Waldenström's Macroglobulinemia/Lymphoplasmacytic Lymphoma Developing Renal AA Amyloidosis: A Case Report and Literature Review.

in Internal medicine (Tokyo, Japan) by Yusuke Ishizuka, Yuji Oe, Sosuke Kinomura, Saori Kin, Yuji Noguchi, Koichi Kikuchi, Mai Yoshida, Rui Makino, Koji Okamoto, Tasuku Nagasawa, Takafumi Toyohara, Mariko Miyazaki, Hiroshi Sato, Yasushi Onishi, Hitoshi Warita, Tetsuhiro Tanaka

TLDR

  • A 66-year-old man with WM/LPL developed nephrotic syndrome and AA amyloidosis, which recurred despite chemotherapy and increased prednisolone treatment. He died of cryptococcal meningitis.
  • The case highlights the importance of considering AA amyloidosis as a complication in patients with WM/LPL, even in remission.

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 COVID-19 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.

Overview

  • The study presents a case of a 66-year-old man with Waldenström's macroglobulinemia/lymphoplasmacytic lymphoma (WM/LPL) who developed nephrotic syndrome and AA amyloidosis.
  • The patient underwent chemotherapy, which resulted in remission of hematologic and nephrotic syndromes.
  • However, during a follow-up, the patient became infected with COVID-19 and had massive proteinuria, despite being in remission from WM/LPL.

Comparative Analysis & Findings

  • AA amyloidosis occurred in the patient despite being in remission from WM/LPL.
  • The patient's proteinuria worsened despite increased prednisolone treatment.
  • The patient died of cryptococcal meningitis, highlighting the difficulty in managing fatal complications.

Implications and Future Directions

  • The study highlights the importance of considering AA amyloidosis as a complication in patients with WM/LPL, even in remission.
  • Further research is needed to understand the mechanisms underlying AA amyloidosis in these patients and to develop effective treatment strategies.
  • Increased awareness and monitoring for complications such as AA amyloidosis and cryptococcal meningitis are essential in patients with WM/LPL.
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