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.
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.
Sakakima
Masanori
M
First Department of Medicine, Hamamatsu University School of Medicine, Japan.
Fujigaki
Yoshihide
Y
Tsuji
Takayuki
T
Fukasawa
Hirotaka
H
Miyaji
Takehiko
T
Naito
Kensuke
K
Yamamoto
Tatsuo
T
Yonemura
Katsuhiko
K
Ohnishi
Kazunori
K
Hishida
Akira
A
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
D018941:Antigens, CD19; D000970:Antineoplastic Agents; D019002:CD56 Antigen; D007147:Immunoglobulin Light Chains