A case of Fanconi syndrome accompanied by crystal depositions in tubular cells in a patient with multiple myeloma
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±èµµÈñ(Kim Do-Hee) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Internal Medicine
ÀӾƿµ(Lim A-Young) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Internal Medicine
°ûÇýºó(Gwag Hye-Bin) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Internal Medicine
ÀÌÁöÇö(Lee Ji-Hyeon) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Internal Medicine
Á¤±â¼±(Jung Ki-Sun) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Internal Medicine
(Lee Keol) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Internal Medicine
Çã¿ì¼º(Huh Woo-Seong) - Sungkyunkwan University School of Medicine Samsung Medical Center Division of Nephrology
±è´ëÁß(Kim Dae-Joong) - Sungkyunkwan University School of Medicine Samsung Medical Center Division of Nephrology
±èÀ±±¸(Kim Yoon-Goo) - Sungkyunkwan University School of Medicine Samsung Medical Center Division of Nephrology
¿ÀÇÏ¿µ(Oh Ha-Young) - Sungkyunkwan University School of Medicine Samsung Medical Center Division of Nephrology
±è±âÇö(Kim Ki-Hyun) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Internal Medicine Division of Hematology
±Ç±â¿µ(Kwon Gee-Young) - Sungkyunkwan University School of Medicine Department of Pathology
ÀÌÁ¤Àº(Lee Jung-Eun) - Sungkyunkwan University School of Medicine Samsung Medical Center Division of Nephrology
Abstract
Fanconi syndrome (FS) is a rare condition that is characterized by defects in the proximal tubular function. A 48-year-old woman was admitted for evaluation of proteinuria. The patient showed normal anion gap acidosis, normoglycemic glycosuria, hypophosphatemia, and hypouricemia. Thus, her condition was compatible with FS. The M peak was found behind the beta globulin region in urine protein electrophoresis. Upon bone marrow examination, we found that 24% of cells were CD138© plasma cells with kappa restriction. From a kidney biopsy, we found crystalline inclusions within proximal tubular epithelial cells. Thereafter, she was diagnosed with FS accompanied by multiple myeloma. The patient received
chemotherapy and autologous stem cell transplantation, and obtained very good partial hematologic response. However, proximal tubular dysfunction was persistent until 1 year after autologous stem cell transplantation. In short, we report a
case of FS accompanied by multiple myeloma, demonstrating crystalline inclusion in proximal tubular cells on kidney biopsy.
Å°¿öµå
Fanconi syndrome, immunoglobulin kappa-chains, multiple myeloma, proteinuria
KMID :
0359920140330020112
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