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Secondary haemophagocytic lymphohistiocytosis in a patient with new-onset systemic lupus erythematosus: the challenges of timely diagnosis and successful treatment
  1. Daming Shao1,
  2. Oscar Pena1,
  3. Miroslav Sekulic2,
  4. Ronald Valdez Imbert1,
  5. Charan Thej Reddy Vegivinti1 and
  6. Belinda Jim1
  1. 1Medicine, Jacobi Medical Center, Bronx, New York, USA
  2. 2Department of Pathology and Cell Biology, Columbia University Medical Center, New York, New York, USA
  1. Correspondence to Dr Belinda Jim; belindajim286{at}


Haemophagocytic lymphohistiocytosis (HLH) is an immune-mediated disease driven by abnormal macrophage activation and regulatory cell dysfunction. HLH can be primary due to genetic mutations or secondary due to infection, malignancy or autoimmune conditions. We describe a woman in her early 30s who developed HLH while being treated for newly diagnosed systemic lupus erythematosus (SLE) complicated by lupus nephritis as well as concomitant cytomegalovirus (CMV) reactivation from a dormant infection. The trigger for this secondary form of HLH may have been either aggressive SLE and/or CMV reactivation. Despite prompt treatment with immunosuppressive therapies for SLE consisting of high-dose corticosteroids, mycophenolate mofetil, tacrolimus, etoposide for HLH and ganciclovir for CMV infection, the patient developed multiorgan failure and passed away. We demonstrate the difficulty in identifying a specific cause for secondary HLH when multiple conditions are present (SLE and CMV) and the fact that, despite aggressive treatment for both conditions, the mortality for HLH remains high.

  • Immunology
  • Acute renal failure
  • Systemic lupus erythematosus

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  • 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—DS, OP, Ronald Valdez, Charan Thej Reddy Vegivinti, MS and BJ. The following authors gave final approval of the manuscript—DS, OP, Ronald Valdez, Charan Thej Reddy Vegivinti, MS and BJ.

  • 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.