Castleman’s Disease: Main Characteristics in a Series of 37 Patients

OBJECTIVE: To analyse retrospectively a series of 37 patients with biopsy proven Castleman’s Disease (CD).
PATIENTS: Retrospective study of patients with biopsy proven CD with a statistical analysis of epidemiologic, clinical, biological, histological, therapeutic and prognostic data.
RESULTS: 25 (68%) males, age 47±15 yrs, 9 (24%) HIV+, 34 multicentric forms (92%).
B symptoms were present in 33 patients (89%): fever 65%, asthenia 81%, weight loss 62%. Lymph node involvement: axillary 76%, cervical 57%, abdominal and retroperitoneal 54%, mediastinal 46%, splenomegaly 48% and hepatomegaly 32%.
Associated diseases: Haemophagocytosis 9 patients (24%); B Non Hodgokin Lymphoma (B-NHL) 9 patients (24%); autoimmune disease 10 patients (27%): antiphospholipid syndrome (n=4), autoimmune hemolytic anaemia (3), systemic sclerosis (1), mixed connective tissue disease (1) and POEMS syndrome (1).
Biological characteristics: anaemia in 65% of patients, thrombocytopenia 40%, thrombocytosis 24%, hyperleucocytosis 27%. An inflammatory syndrome was detected in 86% of patients, hyperferritinemia 71%, hypergammaglobulinemia 68% and autoantibodies 57%.
Pathological characteristics: 18 plasma cell CD (pcCD) forms (49%), 14 mixed CD (mCD) forms (38%) and 5 hyaline-vascular CD (hvCD) forms (14%). Patients with hvCD were older (65±7 yrs) than those with pCD (42±13) and mCD (47±14) [P=0.01]. Multicentric CD were more frequently associated with pCD (100%) and mCD (93%) than with hvCD (60%) [P=0.015]. Patients with mCD were more frequently HIV+ (50%) than those with pCD (12.5%) and hvCD (0%) [P=0.016], and had higher CRP levels (141±98) than those with pCD (90±64) and with hvCD (14±20 mg/L) [P=0.006]. Patients with pCD compared to those with mCD and hvCD tended to have higher immunoglobulins levels (28±19 vs 17±7 g/L; P=0.06).
HIV-infected Patients: HIV+CD compared to HIV-CD, were more frequently HHV8+ (71% vs 14%; P=0.03), had more frequently a mCD (78% vs 25%; P=0.01), higher CRP levels (165±81 vs 79±77 mg/mL; P=0.016), higher ferritin levels (3311±3314 vs 1119±1542 µg/L; P=0.03), lower platelet count (143±115 vs 290±193 x 103/mm3; P=0.02) and more frequently received cytotoxic drugs (89% vs 36%; P=0.01).
Treatment: overall, 35 patients have been treated: steroids alone (n=12), steroids + cytotoxic drugs (15); cytotoxic drugs alone (5), surgery alone (6), Rituximab (6). The clinical response was assessable in 30 patients. After a mean follow-up of 4.8 yrs, 20 patients (67%) achieved a complete remission, 4 (13%) a partial response and 6 patients died (20%). The latter patients were older (57±11 vs 45±15 yrs; P=0.06) and more frequently had B-NHL (6/6 vs 2/30; P<10-5).
Conclusion: In this recent series, CD patients show a good prognosis except for those with associated B-NHL. Significant differences have been highlighted within histological forms, and between HIV-infected versus HIV-negative patients.

 D. Sène, None; Z. Amoura, None; A. Delluc, None; B. Wechsler, None; F. Charlotte, None; P. Bossi, None; S. Choquet, None; J.C. Piette, None; P. Cacoub, None.