Delayed hemolytic transfusion reaction with multiple alloantibody (Anti S, N, K) and a monospecific autoanti-JKb in intermediate β-thalassemia patient in Tabriz
A 37-year-old male with intermediate β-thalassemia syndrome was admitted to Ghazi Tabatabai Hospital. The patient’s blood type was O, Rh positive and his medical history included two previous transfusions. Throughout the past transfusions, not done in our center, he had unknown and unfamiliar transfusion reactions without any follow-up. At the time of admission to our center, laboratory data showed Hb 7.8 g/dl, RBC count 3.33×106/μL, White Blood Cell (WBC) count 4.33×103/μL, Hct 24.2% and serum Ferritin 840 ng/mL. The patient received two units of cross matched compatible and concentrated RBCs.\nEight days later a severe reaction was observed with clinical evidence which included tachycardia, fatigue, fever, back pain, chest discomfort, jaundice, nausea and anorexia. The patient was referred to an intensive care unit, and medication to modulate the immune system, including corticosteroid (Prednisolone 2 mg/kg) and high dose immunoglobulin (IV Immunoglobulin, 0.4 g/kg/day, for 7 days) were initiated.\nLaboratory data showed: Hb 4.8 g/dL, Hct 15.9%, Urea 40 mg/dL, creatinine 0.8 mg/mL; total billirubin 2.8 mg/dl, direct billirubin 0.5 mg/dl, and reticulocyte count 0.7%. He had a mild Hepatomegaly, and severe Splenomegaly (186 × 104 mm). DHTR was suspected. Coombs Test for antibodies against RBC Antibody Identification was performed. Laboratory tests showed the presence of three alloantibody: Anti-N, anti-S, anti-K, and a monospecific anti-JKb autoantibody, which is a warm auto antibody with single specific in the serum of patient, due to an autoimmune process.\nDAT was performed. Red cells from the EDTA tube were washed three times and a 3% cell suspension was made. A drop of cell suspension and the anti-Human Globulin (LORNE Laboratories Ltd, UK) was mixed in a tube and then centrifuged. The eluate was tested with panel cells by indirect antiglobulin test to know whether there were antibodies in patients’ serum which react with RBCs in vitro. Anti-Human Globulin reagents (LORNE Laboratories LTD., UK) and commercial panel cells (Iranian Blood and Transfusion Organization, Iran) were used.\nSeven days later, red cell units lacking anti-JKb, N, K, and anti-S antigens were transfused. He had mild reaction to these red cells, like mild pain in the back, fever, and hemoglobinuria. Further, RBC transfusions were stopped and the patient’s Hb stabilized at approximately 8 g/dL.