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 k...

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Veröffentlicht in:	Internal Medicine 2005, Vol.44(9), pp.970-974
Hauptverfasser:	SAKAKIMA, Masanori, FUJIGAKI, Yoshihide, TSUJI, Takayuki, FUKASAWA, Hirotaka, MIYAJI, Takehiko, NAITO, Kensuke, YAMAMOTO, Tatsuo, YONEMURA, Katsuhiko, OHNISHI, Kazunori, HISHIDA, Akira
Format:	Artikel
Sprache:	eng
Schlagworte:	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
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Zusammenfassung:	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.
ISSN:	0918-2918 1349-7235
DOI:	10.2169/internalmedicine.44.970