Presentation and Outcome of Castleman’s Disease in Immunocompetent Hosts

Abstract

Castleman’s disease (CD) is a heterogeneous lymphoproliferative disorder of unknown aetiology. Mostly, this disorder is seen in immunocompromised hosts. It is known to be associated with systemic disorders like HIV, HHV-8, lymphoma, and Kaposi sarcoma. As of today, the clinical behaviour and outcome of CD in immunocompetent host remains suboptimally studied. We analyzed consecutively treated cases of CD presented to our centre in last 12 years. Case record files were studied for patient’s characteristics, clinical presentation, baseline laboratory and pathologic parameters, therapy and outcome. This study describes presentation and treatment outcome of CD in immunocompetent patients. Total 16 patients of CD were treated during the study period. The median age of patients at the time of presentation was 40.5 years (range 13–72 years). An equal number of patients (8 each) had unicentric and multicentric CD. Sixty-three percent patients had hyaline vascular subtype while 37 % patients had plasma cell or mixed variant. Majority of the patients had good performance status (ECOG PS 0, 1 in 10 (62.5 %) patients; PS2-4 in 6 (37.5 %) patients). The median duration of symptoms was 6 months (range 2–36 months). None of the patients in our study had associated HIV infection. Six patients presented with fever, out of which four had plasma cell variant of CD and three of them had multicentric involvement. In comparison to unicentric CD, patients with multicentric CD had lower albumin levels (4.15 vs. 3.38 g/dl, p = 0.006), haemoglobin levels (11.3 vs. 9.8, p = 0.06), and lower complete remission rates (62.5 % vs. none). Patients were treated according to the stage and clinical status with surgery, chemotherapy or combination of both modalities. Surgery was the predominant treatment for unicentric CD while multicentric CD was treated with various chemotherapy regimens. Eight patients were treated with chemotherapy (CHOP-based regimen-5, melphalan-prednisolone thalidomide-1, chlorambucil-prednisolone-1, and only corticosteroids-1). In the entire study group, ORR was 72 % (CR 36 %, PR 36 %), one patient died of progressive disease during chemotherapy. Two patients lost to follow-up before assessment of treatment response. Five-year overall survival was 100 and 87 % for unicentric and multicentric CD respectively. Castleman’s disease is a rare lymphoproliferative disorder amongst Indian patients. It more commonly presents as hyaline vascular variant and affects middle-aged individuals. The most remarkable fact in our analysis was a lack of HIV positivity in any of the patient that is in contrast to the majority of the published literature. With multimodality therapy, high response rates and long-term survival were noted in the entire study group.