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.

PubMed

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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