“…Acquired Coombs negative hemolytic anemia (CNHA) is rare in the adolescent age group. The differential diagnosis of CNHA (nonimmune hemolysis) associated with splenomegaly, in pediatric patients, includes both inherited and acquired causes such as hemoglobinopathies, RBC enzyme or membrane defects, infections, toxins, HLH, immunodeficiencies, microangiopathies, and uncommonly neoplasms [ 13 , 14 ]. Splenectomy is rarely performed in these patients but may be indicated in severe refractory immune mediated hemolysis, recurrent splenic sequestration, or hypersplenism [ 15 ].…”