Hemophagocytic lymphohistiocytosis in people living with HIV-a single centre experience.

in Infection by Pascal Migaud, Daniela Drauz, Alessia Dalla Pria, Kai Hosmann, Markus Müller, Leyli Ghaeni, Hartmut Stocker

TLDR

  • A retrospective cohort study of 22 HIV-infected patients with hemophagocytic lymphohistiocytosis (HLH) found common triggers to be lymphomas and HHV8-associated diseases, which were linked to a high mortality rate.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that clinically resembles sepsis thus obscuring the underlying condition and delaying its diagnosis and therapy. Among the most common triggers are lymphomas and infectious diseases. Lymphoma-associated HLH appears to be more common in People living with HIV (PLWH). Retrospective cohort study comprising all adult HIV-infected patients with HLH treated at St. Joseph Hospital Berlin-Tempelhof, Germany, defined by HLH 2004-criteria and the HScore, between April 2020 and November 2024. 22 patients were included with at least 5/8 positive HLH criteria and a median HScore of 222 points. Median age was 44 [29-66] years. The median CD4-count at HLH-diagnosis was 100/µL [14-936]. In 8 (36%) patients the HIV-viral load was undetectable. HLH led to the diagnosis of HIV in 6 (27%) patients. In 20/22 patients an LPD was the HLH trigger. Hodgkin's lymphoma, HHV8-positive multicentric Castleman disease and HHV8-positive primary effusion lymphoma accounted for 8 (36%), 5 (23%) and 3 (14%) cases respectively. Kaposi sarcoma inflammatory cytokine syndrome (KICS) HHV8-positive plasmablastic lymphoma, HHV8-positive diffuse large B-cell lymphoma, DLBCL and invasive Aspergillosis were each found in 1 (4%) patient. All patients with Hodgkin's lymphoma had bone marrow involvement. In 1 patient simultaneous malaria and multiple myeloma were diagnosed. 11/22 (50%) patients had HHV8-associated conditions. 5 (23%) patients died within 30 days of the HLH-diagnosis. Lymphomas and HHV8-associated diseases are common triggers of HLH in PLWH and are linked to a high mortality rate.

Overview

  • The study aimed to investigate the triggers of hemophagocytic lymphohistiocytosis (HLH) in HIV-infected patients.
  • The study used a retrospective cohort analysis of 22 adult HIV-infected patients with HLH treated at St. Joseph Hospital Berlin-Tempelhof, Germany between April 2020 and November 2024.
  • The study aimed to identify the common triggers of HLH in HIV-infected patients and assess the mortality rate associated with these triggers.

Comparative Analysis & Findings

  • The study found that lymphomas (including Hodgkin's lymphoma, HHV8-positive multicentric Castleman disease, and HHV8-positive primary effusion lymphoma) were the most common triggers of HLH, accounting for 36% of cases.
  • HHV8-associated conditions were present in 50% of patients, with HHV8-positive plasmablastic lymphoma, HHV8-positive diffuse large B-cell lymphoma, and DLBCL found in 4% of cases each.
  • The mortality rate was high, with 5 (23%) patients dying within 30 days of the HLH diagnosis, with lymphomas and HHV8-associated diseases being linked to a higher mortality rate.

Implications and Future Directions

  • The study highlights the importance of considering the possibility of underlying lymphoma or HHV8-associated disease in HIV-infected patients presenting with hyperinflammatory syndrome and delays in diagnosis and therapy.
  • Future studies should investigate the pathophysiological relationships between HLH, lymphoma, and HHV8-associated disease in HIV-infected patients.
  • The study's findings emphasize the need for close monitoring of HIV-infected patients for signs of HLH and early initiation of treatment to prevent mortality.
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