IgM paraproteinaemia is described in a patient with diffuse plasmacytoma involving the pleura, lymph nodes, and kidneys. Sixteen cases of pure plasmacellular tumor (diffuse or solitary plasmacytoma and plasmacellular leukaemia) associated with IgM paraproteinaemia have only been reported. These cases and experimental data indicate that neoplastic plasma cells can synthetize IgM as well as other immunoglobulins and that this particular M component is not associated solely with Waldenström's macroglobulinaemia.
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Int J Mol Sci
January 2025
Immunology Division, Department of Internal Medicine and Hematology, Semmelweis University, 1088 Budapest, Hungary.
Schnitzler syndrome is a unique autoinflammatory disease, of which 747 cases have been described worldwide to date. The main features of the syndrome are a triad of recurrent urticaria, monoclonal IgM gammopathy, systemic inflammation associated with recurrent fever, joint and bone pain, and atypical bone remodeling (osteosclerosis). The abnormal activation of the NLRP3 inflammasome produces IL-1, which drives the disease pathology, but it also involves IL-6 and IL-18.
View Article and Find Full Text PDFBackground: Waldenström's macroglobulinemia (WM) is a very rare disease with an incidence 10times lower than that of multiple myeloma. The incidence of WM is also significantly lower than that of the other CD20+ low-grade lymphomas. The rarity of WM is the reason why registration studies of new drugs used for multiple myeloma or the more common CD20+low-grade lymphomas do not cover WM.
View Article and Find Full Text PDFCancer Med
December 2024
Niguarda Hospital, Department of Hematology and Oncology, Milano, Italy.
Background: Despite recurrent and activating mutations, including MYD88, CXCR4, ARID1A, KMT2D, and CD79B were identified, the genetic basis for Waldenström's Macroglobulinemia (WM) and the risk of progression of IgM MGUS to WM remain to be fully elucidated.
Methods: We investigated the mutation status of WM (n = 8), sWM (n = 7), and IgM MGUS (n = 5) patients, by performing high-throughput targeted AmpliSeq NGS on 117 target genes. Specifically, we analyzed the CD19+ cells from 15 WM/sWM patients and five IgM MGUS patients.
Int J Hematol Oncol Stem Cell Res
October 2024
Apollo CBCC Cancer Care, Ahmedabad, Gujarat, India.
Waldenström macroglobulinemia (WM) is a rare lymphoproliferative malignancy presenting with para-proteinemia. The symptoms are attributable to both lymphoproliferation and IgM flare. Gastrointestinal manifestations are not uncommon.
View Article and Find Full Text PDFCurr Hematol Malig Rep
December 2024
Bing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute450 Brookline Ave, Mayer 223, Boston, MA, 02215, USA.
Purpose Of Review: Peripheral neuropathy (PN) is more commonly seen in individuals with monoclonal gammopathies, especially in patients with an IgM monoclonal gammopathy or Waldenström macroglobulinemia.
Recent Findings: There are multiple potential ways that the paraprotein may result in peripheral neuropathy. The diagnosis and management of monoclonal gammopathy-associated PN are challenging and necessitate a concerted effort between the hematologist/oncologist and the neurologist.
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