beta 0-Thalassemia complicated by autoimmune hemolytic anemia. Globin synthesis during immunosuppressive therapy.
The unusual occurrence of both beta o-thalassemia and autoimmune hemolytic anemia (AIHA) in a 28-month-old child is reported to illustrate special diagnostic and therapeutic studies. The diagnosis of beta o-thalassemia was established by clinical, family and globin synthesis studies. The diagnosis of AIHA was confirmed by the shortened survival of transfused autologous and homologous red blood cells and by positive direct antiglobulin tests. During treatment of the AIHA were corticosteroids and various immunosuppressive drugs, globin synthesis studies were performed to evaluate the coincidental effects on gamma/alpha-globin chain synthetic ratios. A 50% increase in the gamma/alpha synthetic ratio during cyclophosphamide treatment suggests that further studies of the possible benefits of cytotoxic drug therapy in beta-thalassemia may be indicated.
D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications* / Q000188:drug therapy; D001803:Blood Transfusion; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004905:Erythrocyte Aging; D005260:Female; D005914:Globins / Q000096:biosynthesis; D006801:Humans; D013789:Thalassemia / Q000150:complications*
D003520:Cyclophosphamide; D005914:Globins