| Autor: |
Rajib K. Gupta, Lois J. Arend, Anupama BK, Sriram Narsipur, Ramya Bhargava |
| Jazyk: |
angličtina |
| Rok vydání: |
2020 |
| Předmět: |
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| Zdroj: |
BMC Nephrology, Vol 21, Iss 1, Pp 1-6 (2020) |
| Druh dokumentu: |
article |
| ISSN: |
1471-2369 |
| DOI: |
10.1186/s12882-020-01818-5 |
| Popis: |
Abstract 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. |
| Databáze: |
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| Externí odkaz: |
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