A rare case of autoimmune hemolytic anemia with protein S deficiency with splenic infarct.

Splenic infarction is a rare entity in pediatric population and is associated with predisposing conditions, i.e., hematological and oncological or infections. Among hematological conditions, only few cases associated with autoimmune hemolytic anemia (AIHA) have been reported so far. We encountered a 12-year-old female who presented with left upper quadrant pain and was diagnosed as AIHA with splenic infarction. Further workup revealed decreased protein S levels -63% (normal: 74%-146%); no laboratory parameters were suggestive of any clinical condition, leading to decreased protein S levels. Few cases of protein C deficiency with splenic infraction have been reported in patients with leukemia, hereditary spherocytosis, and Epstein-Barr virus infection, but to the best of our knowledge, no one has reported AIHA with splenic infarction with protein S deficiency so far.