AUTOANTI-ENaFS PRODUCTION IN A PATIENT WITH SCID AFTER CORD BLOOD TRANSPLANTATION

• Published in: Journal of the Japan Society of Blood Transfusion Vol. 50; no. 3; pp. 444 - 452
• Main Authors: Tani, Yoshihiko, Takahashi, Junko, Seno, Taiko, Yamashita, Naoko, Kimura, Keiko, Nakade, Toru, Hirayama, Fumiya, Yamano, Hajime, Ikemiya, Misako, Ishii, Takefumi, Sako, Masahiro, Shibata, Hirotoshi
• Format: Journal Article
• Language: Japanese
• Published: The Japan Society of Transfusion Medicine and Cell Therapy 2004
• Subjects: AIHA (autoimmune hemolytic anemia); autoanti-EnaFS; CBT (cord blood transplantation); CPC (coil planet centrifuge) method; En (a-) blood; SCID (severe combined immunodeficiency disease)
• ISSN: 0546-1448; 1883-8383
• DOI: 10.3925/jjtc1958.50.444

A patient with severe combined immunodeficiency (SCID) underwent cord blood transplantation (CBT) at the age of 7 months. Her blood type was B, D+ and that of the cord blood (CB) offered by the bank was O, D+. Although the complete engraftment of CB had been achived, severe hemolytic anemia and thrombocythemia appeared at day 158 with an episode of fever probably due to an infection. Transfusions of washed O RBCs were not effective. Direct anti-globulin test (DAT) registered positive at day 166. Her serum was reactive with all RBCs including her own by the saline method (at room temperature), and autoanti-I (+i?) were identified. All RBCs except En (a-) and MKMK were reactive by the saline (37°C), bromelin method and the anti-globulin test. The results of reactivities with enzyme-treated RBCs indicated that autoanti-EnaTS was responsible, which was also eluted from her RBCs. Her anemia was improved by transfusions of B En (a-) blood. The coil planet centrifuge (CPC) analysis employed for monitoring of the survival of the B En (a-) blood in the patient revealed that the transfused RBCs survived for up to 90 days. As En (a-) blood donors are very rare in Japan, we need international collaboration in obtaining a supply of blood.