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Waldenström macroglobulinemia (WM) is a rare lymphoplasmacytic disorder characterized by an IgM paraprotein. The clinical presentation of WM varies and can include common manifestations such as anemia and hyperviscosity, in addition to less common features such as cryoglobulinemia, IgM-related neuropathy, and immunoglobulin light chain (AL) amyloidosis. Amyloidosis is a protein-folding disorder in which vital organ damage occurs due to the accumulation of misfolded protein aggregates. The most common type of amyloidosis in patients with an IgM paraprotein is AL amyloidosis, although other types of amyloidosis may occur. IgM-related amyloidosis has distinct clinical features when compared with other subtypes of AL amyloidosis. This review highlights the diagnostic criteria of IgM-related AL amyloidosis, as well as the clinical characteristics and treatment options for this disorder. [ABSTRACT FROM AUTHOR]

Keywords: Waldenstrom-s macroglobulinaemia; plasmablastic lymphoma; ibrutinib EN Waldenstrom-s macroglobulinaemia plasmablastic lymphoma ibrutinib 466 468 3 11/01/21 20211101 NES 211101 A 67-year-old female was diagnosed with Waldenström macroglobulinemia (WM) in October 2004. Plasmablastic lymphoma transformation in a patient with Waldenström macroglobulinemia treated with ibrutinib A CT-guided retroperitoneal lymph node biopsy showed lymphoid tissue diffusely effaced by sheets of large cells with ovoid to irregular nuclei, vesicular chromatin, prominent nucleoli and abundant eosinophilic cytoplasm. Abdominal computed tomography (CT) revealed bulky lymphadenopathy encasing the inferior vena cava. [Extracted from the article]