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

BMJ Case Rep. 2018 May 12:2018:bcr2018224424. doi: 10.1136/bcr-2018-224424.

Abstract

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.

Keywords: HIV / AIDS; infectious diseases; liver disease; malignant and benign haematology.

Publication types

  • Case Reports

MeSH terms

  • Abdominal Pain
  • Adult
  • Checklist*
  • Drug Therapy, Combination*
  • Epstein-Barr Virus Infections / diagnosis*
  • Epstein-Barr Virus Infections / drug therapy
  • Epstein-Barr Virus Infections / immunology
  • Fever
  • Guidelines as Topic
  • HIV Infections / diagnosis*
  • HIV Infections / drug therapy
  • HIV Infections / immunology
  • Hodgkin Disease / diagnosis*
  • Hodgkin Disease / drug therapy
  • Hodgkin Disease / immunology
  • Humans
  • Jaundice, Obstructive
  • Lymphohistiocytosis, Hemophagocytic / diagnosis*
  • Lymphohistiocytosis, Hemophagocytic / drug therapy
  • Lymphohistiocytosis, Hemophagocytic / immunology
  • Male
  • Time-to-Treatment
  • Treatment Outcome