CRP3 HYPEREOSINOPHILIC SYNDROME WITH TRANSUDATIVE BILATERAL PLEURAL EFFUSION

Teh Jia Xin, Amanil Ula Hassan
Pediatric Department, Hospital Sultanah Bahiyah, Kedah, Malaysia

Introduction:

Hypereosinophilic syndrome (HES) is a rare disorder of disarrayed overproduction of eosinophils causing tissue infiltration and organ damage. 

Case report:

We report a 5-month-old boy, who presented with coryza symptoms preceded with 1 month history of skin lesion. Upon presentation, he was in respiratory distress with tachypnea, subcostal recession and reduced breath sound bilateral lungs with crepitations. Other physical examination showed hepatosplenomegaly with multiple hyperpigmented round lesions over upper limbs and chest. He was intubated after 2 days of admission for worsening respiratory distress. Chest X-ray and ultrasound thorax showed evidence of bilateral pleural effusion. Right pigtail was inserted and drained hemoserous fluid. Pleural fluid analysis demonstrated non exudative, non chylous fluid with count predominantly eosinophils. His full blood count showed moderate to severe hypereosinophilia (4740 cell/mm3). Skin biopsy over the lesion revealed eosinophiles infiltration. Extensive workout to look for cause of hypereosinophilia including parapneumonic infection, tuberculosis, auto-immune disorder and malignancy were negative except for slightly raised total immunoglobulin E. He received intravenous antibiotic treatment for a week. The case was referred to Paediatric Hematologist and was treated conservatively for transient hypereosinophilia. The child was ventilated for 2 weeks and prior to extubation he received a single dose of dexamethasone and subsequent absolute eosinophil count (AEC) reduced drastically. He was discharged well. During follow up, he continues showing good weight gain, no recurrent respiratory symptoms and no new skin lesion. His follow up AEC was within normal range.  

Discussion & Conclusion:

HES is a rare myeloproliferative disorder with hypereosinophilia (>1500cell/mm3) and evidence of at least 1 organ dysfunction. HES responded well with steroid and close follow up is essential as unusual evolution to malignancy is common in persistent hypereosinophilia.