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Hemophagocytic Lymphohistiocytosis Associated with Cytomegalovirus Infection in an Immunocompetent Infant: A Diagnostic and Therapeutic Challenge!

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Indian Journal of Hematology and Blood Transfusion Aims and scope Submit manuscript

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal syndrome that results from inappropriate activation of the immune system. Many viral agents are known to trigger HLH but cytomegalovirus (CMV) associated HLH is rarely described. We report a case of CMV related HLH in a 3½ month old immunocompetent male infant who presented with fever, respiratory distress and hepatosplenomegaly. He had fulminant sepsis like course in the hospital as he continued to have hectic fever spikes, progressive pneumonia, increasing hepatosplenomegaly and multiple episodes of generalized convulsions. Investigations revealed bicytopenia, biochemical hepatitis, hyperferritinemia and hypofibrinogenemia. CMV IgM serology was reactive in both infant and mother. Diagnosis of CMV-HLH was made as per HLH 2004 diagnostic protocol. Infant was successfully treated with intravenous ganciclovir along with dexamethasone and etoposide.

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Authors and Affiliations

  1. Department of Paediatrics, K. J. Somaiya Medical College and Hospital, Mumbai, India

    Sujata Kanhere, Manish Bhagat, Purvi Kadakia, Anuradha Joshi, Varsha Phadke & Kushagra Chaudhari

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  1. Sujata Kanhere
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  2. Manish Bhagat
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  3. Purvi Kadakia
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  4. Anuradha Joshi
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  5. Varsha Phadke
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  6. Kushagra Chaudhari
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Corresponding author

Correspondence to Sujata Kanhere.

Additional information

Presentation at a meeting: As a poster in the National conference of Pediatric Hematology and Oncology [PHOCON] 2013 held on 19, 20 October 2013 at New Delhi.

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Kanhere, S., Bhagat, M., Kadakia, P. et al. Hemophagocytic Lymphohistiocytosis Associated with Cytomegalovirus Infection in an Immunocompetent Infant: A Diagnostic and Therapeutic Challenge!. Indian J Hematol Blood Transfus 30 (Suppl 1), 299–302 (2014). https://doi.org/10.1007/s12288-014-0366-4

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  • DOI: https://doi.org/10.1007/s12288-014-0366-4

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