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Haemophagocytic lymphocytic histiocytosis/macrophage activation syndrome with acute inflammatory gastroenteritis
  1. Sindhura Kolachana1,
  2. Albahi Malik2,
  3. Athmananda Nanjudappa3,
  4. Jeffrey Iding4,
  5. Deepty Bhansali5 and
  6. Christopher J Haas3,6
  1. 1Medicine, Georgetown University Medical Center, Washington, District of Columbia, USA
  2. 2Medicine, MedStar Union Memorial Hospital, Baltimore, Maryland, USA
  3. 3Medicine, MedStar Franklin Square Medical Center, Baltimore, Maryland, USA
  4. 4Pathology, MedStar Franklin Square Medical Center, Baltimore, Maryland, USA
  5. 5Pathology, MedStar Union Memorial Hospital, Baltimore, Maryland, USA
  6. 6Medicine, MedStar Harbor Hospital, Baltimore, Maryland, USA
  1. Correspondence to Ms Sindhura Kolachana; smk293{at}


Haemophagocytic lymphocytic histiocytosis (HLH) is a rare, life-threatening condition caused by abnormal activation of cytotoxic T lymphocytes, natural killer cells and macrophages resulting in hypercytokinaemia and immune-mediated injury of multiple organ systems. Secondary HLH occurs in the setting of a malignant, infectious or autoimmune stimulus. Macrophage activation syndrome (MAS) is the term used to describe HLH that develops secondary to rheumatological diseases such as lupus and juvenile idiopathic arthritis, among others. Commonly observed and documented symptoms include fever, organomegaly and lymphadenopathy. Given the potential for multiorgan failure in HLH/MAS, early identification, diagnosis and initiation of treatment is essential. We present a case of secondary HLH/MAS with acute inflammatory gastroenteritis in a middle-aged woman with a history of systemic lupus erythematosus.

  • Gastroenterology
  • Rheumatology

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  • Contributors All authors have read and approved the final manuscript. Manuscript writing was done by SK, AM, JI and DB. Manuscript editing was performed by AN and CH. Supervision by CH. Patient was under the care of CH.

  • 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.