PMID- 33485303 OWN - NLM STAT- MEDLINE DCOM- 20211203 LR - 20211214 IS - 1471-2369 (Electronic) IS - 1471-2369 (Linking) VI - 22 IP - 1 DP - 2021 Jan 23 TI - Light chain deposition disease involving kidney and liver in a patient with IgD myeloma. PG - 40 LID - 10.1186/s12882-021-02246-9 [doi] LID - 40 AB - 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 Igkappa 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 Igkappa light chain with more than 95% probability confirm the diagnosis of kappa-LCDD but not heavy/light chain deposition disease. Liver biopsy with immunofluorescence study revealed the linear deposition of Igkappa chain along the perisinusoidal space indicating the hepatic involvement of kappa-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. FAU - Tsushima, Takafumi AU - Tsushima T AD - Division of Hematology/Oncology, Department of Internal Medicine, Kameda Medical Center, 929 Higashi-chou, Kamogawa-shi, Chiba, 296-8601, Japan. FAU - Suzuki, Tomo AU - Suzuki T AD - Division of Nephrology, Department of Medicine, Kameda Medical Center, Kamogawa-shi, Chiba, 296-8601, Japan. FAU - Terao, Toshiki AU - Terao T AD - Division of Hematology/Oncology, Department of Internal Medicine, Kameda Medical Center, 929 Higashi-chou, Kamogawa-shi, Chiba, 296-8601, Japan. FAU - Miura, Daisuke AU - Miura D AD - Division of Hematology/Oncology, Department of Internal Medicine, Kameda Medical Center, 929 Higashi-chou, Kamogawa-shi, Chiba, 296-8601, Japan. FAU - Narita, Kentaro AU - Narita K AD - Division of Hematology/Oncology, Department of Internal Medicine, Kameda Medical Center, 929 Higashi-chou, Kamogawa-shi, Chiba, 296-8601, Japan. FAU - Takeuchi, Masami AU - Takeuchi M AD - Division of Hematology/Oncology, Department of Internal Medicine, Kameda Medical Center, 929 Higashi-chou, Kamogawa-shi, Chiba, 296-8601, Japan. FAU - Shimuzu, Akira AU - Shimuzu A AD - Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan. FAU - Matsue, Kosei AU - Matsue K AD - Division of Hematology/Oncology, Department of Internal Medicine, Kameda Medical Center, 929 Higashi-chou, Kamogawa-shi, Chiba, 296-8601, Japan. koseimatsue@gmail.com. LA - eng PT - Case Reports PT - Journal Article DEP - 20210123 PL - England TA - BMC Nephrol JT - BMC nephrology JID - 100967793 RN - 0 (Immunoglobulin D) SB - IM MH - Aged MH - Female MH - Humans MH - *Immunoglobulin D MH - Kidney Diseases/*immunology MH - Liver Diseases/*immunology MH - Multiple Myeloma/*complications/immunology PMC - PMC7824927 OTO - NOTNLM OT - CyBorD OT - Daratumumab OT - IgD myeloma OT - Light chain deposition disease OT - Liver involvement COIS- The authors declare that all authors have no competing interests. EDAT- 2021/01/25 06:00 MHDA- 2021/12/15 06:00 PMCR- 2021/01/23 CRDT- 2021/01/24 20:25 PHST- 2020/10/12 00:00 [received] PHST- 2021/01/01 00:00 [accepted] PHST- 2021/01/24 20:25 [entrez] PHST- 2021/01/25 06:00 [pubmed] PHST- 2021/12/15 06:00 [medline] PHST- 2021/01/23 00:00 [pmc-release] AID - 10.1186/s12882-021-02246-9 [pii] AID - 2246 [pii] AID - 10.1186/s12882-021-02246-9 [doi] PST - epublish SO - BMC Nephrol. 2021 Jan 23;22(1):40. doi: 10.1186/s12882-021-02246-9.