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Hunter syndrome with persistent thrombocytopenia
  1. Inusha Panigrahi,
  2. Manoj Dhanorkar,
  3. Siyaram Didel and
  4. Raja Ashok Koganti
  1. Department of Pediatrics, APC, PGIMER, Chandigarh, India
  1. Correspondence to Prof. Inusha Panigrahi, inupan{at}


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.

  • chronic EBV infection
  • MPS II
  • platelets
  • bleeding
  • anaemia

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  • Contributors All authors—IP, SD, MD and RAK—were involved in patient management and drafting of the article. MD prepared the initial draft of the manuscript. MD and IP did the interpretation of the patient information. All authors approve the final version of the article. The corresponding author, IP, will act as a guarantor for the paper.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Parental/guardian consent obtained.