Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
CASE REPORTS
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
Masanori SAKAKIMAYoshihide FUJIGAKITakayuki TSUJIHirotaka FUKASAWATakehiko MIYAJIKensuke NAITOTatsuo YAMAMOTOKatsuhiko YONEMURAKazunori OHNISHIAkira HISHIDA
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JOURNAL OPEN ACCESS

2005 Volume 44 Issue 9 Pages 970-974

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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.

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© 2005 by The Japanese Society of Internal Medicine
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