Catleman's disease at Chris Hani baragwanath academic hospital-a retrospective study

Abstract

Castleman’s disease (CD) is a rare B-cell lymphoproliferative disorder that manifests clinically as unicentric or multicentric disease and pathologically as hyaline vascular, plasma cell or mixed variants. Multicentric Castleman’s Disease (MCD) is aetiologically linked to HIV and HHV-8 and has recently been classified into HHV-8 positive and HHV-8 negative or idiopathic MCD subtypes. In this study, there was a total of 41 patients diagnosed with CD during the period 01/01/1990 to 31/12/2015, in the Division of Clinical Haematology, Department of Medicine, CHBAH. The mean age at presentation was 40 years, with a range of 18-69 years. There were 24 males (24/41 – 59%) and 17 females (17/41 – 41%), with a male to female ratio of 1.4:1. Seventy eight percent of the patients were HIV sero-positive. Lymphadenopathy was the most common clinical finding, occurring in 97.5% (39/40 patients) of the patients at presentation. Other common findings at presentation included fatigue (26/39 – 66.7%), hepatomegaly (25/39 – 64.1%), and fever, night sweats, weight loss, and splenomegaly (all occurring in 24/39 – 61.5%) of the patients at presentation. The two most common comorbidities seen in association with CD were Tuberculosis and Kaposi’s sarcoma. Morphologically, the mixed variant of CD was most frequently encountered (52%), followed by the plasma cell variant (28%) and hyaline vascular variant (20%). HHV-8 positivity was seen in 92% of the biopsies in which the stain was performed. Therapy included both supportive care and specific modalities of treatment. In UCD, the mainstay of treatment was surgical resection, with a favourable outlook in all the patients. In MCD, combination chemotherapy was the mainstay of treatment, with CHOP as the backbone and the addition of etoposide and rituximab in a number of patients. Nineteen patients were lost to follow up (19/41 – 46.3%), and six patients (6/41 – 14.6%) are reported alive at the last follow up visit, while a total of 16 patients (16/41 – 39%) demised. Of the nineteen patients that were lost to follow up, twelve were not in remission (12/19 – 63.2%), and six had achieved complete remission (6/19 – 31.6%) at their last follow up visit. Of the six patients that were alive, five were reported to have achieved a complete remission at the last follow up visit (5/6 – 83.3%). Sixteen patients have been reported dead (16/41 – 39%), of which thirteen (13/16 – 81.2%) of them were reported to have died from some form of sepsis. The mean survival time for the patients in this study was 78.6 months.