Haemophagocytic lymphohistiocytosis in HIV-associated HHV-8-positive multicentric Castleman disease.

OBJECTIVE

The clinical and laboratory characteristics of HHV-8-associated multicentric Castleman disease (MCD) in people with HIV (PWH) overlap with those of haemophagocytic lymphohistiocytosis (HLH) disease and indeed the two diagnoses may co-exist. A risk-stratified treatment approach to MCD based on Rituximab immunotherapy for mild cases and chemo-immunotherapy for severe cases has been shown to yield excellent outcomes in PWH. In contrast, HLH disease, previously known as secondary HLH, has a dismal prognosis even when promptly treated according to guidelines.

DESIGN

A retrospective multicentre cohort study.

METHODS

Retrospective analysis of prospectively collected clinical and pathological data on patients with biopsy-proven HIV-associated MCD at the National Centre for HIV Malignancy at Chelsea and Westminster Hospital, London between 2008 and 2024 and at the Department of Infectious Diseases at St. Joseph Hospital Berlin-Tempelhof, Germany between 2020 and 2024.

RESULTS

In our UK-German cohort, including 113 PWH with MCD, we confirmed that HLH disease secondary to MCD is common (30%), and we demonstrated that HLH disease in this context does not adversely influence survival or risk of MCD relapse.

CONCLUSION

We suggest that a high HScore in MCD should not lead to a change in the treatment in this specific setting.