Refractory Castleman's Disease

Abstract

Angiofollicular lymph node hyperplasia is an uncommon low-grade lymphoproliferative disorder first described by Castleman. It is typically asymptomatic with diagnosis incidentally made in young males on chest radiology. Biopsy, in 90% of cases, shows the hyaline vascular variant. In contrast the plasma cell sub-type is accompanied by fever, sweating, weight loss, anaemia, lymphadenopathy, splenomegaly and hypergammaglobulinaemia. Treatment, when necessary because of symptoms, has variable outcome in response to corticosteroids, combinations of cytotoxic drugs, or radiotherapy. Failure to promptly achieve disease control has ominous significance and most patients die. High-dose chemoradiotherapy with bone marrow salvage, using haematopoietic stem and progenitor cells derived from bone marrow or peripheral blood, has precedent in this situation.

We report a second successful outcome to this procedure in a 37 year old man with profound constitutional complaints of weight loss, drenching sweats, relentless high fevers and massive organomegaly, resistant to all previous therapy. After conditioning with total body irradiation, cyclophosphamide and high-dose melphalan followed by total nodal irradiation, he underwent peripheral blood stem cell allograft from an HLA-compatible sister. Despite his course being complicated by pulmonary tuberculosis he achieved immediate complete remission, and restaging at one year confirms this to be durable. This further anecdotal case report supports this option in refractory aggressive variants of this disease.

Keywords:

• Castleman's disease
• bone marrow transplantation
• allogeneic
• complete remission