology for hepatitis A, B and E, leptospirosi s and HIV were negative. Smear study for microfilaria and malarial parasite was negative and repeated stool microscopy for parasites did not reveal any ova, cysts or occult blood. Chest X-ray and echocardiogram were normal. Ultra sonogram of abdomen revealed hepatosplenomegaly and bilateral renomegaly (11.8&12cm) with normal echo texture and no free fluid in the abdomen. Bone marrow aspiration revealed plasmacytosis and 237
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C. Venkatesh et al increased eosinophilic precursors. Karyotyping was normal. Serum protein electrophoresis revealed elevated gamma globulin of 3.5gm/dl and immunoglobulin profile showed elevated S.IgE>2000 IU/L (
