Autoimmune hemolytic anemia associated with anti‐Sc1

Abstract

A case of autoimmune hemolytic anemia (AIHA) in a young child is described. The hemolysis was resistant to steroid therapy but responded to splenectomy and intravenous immunoglobulin. The autoantibody was shown to be anti-Sc1 by both serologic and immunoblotting techniques. This seems to be the first report of an autoanti-Sc1 detected by immunoblotting and the first example of AIHA in a child caused by autoanti-Sc1.

The Clinical Pictures of Autoimmune Hemolytic Anemia

“…The remaining patients with warm antibody AIHA have IgG autoantibodies that react with Rh-null RBCs [ 29 , 30 , 31 , 32 ]. Autoantibody specificity for these so-called non-Rh-related blood group antigens has been identified, including Wr b [ 29 ], En a [ 34 ], LW [ 35 ], U [ 36 ], Ge [ 23 , 37 ], Sc1 [ 38 ], and K [ 39 ].…”

Review: the Scianna blood group system

“…McDowell et al 25 described two cases of autoimmune hemolytic anemia (AIHA) associated with auto anti-Sc1 due to transient suppression of Sc1. Similarly, Owen et al 26 described a case of severe, acute AIHA in infancy found in a West Indian girl that was associated with auto anti-Sc1. The hemolysis was resistant to steroid therapy, but was resolved following splenectomy and two courses of high-dose IVIG given perioperatively.…”

Friday 3rd October Programme for the day Saturday 4th October Programme for the day Sunday 5th October Programme for the day Poster Sessions Friday 3rd October Programme for the day

“…Introduction Scianna blood group system is carried on erythrocyte membrane-associated protein (ERMAP) (Wagner et al, 2003). Auto anti-Sc1 (Tregellas et al, 1979;Steane et al, 1982;McDowell et al, 1986;Owen et al, 1992) and anti-Sc3 (Peloquin et al, 1989) have been described, but is extremely rare. Case history/investigation A 61-year-old Caucasian female with Evan's syndrome and Ca breast with Hb 5Á5 g dL À1 was referred for investigation.…”