دورية أكاديمية

Crystalglobulin-associated nephropathy presenting as MGRS in a case of monoclonal B-cell lymphocytosis: a case report

التفاصيل البيبلوغرافية
العنوان: Crystalglobulin-associated nephropathy presenting as MGRS in a case of monoclonal B-cell lymphocytosis: a case report
المؤلفون: Gupta, Rajib K., Arend, Lois J., BK, Anupama, Narsipur, Sriram, Bhargava, Ramya
المصدر: BMC Nephrology ; volume 21, issue 1 ; ISSN 1471-2369
بيانات النشر: Springer Science and Business Media LLC
سنة النشر: 2020
مصطلحات موضوعية: Nephrology
الوصف: Background Crystalglobulin-associated nephropathy (CAN), a rare subtype of monoclonal gammopathy, usually associated with multiple myeloma and occasionally monoclonal gammopathy of uncertain significance (MGUS), is characterized by occluding monoclonal pseudothrombi within renal glomerular capillaries and/or interstitial arterioles. Ultrastructurally, these pseudothrombi are unique for having a crystalline substructure. We describe a case of an adult patient with monoclonal B-cell lymphocytosis (MBL) and acute renal failure whose kidney biopsy revealed a rare diagnosis of CAN. Case presentation A 63-year old male presented with a 2-month history of edema, arthralgia and malaise. He had acute kidney injury with hematoproteinuria on urine analysis. Serum and urine protein electrophoresis were both negative. A renal biopsy however revealed features of CAN. Organomegaly, bone pain and lymphadenopathy were absent. A repeat serum electrophoresis was positive for IgA kappa and a free light chain assay showed elevated free kappa light chains. Flow cytometry done subsequently revealed a diagnosis of MBL, chronic lymphocytic leukemia (CLL) type. Conclusion CAN in association with MBL/CLL has not been previously described in literature, and our case highlights yet another instance of monoclonal gammopathy of renal significance (MGRS) where a small B-cell clone resulted in extensive renal pathology without systemic manifestations.
نوع الوثيقة: article in journal/newspaper
اللغة: English
DOI: 10.1186/s12882-020-01818-5
DOI: 10.1186/s12882-020-01818-5.pdf
DOI: 10.1186/s12882-020-01818-5/fulltext.html
الإتاحة: https://doi.org/10.1186/s12882-020-01818-5Test
حقوق: https://creativecommons.org/licenses/by/4.0Test ; https://creativecommons.org/licenses/by/4.0Test
رقم الانضمام: edsbas.94F3B719
قاعدة البيانات: BASE