Complications of Evans' syndrome in an infant with hereditary spherocytosis: a case report.
Hereditary spherocytosis (HS) is a genetic disorder of the red blood cell membrane clinically characterized by anemia, jaundice and splenomegaly. Evans' syndrome is a clinical syndrome characterized by autoimmune hemolytic anemia (AIHA) accompanied by immune thrombocytopenic purpura (ITP). It results from a malfunction of the immune system that produces multiple autoantibodies targeting at least red blood cells and platelets. HS and Evans' syndrome have different mechanisms of pathophysiology one another. We reported the quite rare case of an infant who had these diseases concurrently. Possible explanations of the unexpected complication are discussed.
Yoshida
Hideki
H
Department of Pediatrics, Matsushita Memorial Hospital, Moriguchi, Japan. hide0519@koto.kpu-m.ac.jp
Ishida
Hiroyuki
H
Yoshihara
Takao
T
Oyamada
Takashi
T
Kuwana
Masataka
M
Imamura
Toshihiko
T
Morimoto
Akira
A
D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D007223:Infant; D008297:Male; D010976:Platelet Count; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D013103:Spherocytosis, Hereditary / Q000097:blood / Q000150:complications* / Q000175:diagnosis*; D013577:Syndrome