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Background and Objectives Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare and fatal complication caused by blood transfusion. In this condition, donated lymphocyte cells attack host tissues. In this review article, the history, prevalence, pathogenesis, clinical symptoms, risk factors, diagnostic methods, strategies to reduce its occurrence, prognosis, disease management, and laboratory characteristics of this condition are discussed. Materials and Methods For this purpose, a search was made using published reliable research in Google Scholar, PubMed, and MEDLINE databases and the findings of more than 70 articles were cited. Results The following study showed that live T lymphocytes from donated blood products and the resulting cytokine storm play a role in the occurrence of this complication. The symptoms of this disease appear in the form of skin rash, pancytopenia, and fever, and its diagnosis is based on clinical symptoms and laboratory findings of skin biopsy, increased liver enzymes, confirmation of lymphocyte chimerism using STR-PCR, and determination of the HLA phenotype of lymphocytes and FISH method. This complication has a higher prevalence in populations with high genetic similarity or people with immune deficiencies, however, it also occurs in other people who do not have these conditions or who have partial donor-recipient HLA matching (one-way HLA matching). Conclusions This complication is one of the fatal complications caused by blood transfusion that occurs within 2 to 30 days after blood products transfusion; the typical cases are associated with more than 90% mortality and mild or atypical cases have longer survival and are associated with a better prognosis that may go undetected or be treated spontaneously. [ABSTRACT FROM AUTHOR]