Akademik Veri Yönetim Sistemi
Thalassemia and Hemoglobinopathies, Duran Canatan, Editör, Nobel Yayın Dağıtım, Ankara, ss.234-244, 2024
Thalassemias are a heterogeneous
group of diseases that are inherited in an autosomal recessive manner and are
characterized by hypochromic, microcytic anemia that develops as a result of defective
synthesis of one or more of the hemoglobin chains. A significant portion of
patients defined as transfusion-dependent thalassemia (TDT) patients require
frequent and continuous blood transfusion. Transfusion practices, as is known,
involve many risks of complications. One of the most important difficulties
encountered in thalassemia patients during the transfusion process is alloantibodies against foreign RBC antigens. In
alloimmunized patients, treatment effectiveness will decrease as a result of
increased RBC destruction after transfusion. Additionally, RBC autoantibodies
may develop in addition to alloantibodies in TDT patients. To date, 45 blood group
systems and 360 blood group antigens assigned to these systems have been
identified. Current guidelines recommend transfusion of blood matched for
clinically important antigens such as C, c, E, e and K, in addition to ABO and RhD
blood groups, in thalassemia patients. In fact, expanded antigen typing is also
recommended in allo- and/or autoimmunized patients. However, various
difficulties are encountered in serological antigen typing (phenotyping) in
patients who have recently transfused or have developed allo-/autoantibodies.
Therefore, in such patients, molecular blood group typing (genotyping) seems to
be a solution that has recently been proposed and become widespread. It should
also be noted that a large donor database with typed RBC antigens is required
in order to find blood components with the required antigenic properties. For this
purpose, particularly high-throughput molecular methods are used. Another
problem that is rarely encountered is providing the rare blood component needed
to a patient who is negative for a high incidence antigen. For his purpose the International
Rare Donor Panel (IRDP) was established in 1965 under a joint initiative of the
World Health Organization (WHO) and ISBT to facilitate the rapid availability
and exchange of rare blood between countries.