Hyperhemolysis Syndrome in a Patient With B-Thalassemia Due to an Anti-Jka Alloantibody

Abstract

Hyperhemolysis syndrome (HS) is a delayed type of transfusion reactions (DTHRs). These may occur in patients with hemoglobinopathies due to destruction of red blood cells (RBCs). A 17-year-old boy with B-thalassemia and a history of multiple transfusions, after several RBCs transfusions developed hemolysis and hemoglobinuria with a decreased hemoglobin (Hb) level in the absence of bleeding that continued with splenomegaly. The antibody screen test (LISS AHG) and direct anti-globulin test (DAT) were performed. An 11-cell antibody identification panel (IBTO home-made) was used. Acid elution procedure was done using a commercial kit. The patient RBC phenotyping was attempted by treating the cells, following a chloroquine treatment procedure. Laboratory findings showed that his blood group was "A" Rh positive. His RBC phenotyping was negative for Jka antigen. The LISS antibody screening test result was negative with a weakly positive auto control result but DAT was positive for both C3d and IgG. The antibody identification test showed the presence of anti-Jka alloantibody and also anti-Jka was identified with eluate reacting stronger with homozygous cells (Jka+Jka+). The patient received steroid and IVIG as the main treatment and followed by receiving compatible blood units but hemolysis was not resolved and his Hb level was not increased, finally he was splenectomized. The patient's Hb level and clinical symptoms improved after the splenectomy, showing that the spleen may have a role in the destruction of the recipient and the donor blood cells.

Red Blood Cell Immunization and Contributing Factors in 685 Thalassemia Patients

“…These patients consented to participate in this study allowing for their blood samples to be collected and analyzed as problematic cases. ABO and Rh(D) typing, antibody screening and antibody identification (ABID) tests, and direct antiglobulin testing (DAT) were performed on the patients, according to our previous report 9 . In brief, ABO and Rh(D) blood typing was done (Anti-A, Anti-B, & Anti-D IBRF Holding Co., Tehran, Iran) using the automated technique (Diagast 251/AV.AVINEE-5912 Loos, France).…”