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Published on: May 6, 2018
Nephrotic syndrome caused by protein thrombi in glomerulocapillary lumen in Waldenström's macroglobulinaemia
1The Third Department of Internal Medicine, Tokyo Medical University Hospital, Tokyo, Japan. yharada@tokyo-med.ac.jp
Abstract:
Waldenström's macroglobulinaemia (WM) is described as a disorder of plasmacytoid lymphocytes. The renal complications of WM are less common and severe than those of multiple myeloma. We present a case of WM complicated by nephrotic syndrome. A biopsy specimen of the kidney revealed the intraglomerular thrombi of immunoglobulin M paraprotein. Corticosteroid pulse therapy and plasmapheresis were effective in improving proteinuria and reducing protein thrombi. The nephrotic syndrome caused by protein thrombi in WM may be reversible, at least in its early stage.
Insights
Waldenström's macroglobulinaemia can cause kidney problems like nephrotic syndrome due to immunoglobulin M paraprotein thrombi. Treatment with corticosteroids and plasmapheresis showed promise in reversing these kidney complications.
Area of Science:
- Nephrology
- Hematology
- Oncology
Background:
- Waldenström's macroglobulinaemia (WM) is a rare lymphoproliferative disorder affecting plasmacytoid lymphocytes.
- Renal complications in WM are infrequent but can be severe, unlike those in multiple myeloma.
- Nephrotic syndrome is an uncommon but significant renal manifestation of WM.
Observation:
- A case study presented a patient with Waldenström's macroglobulinaemia experiencing nephrotic syndrome.
- Kidney biopsy revealed intraglomerular thrombi composed of immunoglobulin M (IgM) paraprotein.
- These IgM paraprotein thrombi were identified as the cause of nephrotic syndrome.
Findings:
- Corticosteroid pulse therapy and plasmapheresis were administered to the patient.
- These therapeutic interventions led to significant improvement in proteinuria.
- A reduction in the size and presence of protein thrombi was observed post-treatment.
Implications:
- The findings suggest that nephrotic syndrome secondary to protein thrombi in WM may be reversible.
- Early diagnosis and intervention are crucial for managing renal complications in WM.
- This case highlights the potential efficacy of combined corticosteroid and plasmapheresis therapy in WM-related nephropathy.
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