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2022, Number 1

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Invest Medicoquir 2022; 14 (1)

Cold agglutinin syndrome in association with small lymphocytic malignancy low-grade non-Hodgkin's lymphoma. Report of a case

Figueredo PYE, Luna CCM

Language: Spanish
References: 22
Page: 1-14
PDF size: 178.95 Kb.


ABSTRACT

Cold agglutinin syndrome is a rare form of autoimmune hemolytic anemia, accounting for 20% of all autoimmune hemolytic anemia. In this entity, red blood cell lysis is mediated by complement activation. This hemolysis occurs at temperatures below 37 ° C. The presentation can be idiopathic or secondary and its treatment is related to the cause. Treatment consists of steroid-based regimens associated with cyclophosphamide or chlorambucil and immunotherapy such as rituximab. A 53-year-old female patient is presented, who attends the hematology service of the Center for Surgical Medical Research due to presenting marked decay. When carrying out complementary studies, a monoclonal gammopathy and a positive direct Coomb test were found, with high titers of IgM-type cold agglutinins. The definitive diagnosis was made post-mortem, which revealed a small lymphocytic non-Hodgkin lymphoma.


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