title: Durable hematological response and improvement of nephrotic syndrome on thalidomide therapy in a patient with refractory light chain deposition disease.
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.
authors.affiliation: Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawaharacho Sakyo-ku, Kyoto, 606-8507, Japan.
authors.forename: Haruyuki
authors.initials: H
authors.lastname: Fujita
authors.affiliation: Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawaharacho Sakyo-ku, Kyoto, 606-8507, Japan. hishiza@kuhp.kyoto-u.ac.jp.
authors.forename: Masakatsu
authors.initials: M
authors.lastname: Hishizawa
authors.affiliation: Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawaharacho Sakyo-ku, Kyoto, 606-8507, Japan.
authors.forename: Soichiro
authors.initials: S
authors.lastname: Sakamoto
authors.affiliation: Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawaharacho Sakyo-ku, Kyoto, 606-8507, Japan.
authors.forename: Tadakazu
authors.initials: T
authors.lastname: Kondo
authors.affiliation: Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawaharacho Sakyo-ku, Kyoto, 606-8507, Japan.
authors.forename: Norimistu
authors.initials: N
authors.lastname: Kadowaki
authors.affiliation: Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawaharacho Sakyo-ku, Kyoto, 606-8507, Japan.
authors.forename: Takayuki
authors.initials: T
authors.lastname: Ishikawa
authors.affiliation: Department of Laboratory Medicine, Nagoya City Midori General Hospital, Nagoya, Japan.
authors.forename: Junji
authors.initials: J
authors.lastname: Itoh
authors.affiliation: Department of Nephrology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Fukatsu
authors.affiliation: Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawaharacho Sakyo-ku, Kyoto, 606-8507, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Uchiyama
authors.affiliation: Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawaharacho Sakyo-ku, Kyoto, 606-8507, Japan.
authors.forename: Akifumi
authors.initials: A
authors.lastname: Takaori-Kondo
mesh_terms: D000328:Adult; D003907:Dexamethasone / Q000008:administration & dosage / Q000627:therapeutic use; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000097:blood* / Q000737:chemistry; D007155:Immunologic Factors / Q000008:administration & dosage* / Q000627:therapeutic use; D007668:Kidney / Q000378:metabolism* / Q000473:pathology; D009101:Multiple Myeloma / Q000188:drug therapy; D009404:Nephrotic Syndrome / Q000097:blood* / Q000188:drug therapy* / Q000473:pathology; D012074:Remission Induction / Q000379:methods*; D013792:Thalidomide / Q000008:administration & dosage* / Q000627:therapeutic use
chemical_list: D007147:Immunoglobulin Light Chains; D007155:Immunologic Factors; D013792:Thalidomide; D003907:Dexamethasone