1. Proteinuria e albuminuria nelle discrasie plasmacellulari.
- Author
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Basset, Marco, Milani, Paolo, Bosoni, Tiziana, Nuvolone, Mario, Albertini, Riccardo, Merlini, Giampaolo, and Palladini, Giovanni
- Subjects
PLASMA cell diseases ,IMMUNOGLOBULIN light chains ,FANCONI syndrome ,BONE marrow cells ,KIDNEY failure - Abstract
Plasma cell dyscrasias (PCDs) are characterized by a bone marrow plasma cell clone producing a monoclonal component (MC). This heterogeneous group comprehends malignant diseases as multiple myeloma (MM), conditions requiring only observation, like monoclonal gammopathy of undetermined significance (MGUS), and rarer entities where the MC causes organ dysfunction in absence of malignant PCD [(i.e. monoclonal gammopathy of clinical significance (MGCS)]. The evaluation of urine proteins has a key role in the management of PCDs, particularly quantification of proteinuria and albuminuria in the diagnostic workflow and clinical management of MGCS with renal involvement. In immunoglobulin light chain (AL) amyloidosis, proteinuria (cut-off: 0.5 g/24h) and urine albumin/creatinine ratio (UACR; cut-off: 300 mg/g) are used for diagnosis of renal involvement. These biomarkers are also included in staging systems that stratify the severity of renal involvement (proteinuria cut-off: 5 g/24h; UACR cut-off: 3 600 mg/g) and in renal response criteria (decrease of proteinuria/UACR >30% from baseline). In light chain deposition disease (LCDD), relatively low grade proteinuria (cut-off: 2 g/24h) results in higher risk of end-stage renal failure and its reduction after treatment (>50% from baseline) associates with better outcome. Finally, the presence of a MC and tubular proteinuria raises suspicion of light chain proximal tubulopathy (LCPT) with acquired Fanconi syndrome, especially in presence of other abnormalities at the urine examination (e.g. glycosuria, phosphaturia, uricosuria). The clinical laboratory, with the determination of biomarkers in urine provides crucial data in PCD management: for the diagnostic workflow, assessment of severity of the disease and evaluation of treatment effectiveness. [ABSTRACT FROM AUTHOR]
- Published
- 2023
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