Hunter syndrome with persistent thrombocytopenia. Issue 4 (20th April 2019)

• Record Type: Journal Article
• Authors: Panigrahi, Inusha; Dhanorkar, Manoj; Didel, Siyaram; Koganti, Raja Ashok
• Is Part Of: BMJ case reports. Volume 12:Issue 4(2019)
• Publication Date: 2019-04-20
• Subjects: chronic EBV infection -- MPS II -- platelets -- bleeding -- anaemia; Medicine -- Case studies -- Periodicals; 610.5
• DOI: 10.1136/bcr-2018-226518
• Languages: English
• ISSNs: 1757-790X

Abstract

Abstract : A case of Hunter syndrome, 6½-year-old boy presented with persistent thrombocytopenia and bleeding diathesis. However, cytopenia is not a usual presentation in patients with mucopolysaccharidosis II. After ruling out other causes of severe thrombocytopenia, a clinical possibility of chronic Epstein-Barr virus (EBV) infection was considered. He was treated with intravenous immunoglobulin for refractory thrombocytopenia and intracranial bleed. This was followed by oral prednisolone. The EBV serology was found positive. Platelet counts gradually recovered and no recurrence of bleeds was observed. EBV infection usually causes haematological abnormalities, mainly atypical lymphocytosis, which is a feature of infectious mononucleosis, and uncomplicated cases often present with mild decreases in platelet counts. Severe thrombocytopenia is an extremely rare complication of acute or chronic EBV infection. In Asians, EBV infection should be considered in children presenting with thrombocytopenia, bleeding diathesis and anaemia along with organomegaly.