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 |