HIV-associated multicentric Castleman disease: a report of 4 cases and review of the literature.

BACKGROUND

Multicentric Castleman's disease (MCD) is a rare, non-clonal lymphoproliferative disorder characterized by constitutional symptoms, anaemia and generalised lymphadenopathy. Its incidence among the HIV-positive population seems to have increased during the past decades.

AIM

The present study intends to compare demographic features, clinical presentation, laboratory studies, imaging results as well as treatment regimens and outcome in our MCD patients to those of larger reported series.

METHOD

We reviewed the files of 920 HIV-1-infected patients from our AIDS Reference Centre. Data was collected from the operating software for the patients' medical records of our institution (Medical Explorer v3r3, Cliniques St Luc, 2008).

RESULTS

We report a series of four cases of MCD among our HIV/AIDS patients' cohort. Three were of African origin. They were diagnosed after 2003, after a mean duration of 54 months of HIV-seropositivity (ranging from 7 to 120 months) All presented with characteristic clinical features and laboratory findings, and were started on HAART a few months before or upon MCD diagnosis. Three patients were treated with chemotherapy (ABV), and one with HAART only. One patient who was given ABV is in continuous remission after 3 years of follow-up. The remaining three are alive, with good symptom control, regardless of the treatment they received.

CONCLUSION

MCD is a rare, but rising issue among HIV-infected patients. The clinical and paraclinical features of our series of four patients are in keeping with those of larger reported series. Currently, treatment is mainly chemotherapy-based, but a wide variety of protocols have been used, mainly because of the lack of available evidence. New approaches such as anti-CD 20 antibodies seem highly effective, and the role of HHV-8 needs to be further investigated, as it might be an important target for future treatment. In light of this review, we are looking forward to offer these opportunities to our patients, despite unhelpful regulations.