title: High dose chemotherapy and stem cell support in a patient of light- and heavy-chain deposition disease with abnormal marrow cell surface antigens and no monoclonal protein.
abstract: A 53-year-old man with nephrotic syndrome and severe renal failure was diagnosed with light- and heavy-chain deposition disease (LHCDD) by renal biopsy. The patient had no monoclonal protein and mild marrow plasmacytosis (6%), but marrow plasma cells expressed CD19(-)CD56+ and predominant monoclonal kappa-chain, indicating plasma cell dyscrasia. Conventional chemotherapy was ineffective and did not improve renal failure. High dose chemotherapy/peripheral blood stem cell transplantation (HDC/PBSCT) was introduced even after hemodialysis to eliminate aberrant clone and normalization of bone marrow cell surface markers. Immunophenotypic analysis of marrow cells facilitates clinical decision making regarding the use of HDC/PBSCT for LHCDD patients without monoclonal protein.
authors.affiliation: First Department of Medicine, Hamamatsu University School of Medicine, Japan.
authors.forename: Masanori
authors.initials: M
authors.lastname: Sakakima
authors.forename: Yoshihide
authors.initials: Y
authors.lastname: Fujigaki
authors.forename: Takayuki
authors.initials: T
authors.lastname: Tsuji
authors.forename: Hirotaka
authors.initials: H
authors.lastname: Fukasawa
authors.forename: Takehiko
authors.initials: T
authors.lastname: Miyaji
authors.forename: Kensuke
authors.initials: K
authors.lastname: Naito
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Yamamoto
authors.forename: Katsuhiko
authors.initials: K
authors.lastname: Yonemura
authors.forename: Kazunori
authors.initials: K
authors.lastname: Ohnishi
authors.forename: Akira
authors.initials: A
authors.lastname: Hishida
mesh_terms: D018941:Antigens, CD19 / Q000378:metabolism; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001854:Bone Marrow Cells / Q000276:immunology; D019002:CD56 Antigen / Q000378:metabolism; D003131:Combined Modality Therapy; D006362:Heavy Chain Disease / Q000188:drug therapy / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007147:Immunoglobulin Light Chains; D008297:Male; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000188:drug therapy / Q000628:therapy; D010265:Paraproteinemias / Q000188:drug therapy / Q000276:immunology / Q000628:therapy*; D036102:Peripheral Blood Stem Cell Transplantation
chemical_list: D018941:Antigens, CD19; D000970:Antineoplastic Agents; D019002:CD56 Antigen; D007147:Immunoglobulin Light Chains