Autoimmune Hemolytic Anemia, Inborn Errors of Immunity and Genetics: An Evolving Arena

Excerpt

Autoimmune hemolytic anemia (AIHA) in children is a rare and often perplexing disease. The journal's current issue contains a single-center ambispective analysis of childhood AIHA from a center in Puducherry, India [1]. The authors have done an impressive and detailed assessment while describing a cohort of 46 children with AIHA over 8 y, including 2 y of prospective enrolment. The demonstrated epidemiology of childhood AIHA stands out in the current study, with a predominance of mixed-AIHA (m-AIHA) in the cohort. The data of large studies from the West has warm antibody AIHA (w-AIHA) as the commonest subtype, accounting for 60–70% of cases, followed by cold agglutinin syndrome and paroxysmal cold hemoglobinuria (PCH). The m-AIHA is reported to be the rarest subtype, accounting for <5% of all AIHA [2, 3]. Multiple single-center analyses from India have shown a variable distribution of the AIHA subtypes [4‐6]. In the current study, the majority (59%) had m-AIHA, while w-AIHA, the next most common subtype, was observed in merely one-fourth of the cohort. Another interesting finding was the absence of PCH; none of the cases had a demonstrable Donath-Landsteiner antibody. Detecting Donath-Landsteiner antibodies involves a laborious investigation and is not readily available; hence, PCH in children often goes undetected. …