title: Prolonged remission of refractory lymphomatoid granulomatosis after autologous hemopoietic stem cell transplantation with post-transplantation maintenance interferon.
abstract: Lymphomatoid granulomatosis (LYG) is a rare Epstein Barr virus (EBV)-associated lymphoproliferative disease. Even with combination chemotherapy, mortality is high. There is no standard therapy for relapsed or refractory disease. There is only one report in the literature of a complete remission with high-dose chemotherapy and autologous stem cell transplantation. This study presents the case of a patient with progressive LYG, who was successfully treated with autologous stem cell transplantation after conditioning with high-dose chemotherapy and total body irradiation. After transplantation, maintenance therapy with interferon alpha 2a was administered for 3.75 years. The patient remains well and in remission 8 years post-transplantation. This is the first report of a durable (>1 year) complete remission after high-dose chemotherapy and autologous stem cell transplantation in LYG. The role of high-dose chemotherapy and autologous stem cell transplantation in relapsed or refractory cases merits further evaluation. The exact place of interferon in treatment of LYG remains to be clarified but is promising.
authors.affiliation: Department of Haematology and Transfusion Services, Royal North Shore Hospital, Pacific Highway, St Leonards, NSW, Australia. markanna@tpg.com.au
authors.forename: Anna
authors.initials: A
authors.lastname: Johnston
authors.forename: Luke
authors.initials: L
authors.lastname: Coyle
authors.forename: David
authors.initials: D
authors.lastname: Nevell
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003131:Combined Modality Therapy; D018450:Disease Progression; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000008:administration & dosage* / Q000627:therapeutic use; D008175:Lung Neoplasms / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy*; D008230:Lymphomatoid Granulomatosis / Q000150:complications* / Q000175:diagnosis / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D011994:Recombinant Proteins; D012074:Remission Induction / Q000379:methods; D014182:Transplantation, Autologous; D016896:Treatment Outcome; D014916:Whole-Body Irradiation
chemical_list: D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins