HIV-associated multicentric castelman disease, a report of 5 cases

Introduction

Multicentric Castleman’s disease (MCD) is a rare, non-clonal lymphoproliferative disorder characterized by constitutional symptoms, anaemia and generalised lymphadenopathy.

Aim

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

Method

We reviewed the files of 930 HIV-1-infected patients from our AIDS Reference Centre. Data was collected from the operating software for the patients’ medical records of our institution.

Results

We report a series of five cases of MCD among our HIV/AIDS patients’ cohort. Three were of African origin. They were diagnosed after 2003, after a mean duration of 85 months of HIV-seropositivity. All presented with characteristic clinical features and laboratory findings (table 1), and all but one patient were started on HAART only a few months before or upon MCD diagnosis. Four patients were treated with chemotherapy, and one with HAART only. One patient who was given Adriamycin/Bleomycin/Vinblastin is in continuous remission after 6 years of follow-up. Two are alive, with good symptom control, regardless of the treatment they received. One recently relapsed, and one unfortunately died before completing the intended 6-courses chemotherapy regimen.

Conclusion

MCD is a rare, but rising issue among HIV-infected patients. The clinical and paraclinical features of our series of five 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.