Cold AIHA and the best treatment strategies

Cold - reac tive auto im mune hemo lytic ane mia (AIHA) is rare among the hemo lytic ane mias. It results when 1 of a vari-ety of pro cesses causes the gen er a tion of immu no glob u lin M (IgM) autoantibodies against endog e nous eryth ro cytes, resulting in complement activation and predominantly intravascular hemolysis. Cold AIHA is typically a primary lympho-proliferative dis or der with mar row B - cell clones pro duc ing path o genic IgM. More rarely, sec ond ary cold AIHA (cAIHA) can develop from malig nancy, infec tion, or other auto im mune dis or ders. However, in chil dren cAIHA is typ i cally post infec tion, mild, and self - lim ited. Symptoms include a sequelae of ane mia, fatigue, and acrocyanosis. The sever ity of dis-ease is var i able and highly depen dent on the ther mal bind ing range of the auto an ti body. In adults, treat ment 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 tol er a bil ity. Recent insights into the cause of cold agglu ti nin dis ease as a clonal dis or der driven by com ple ment form the basis of newer ther a peu tic options. While rituximab - based reg i mens are still the main stay of ther apy, options have now expanded to include com ple ment - directed treat ments and other B-cell-directed or plasma-cell-directed therapies.

Automated summary

Cold reactive autoimmune hemolytic anemia is a rare condition caused by the production of autoantibodies against endogenous red blood cells. Treatment mainly focuses on reducing antibody production to manage the disease.