Light chain deposition disease involving kidney and liver in a patient with IgD myeloma

BMC Nephrology

Akira Shimuzu¹  Daisuke Miura²  Kentaro Narita²  Kosei Matsue²  Toshiki Terao²  Masami Takeuchi²  Takafumi Tsushima²  Tomo Suzuki³ 

[1] Department of Analytic Human Pathology, Nippon Medical School;Division of Hematology/Oncology, Department of Internal Medicine, Kameda Medical Center;Division of Nephrology, Department of Medicine, Kameda Medical Center;

关键词: IgD myeloma;    Light chain deposition disease;    Liver involvement;    Daratumumab;    CyBorD;   

DOI  :  10.1186/s12882-021-02246-9

来源: DOAJ

【 摘 要 】

Abstract Background IgD multiple myeloma (MM) is a rare subtype of MM and light chain deposition disease (LCDD) outside the kidney is also a rare and has scarcely been reported. We report herein the details of the first reported case of LCDD involving the kidney and liver co-occurring with IgD myeloma. Case presentation A 66-year-old female with IgD MM presented with rapidly progressive acute renal failure, ascites and pleural effusion. Immunofluorescent study of revealed the characteristic linear deposition of Igκ light chain along the glomerular and tubular basement membrane in kidney. Electron microscopy showed the powdery electron-dense deposits along the tubular and glomerular basement membrane consistent with the diagnosis of LCDD. Laser microdissection followed by mass spectrometry identified only Igκ light chain with more than 95% probability confirm the diagnosis of κ-LCDD but not heavy/light chain deposition disease. Liver biopsy with immunofluorescence study revealed the linear deposition of Igκ chain along the perisinusoidal space indicating the hepatic involvement of κ-LCDD. The patient was successfully treated with combination therapy with bortezomib, cyclophosphamide, dexamethasone, and daratumumab. Conclusions This report emphasizes that prompt biopsy of affected organs and initiation of clone directed therapy led to the correct diagnosis and favorable outcome in patient with LCDD who has extrarenal involvement.

【 授权许可】

Unknown