Updates in the Management of Warm Autoimmune Hemolytic Anemia. 2022

Jennifer C Yui, and Robert A Brodsky
Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, 1830 East Monument Street, Suite 7300, Baltimore, MD 21205, USA. Electronic address: jyui1@jhmi.edu.

Warm autoimmune hemolytic anemia (wAIHA) is an uncommon and heterogeneous disorder caused by autoantibodies to RBC antigens. Initial evaluation should involve the DAT, with wAIHA typically IgG positive with or without C3 positivity, and a search for underlying conditions associated with secondary wAIHA, which comprise 50% of cases. First-line therapy involves glucocorticoids, increasingly with rituximab, though a chronic relapsing course is typical. While splenectomy and a number of immunosuppressive therapies have been used in the setting of relapsed and refractory disease, the optimal choice and sequence of therapies is unknown, and clinical trials should be offered when available. Newer investigational targets include spleen tyrosine kinase inhibitors, monoclonal antibodies targeting CD38, Bruton's tyrosine kinase inhibitors, complement inhibitors, and antibodies against neonatal Fc receptors.

UI MeSH Term Description Entries
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000069283 Rituximab A murine-derived monoclonal antibody and ANTINEOPLASTIC AGENT that binds specifically to the CD20 ANTIGEN and is used in the treatment of LEUKEMIA; LYMPHOMA and RHEUMATOID ARTHRITIS. CD20 Antibody, Rituximab,GP2013,IDEC-C2B8,IDEC-C2B8 Antibody,Mabthera,Rituxan,IDEC C2B8,IDEC C2B8 Antibody,Rituximab CD20 Antibody
D000744 Anemia, Hemolytic, Autoimmune Acquired hemolytic anemia due to the presence of AUTOANTIBODIES which agglutinate or lyse the patient's own RED BLOOD CELLS. Anemia, Hemolytic, Idiopathic Acquired,Autoimmune Haemolytic Anaemia,Autoimmune Hemolytic Anemia,Cold Agglutinin Disease,Hemolytic Anemia, Autoimmune,Acquired Autoimmune Hemolytic Anemia,Anemia, Hemolytic, Cold Antibody,Cold Antibody Disease,Cold Antibody Hemolytic Anemia,Idiopathic Autoimmune Hemolytic Anemia,Agglutinin Disease, Cold,Anaemia, Autoimmune Haemolytic,Anemia, Autoimmune Hemolytic,Autoimmune Haemolytic Anaemias,Autoimmune Hemolytic Anemias,Cold Agglutinin Diseases,Cold Antibody Diseases,Haemolytic Anaemia, Autoimmune
D001323 Autoantibodies Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them. Autoantibody
D013156 Splenectomy Surgical procedure involving either partial or entire removal of the spleen. Splenectomies
D047428 Protein Kinase Inhibitors Agents that inhibit PROTEIN KINASES. Protein Kinase Inhibitor,Inhibitor, Protein Kinase,Inhibitors, Protein Kinase,Kinase Inhibitor, Protein,Kinase Inhibitors, Protein

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