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  • Omission of etoposide in the treatment of haemophagocytic lymphohistiocytosis secondary to primary central nervous system lymphoma with satisfactory response

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Rheumatology
Omission of etoposide in the treatment of haemophagocytic lymphohistiocytosis secondary to primary central nervous system lymphoma with satisfactory response
  1. Rachel Laches1,
  2. Robert J Hall1,
  3. Joanna Chaffin2 and
  4. Paulette Hahn3
  1. 1Internal Medicine, University of Florida, Gainesville, Florida, USA
  2. 2Department of Pathology, University of Florida College of Medicine, Gainesville, Florida, USA
  3. 3Department of Medicine, Division of Rheumatology, University of Florida, Gainesville, Florida, USA
  1. Correspondence to Rachel Laches; rachel.laches{at}gmail.com

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory condition that can be either familial or acquired and, if untreated, frequently results in multiorgan failure and death. Treatment of HLH typically requires a combination of glucocorticoids and cytotoxic chemotherapy. We describe the case of a woman who presented with signs and symptoms concerning for HLH who was later found to have a primary central nervous system (CNS) diffuse large B-cell lymphoma. Her HLH symptoms were successfully treated with high doses of dexamethasone, and her primary CNS lymphoma was treated with high-dose methotrexate and rituximab. This is a rare case of HLH secondary to primary CNS lymphoma where HLH was controlled with steroids alone and did not require the use of an etoposide-based regimen or cyclophosphamide, doxorubicin, vincristine and prednisone.

  • Rheumatology
  • Cancer intervention
  • Malignant disease and immunosuppression
  • Medical management
  • CNS cancer

https://doi.org/10.1136/bcr-2023-258559

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    Footnotes

    • Contributors The following authors were responsible for drafting the text, sourcing and editing clinical images, investigating results, drawing original diagrams and algorithms and critically revising important intellectual content: RL and RJH were the primary authors of the manuscript. PH supervised the development of the manuscript and oversaw patient care. JC was the pathologist who read the patient’s bone marrow biopsy and provided the images as well as the descriptions. The following authors gave final approval of the manuscript: RL, RJH, PH and JC.

    • 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.