Article Text

Download PDFPDF
Successful management of haemophagocytic lymphohistiocytosis in an adolescent with newly diagnosed HIV/AIDS and histoplasmosis
  1. Yagiz Matthew Akiska1,
  2. Wei Li Adeline Koay2,3,
  3. Justin Unternaher2 and
  4. Natella Y Rakhmanina1,2,4
  1. 1 School of Medicine, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA
  2. 2 Division of Infectious Diseases, Children's National Hospital, Washington, District of Columbia, USA
  3. 3 Division of Pediatric Infectious Diseases, Medical University of South Carolina, Charleston, South Carolina, USA
  4. 4 Elizabeth Glaser Pediatric AIDS Foundation, Washington, DC, USA
  1. Correspondence to Mr Yagiz Matthew Akiska; yakiska{at}


Haemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening hyperinflammatory syndrome characterised by persistent fevers, cytopenia, hepatosplenomegaly and systemic inflammation. Secondary HLH can be triggered by various aetiologies including infections, malignancies and autoimmune conditions. We highlight the complexity of HLH diagnosis and management by describing a case of an adolescent Salvadoran immigrant with HLH, newly diagnosed HIV, Streptococcal bacteraemia and disseminated histoplasmosis. The patient presented with neurological and ocular findings along with persistent fevers and cytopenia. He was diagnosed with HLH and treated with anakinra in addition to receiving treatment for HIV, Streptococcal bacteraemia and histoplasmosis. The patient’s HLH resolved without corticosteroids or chemotherapy, which are considered the mainstays for HLH treatment. This case underscores the need for the evaluation and management of multiple infections and individualised management in patients presenting with HLH to achieve favourable outcomes.

  • HIV / AIDS
  • Haematology (incl blood transfusion)
  • Infectious diseases
  • Paediatrics
  • Dermatology

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.


  • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms and critical revision for important intellectual content: YMA, WLAK, JU, NYR. The following authors gave final approval of the manuscript: YMA, WLAK, JU, NYR.

  • 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.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

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