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

BACKGROUND

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).

METHODS

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.

RESULTS

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.

CONCLUSION

Lymphomas and HHV8-associated diseases are common triggers of HLH in PLWH and are linked to a high mortality rate.