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Case report
Chemotherapy-sparing treatment of haemophagocytic lymphohistiocytosis with intravenous immunoglobulins and corticosteroids
  1. Evan C Chen1,2,
  2. Jonathan A Stefely3,
  3. Bimalangshu R Dey2 and
  4. Walter H Dzik1,4
  1. 1Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA
  2. 2Division of Hematology/Oncology, Massachusetts General Hospital, Boston, Massachusetts, USA
  3. 3Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA
  4. 4Hematology and Blood Transfusion Service, Massachusetts General Hospital, Boston, MA, USA
  1. Correspondence to Dr Evan C Chen; ecchen{at}


Haemophagocytic lymphohistiocytosis (HLH) can be a rapidly fatal disease. Current treatment in adults is extrapolated from the HLH-2004 protocol that specifies a regimen of etoposide, dexamethasone and cyclosporine. However, HLH presents as a spectrum of disease severity. A therapeutic challenge arises for milder cases where the harms of potent chemotherapy such as etoposide may outweigh its benefit. We present a case of an adult with HLH who developed significant pancytopenia but was otherwise not critically ill and who responded to treatment with a chemotherapy-sparing approach consisting of intravenous immunoglobulins and corticosteroids alone. The case illustrates that tailored therapy may allow effective treatment of the disorder while minimising therapy-related toxicities.

  • haematology (incl blood transfusion)
  • infectious diseases
  • rheumatology
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  • Contributors ECC performed data collection, analysis and manuscript preparation. JAS performed data collection and analysis. BRD performed data collection and manuscript editing. WHD performed data analysis and manuscript preparation.

  • 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.

  • Patient consent for publication Obtained.

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

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