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Waldenström's Macroglobulinemia/Lymphoplasmacytic Lymphoma Developing Renal AA Amyloidosis: A Case Report and
Yusuke Ishizuka1, Yuji Oe1, Sosuke Kinomura1
1Department of Nephrology, Tohoku University Graduate School of Medicine, Japan.
Abstract:
AA amyloidosis is a rare renal complication of Waldenström's macroglobulinemia/lymphoplasmacytic lymphoma (WM/LPL). A 66-year-old man with WM/LPL presented with nephrotic syndrome. A renal biopsy showed AA amyloidosis. Chemotherapy resulted in the remission of hematologic and nephrotic syndromes. Two years into follow-up, he became infected with coronavirus disease 2019 and had massive proteinuria, despite no relapse of WM/LPL. A second renal biopsy confirmed a diagnosis of AA amyloidosis. However, increased prednisolone did not improve proteinuria. The patient ultimately died of cryptococcal meningitis. This case highlights the diverse spectrum of renal involvement in monoclonal IgM-secreting diseases and difficulty in managing fatal complications.
Insights
AA amyloidosis, a rare kidney complication of Waldenström's macroglobulinemia, can recur even after treatment. This case shows challenges in managing recurrent amyloidosis and its fatal complications, including during COVID-19 infection.
Area of Science:
- Nephrology
- Hematology
- Oncology
Background:
- AA amyloidosis is a rare renal complication associated with Waldenström's macroglobulinemia/lymphoplasmacytic lymphoma (WM/LPL).
- Monoclonal IgM-secreting diseases can present with diverse renal manifestations.
- Management of AA amyloidosis in the context of other severe illnesses poses significant challenges.
Purpose of the Study:
- To report a case of recurrent AA amyloidosis in a patient with WM/LPL.
- To highlight the complexities in managing renal complications of WM/LPL.
- To discuss the impact of COVID-19 on a patient with pre-existing WM/LPL and AA amyloidosis.
Main Methods:
- Case report of a 66-year-old male patient with WM/LPL.
- Renal biopsy to diagnose AA amyloidosis.
- Chemotherapy for hematologic and nephrotic syndromes.
- Monitoring during COVID-19 infection and subsequent renal biopsy.
Main Results:
- Initial chemotherapy led to remission of hematologic and nephrotic syndromes.
- Recurrence of AA amyloidosis with massive proteinuria was observed during COVID-19 infection.
- Treatment with increased prednisolone was ineffective for proteinuria.
- The patient died from cryptococcal meningitis.
Conclusions:
- AA amyloidosis presents a diverse spectrum of renal involvement in monoclonal IgM-secreting diseases.
- Managing fatal complications, especially during superimposed infections like COVID-19, is difficult.
- This case underscores the need for vigilant monitoring and tailored management strategies for patients with WM/LPL and AA amyloidosis.
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