2 ‐Chloro‐deoxyadenosine therapy for giant lymph node hyperplasia

Giant lymph node hyperplasia (GLNH) or Castleman disease is a heterogenous group of atypical lymphoproliferative disorders. Two main histologic variants, the hyaline vascular variant and the plasma cell variant, have been recognized. Although localized GLNH can often be managed successfully with surgery, optimal therapy for multifocal disease has yet to be identified. We report two cases of GLNH treated with 2-chloro-deoxyadenosine (2-CDA), a synthetic purine analogue. 2-CDA was utilized based on its relative lymphocytic toxicity and the putative pathophysiologic process in GLNH being either hamartomatous overgrowth (hyaline-vascular variant) or immune dysfunction and lymphoproliferation (plasma cell variant). One patient with unresectable localized hyaline-vascular GLNH has had a 9-month continuous complete remission following two courses of 2-CDA therapy followed by radiation therapy. The second patient with disseminated plasma cell type had a partial response to two cycles of 2-CDA therapy; however, further cycles were not given due to development of possible early neurotoxicity. Although the optimal management of non-resectable GLNH is yet to be determined, 2-CDA appears to be a viable therapeutic option for patients with this disease process.