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Low-grade B cell lymphoma in the perirenal space of the left kidney associated with high titer cold agglutinin disease. | LitMetric

AI Article Synopsis

  • - Cold agglutinin disease (CAD) is a type of autoimmune hemolytic anemia marked by the production of abnormal antibodies due to specific B lymphocyte activity and can be categorized into primary CAD or secondary cold agglutinin syndromes (CAS).
  • - A case study is presented where a patient developed low-grade B cell lymphoma just 3 months after being diagnosed with CAD and had notably high cold agglutinin levels and elevated IgM.
  • - The patient underwent successful treatment with a bendamustine and rituximab (BR) regimen, leading to significant reductions in cold agglutinin titers and achieving complete remission for over 34 months.

Article Abstract

Cold agglutinin disease (CAD) is a subgroup of autoimmune hemolytic anemia caused by monoclonal cold agglutinins produced by clonally expanded B lymphocytes. In primary CAD, lymphoproliferative bone marrow disorder is noted, while as one of the secondary cold agglutinin syndromes (CAS), the initial manifestation of CAD is followed by development of lymphoma. Here, we report a case of low-grade B cell lymphoma developed 3 months after an initial CAD diagnosis. The patient had an extremely high serum cold agglutinin titer (1:16,384) and slightly elevated serum IgM (452 mg/dL; reference, 31-200) with positive monoclonal IgM-kappa chain. After diagnosis of lymphoma-associated CAS, he was managed successfully with six cycles of a BR (bendamustine and rituximab) regimen. Cold agglutinin titers fell rapidly to 1:2048 at 5 months and to 1:512 at 10 months after chemotherapy, and the patient has been in a complete remission for 34 months.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10349293PMC

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