A 15-year-old girl who was being followed up with the diagnosis of thalassemia major applied to us for bone marrow transplantation (BMT) to be performed from her sibling. From her medical history, it was learnt that she had not gained enough weight after her birth, and her hematological tests had revealed low hemoglobin (6.2 g/dl) levels, hepatomegaly, and splenomegaly that had been detected when she was 4 months old. Detection of HbF 89.5%, HbA2 1.9%, HbA 3.1% in her hemoglobin electrophoresis, and parents being thalassemia carriers had established the diagnosis of thalassemia major. The patient was followed up with recurrent blood transfusions. It was learned that tissue groups of her brother born when she was at the age of 13 were compatible with hers. So his cord blood was stored for future use. However, it was not in sufficient amount; therefore, bone marrow samples were harvested from her sibling and frozen. When the patient with O Rh (+) blood group was 15 years old, BMT was performed from his brother who had A Rh (+) blood group. Previously, as a preparatory protocol, the patient was given busulfan for 4 days, then cyclophosphamide + mesna for another 4 days followed by cyclosporine treatment. From the beginning of the preparatory phase, she received maintenance dose of defibrotide. The patient developed macroscopic hematuria, oligoanuria, and concomitant hypertension immediately after the application of the BMT product. The baseline creatinine value of the patient was 0.6 mg/dl, but increased to 1 mg/dl at 24 h after transplantation, and then up to 4.5 mg/dl. Simultaneous AST (145 U/L), ALT (169 U/L), LDH (1132 U/L) elevation, anemia (Hb, 6.5 g /dl), thrombocytopenia (60.000), and indirect hyperbilirubinemia (3.8 g/dl) were detected. Uric acid was in normal range. There was no evidence of hemolysis in peripheral smear. Urine was dark red-brown in color (Fig. 1). Strip test revealed the presence of blood (3+) in urine, while erythrocytes were not detected during microscopic examination. During physical examination, painful hepatomegaly which descended 6 cm below the costal margin was detected on palpation. Renal Doppler US performed due to sudden development of hematuria and oligoanuria was not remarkable. Furosemide infusion was started, while IV hydration at a rate of 3000 cc/m2 was maintained, and the patient was monitored with CVP. ADAMTS-13 test was requested to detect possible TTP. Since vaso-occlusive disease could not be excluded because of the presence of thrombocytopenia and painful hepatomegaly, the treatment dose of defibrotide was increased. The treatments she was receiving were adjusted in consideration of her kidney injury, and cyclosporine was discontinued. In the follow-up, her anemia and thrombocytopenia persisted and resisted to treatment. Although furosemide infusion was increased to the maximum dose, azotemia of the patient with insufficient urine output deteriorated further, and she had respiratory distress with symptoms of fluid overload (pulmonary edema). Therefore, her fluid intake was restricted, and continuous hemodiafiltration (CHDF) and high-flow oxygen treatments were started in the intensive care unit. On the second day of the intensive care follow-up, urine color of the patient was lightened, increase in LDH ceased, and her urine output increased. After 2 days of CHDF and three sessions of conventional hemodialysis, the patient did not need additional dialysis. In the follow-up, the creatinine values returned to baseline levels, and chimerism was 100% in the post-BMT assessment. The patient’s normal kidney function is still normal 1 year after the transplantation.
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