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BMJ Case Reports 2018; doi:10.1136/bcr-2018-224424
  • Rare disease
  • CASE REPORT

Cholestatic jaundice: a unique presentation leading to the diagnosis of HLH with Hodgkin lymphoma, HIV and EBV

  1. Prerna Mewawalla2
  1. 1Internal Medicine, Allegheny Health Network, Pittsburgh, Pennsylvania, USA
  2. 2Hematology-Oncology, Allegheny Health Network, Pittsburgh, PA, USA
  1. Correspondence to Dr Hira Shaikh, hira.shaikh{at}ahn.org
  • Accepted 25 April 2018
  • Published 12 May 2018

Summary

Haemophagocytic lymphohistiocytosis (HLH) is a syndrome of dysregulated immune activity with macrophage activation that can manifest as pancytopenia, coagulopathy and other laboratory abnormalities, usually progressing to multiorgan failure and death. This report documents the rarely reported association between HLH and Hodgkin’s lymphoma (HL) with simultaneous HIV and Epstein-Barr virus (EBV) and complete resolution with chemotherapy. The patient initially presented with cholestatic jaundice. He was then found to have HL associated with HLH with coexistent HIV and EBV viraemia. A-Brentuximab-VD regimen for the lymphoma was initiated, resulting in rapid resolution of HLH and ultimately remission of HL. HLH is a syndrome known to have high mortality; thus, early diagnosis and prompt treatment are crucial. Usual presentation includes non-specific symptoms and can easily be overlooked. This case highlights the importance of diagnosing the condition in unusual settings and attempting treatment by targeting the cause of HLH, HL in our case.

Footnotes

  • Contributors HS and SS wrote the manuscript. While AK and PM extensively revised the case report.

  • 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.

  • Competing interests None declared.

  • Patient consent Obtained.

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

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