Durable hematological response and improvement of nephrotic syndrome on thalidomide therapy in a patient with refractory light chain deposition disease |
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Authors: | Haruyuki Fujita Masakatsu Hishizawa Soichiro Sakamoto Tadakazu Kondo Norimistu Kadowaki Takayuki Ishikawa Junji Itoh Atsushi Fukatsu Takashi Uchiyama Akifumi Takaori-Kondo |
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Institution: | (1) Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawaharacho Sakyo-ku, Kyoto 606-8507, Japan;(2) Department of Laboratory Medicine, Nagoya City Midori General Hospital, Nagoya, Japan;(3) Department of Nephrology, Graduate School of Medicine, Kyoto University, Kyoto, Japan; |
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Abstract: | Light chain deposition disease (LCDD) is a rare disease for which an optimal treatment is not yet available. Here, we report
the clinical course of a 32-year-old woman with LCDD who was successfully treated with thalidomide. She presented with nephrotic
syndrome. Based on the renal biopsy findings and the presence of monoclonal immunoglobulin light chains in her serum and urine,
LCDD was diagnosed. Prednisolone and cytotoxic chemotherapy used for multiple myeloma proved ineffective. We initiated administration
of thalidomide (100 mg daily) and dexamethasone (20 mg for 4 days per month). After 8 months of treatment, she achieved complete
hematological remission, defined as the disappearance of monoclonal protein and a normalized free light chain ratio, which
led to improvement of her renal insufficiency. She has shown sustained hematological and organ response for 31 months with
thalidomide therapy. Thus, thalidomide therapy seems to be a promising approach to the treatment of LCDD. |
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