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

• Published in: Internal Medicine Vol. 44; no. 9; pp. 970 - 974
• Main Authors: SAKAKIMA, Masanori, FUJIGAKI, Yoshihide, TSUJI, Takayuki, FUKASAWA, Hirotaka, MIYAJI, Takehiko, NAITO, Kensuke, YAMAMOTO, Tatsuo, YONEMURA, Katsuhiko, OHNISHI, Kazunori, HISHIDA, Akira
• Format: Journal Article
• Language: English
• Published: Tokyo The Japanese Society of Internal Medicine 01-09-2005; Japanese Society of Internal Medicine
• Subjects: Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Antigens, CD19 - metabolism; Antineoplastic Agents - therapeutic use; Biological and medical sciences; Bone Marrow Cells - immunology; CD56 Antigen - metabolism; Combined Modality Therapy; Emergency and intensive care: renal failure. Dialysis management; Heavy Chain Disease - drug therapy; Heavy Chain Disease - immunology; Heavy Chain Disease - therapy; hemodialysis; Humans; Immunoglobulin Light Chains; Intensive care medicine; light- and heavy-chain deposition disease; Male; marrow cell surface antigens; Medical sciences; Middle Aged; monoclonal protein; Nephrotic Syndrome - drug therapy; Nephrotic Syndrome - therapy; Paraproteinemias - drug therapy; Paraproteinemias - immunology; Paraproteinemias - therapy; Peripheral Blood Stem Cell Transplantation; renal failure; Hemodialysis; Stem cell; Hematopoietic cell; Homograft; Light peptide chain; peripheral blood stem cell transplantation; Posology; Cell surface; Blood; light- and heavy-chain deposition disease; Extrarenal dialysis; Immunoglobulinopathy; Bone marrow; Graft; High dose; Deposition; Monoclonal immunoglobulin; Kidney disease; Human; monoclonal protein; Immunopathology; Urinary system disease; marrow cell surface antigens; Heavy chain disease; Protein; Antigen; Case study; Chemotherapy; Treatment; Renal failure
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.44.970

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.