Autoimmune Hemolytic Anemia in Adolescent Females

Kim, Taylor Olmsted; Bussel, James B.

doi:10.1007/978-3-030-48446-0_22

Cited by 1 publication

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“…EpsteinBarr virus, influ enza, cyto meg a lo vi rus, and var i cella are also impli cated. 38 Infectious test ing is recommended for all chil dren, and auto im mune screen ing is suggested (eg, anti nu clear antibodies titer) in ado les cents and teens. An exten sive workup to dis tin guish CAD from CAS such as that described in Clinical Case 1 is not nec es sary; cAIHA caused by lowgrade clonal Bcell lymphop roliferation is not reported in chil dren.…”

Section: Other Prom Is Ing/exper I Men Tal Ther a Piesmentioning

confidence: 99%

Cold AIHA and the best treatment strategies

Despotovic

Kim

2022

Hematology

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Cold-reactive autoimmune hemolytic anemia (AIHA) is rare among the hemolytic anemias. It results when 1 of a variety of processes causes the generation of immunoglobulin M (IgM) autoantibodies against endogenous erythrocytes, resulting in complement activation and predominantly intravascular hemolysis. Cold AIHA is typically a primary lymphoproliferative disorder with marrow B-cell clones producing pathogenic IgM. More rarely, secondary cold AIHA (cAIHA) can develop from malignancy, infection, or other autoimmune disorders. However, in children cAIHA is typically post infection, mild, and self-limited. Symptoms include a sequelae of anemia, fatigue, and acrocyanosis. The severity of disease is variable and highly dependent on the thermal binding range of the autoantibody. In adults, treatment has most commonly focused on reducing antibody production with rituximab-based regimens. The addition of cytotoxic agents to rituximab improves response rates, but at the expense of tolerability. Recent insights into the cause of cold agglutinin disease as a clonal disorder driven by complement form the basis of newer therapeutic options. While rituximab-based regimens are still the mainstay of therapy, options have now expanded to include complement-directed treatments and other B-cell-directed or plasma-cell-directed therapies.

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Section: Other Prom Is Ing/exper I Men Tal Ther a Piesmentioning

confidence: 99%