Importance of immunosuppression in haemophagocytic lymphohistiocytosis caused by miliary tuberculosis

Tine Nielsen, Andreja Dimitrijevic, Mia Dahl Sørensen, Isik Somuncu Johansen, Dennis Lund Hansen*

*Corresponding author for this work

Research output: Contribution to journalJournal articleResearchpeer-review

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is a syndrome with an abnormal activation of the immune system and is associated with a high mortality even with treatment. We present a case of a woman in her mid-50s who developed HLH triggered by miliary tuberculosis (TB) while receiving a tumour necrosis factor alpha inhibitor.The patient was admitted with a high fever and respiratory pain. Her condition deteriorated despite empirical treatment. Diagnosis of HLH was established based on clinical presentation, H-score and HLH-04 criteria. Concurrently, miliary TB was identified as the trigger. She was treated with anti-tuberculous therapy and HLH-directed treatment with dexamethasone, etoposide and anakinra. Initial improvement was observed, leading to the withholding of HLH-orientated treatment. However, several relapses occurred, necessitating prolonged HLH treatment.A literature review corroborated the importance of combined anti-tuberculous and immunosuppressive therapy for managing HLH. This case underscores the necessity of timely and comprehensive management of HLH-oriented treatment.

Original languageEnglish
Article numbere261936
JournalBMJ Case Reports
Volume17
Issue number9
ISSN1757-790X
DOIs
Publication statusPublished - 25. Sept 2024

Keywords

  • Haematology (incl blood transfusion)
  • Immunology
  • TB and other respiratory infections

Fingerprint

Dive into the research topics of 'Importance of immunosuppression in haemophagocytic lymphohistiocytosis caused by miliary tuberculosis'. Together they form a unique fingerprint.

Cite this