Deferasirox/deferoxamine/prednisone

Abstract

In a case report, 2 patients aged (a girl and a 5-year-old boy) [not all exact ages on event onsets stated] were described, who developed acute renal failure following treatment with deferasirox for Diamond-Blackfan anaemia (DBA). Additionally, the boy developed abdominal pain, polyuria, poor general condition, vomiting and proximal tubule dysfunction following treatment with deferasirox while the girl developed acute gastroenteritis, acute renal injury, complex proximal tubulopathy and cutaneous abscesses at the puncture sites following treatment with deferoxamine and deferasirox, and exhibited lack of efficacy during treatment with prednisone [routes not stated; not all dosages stated].Case 1 (the girl): The girl had a history of DBA at 3 months of the age. Hence, from 3 months to 6 years of the age, she was treated with prednisone. However, she showed resistance to prednisone (lack of efficacy). Therefore, she initiated treatment with periodic transfusions. Further, laboratory tests and MRI findings showed hepatic iron overload. Consequently, she was treated with deferoxamine. However, she developed cutaneous abscesses at the puncture si­tes. Hence, her treatment was switched to deferasirox 23 mg/Kg/day. Subsequently, she presented with acute gastroenteritis and acute renal injury (AKI). The laboratory findings showed; elevated crea­tinine, hypokalaemia, hypophospha­taemia and hypouricaemia. All signs and symptoms were compatible with complex proximal tubulopathy. Therefore, deferasirox was discontinued, and she was initiated treatment with potassium and phosphate supplements. Thereafter, nor­mal lab values were achieved. Later, potassium and phosphate supplements were reduced, and a low dose of deferasirox 16 mg/kg/day was re-initiated. Thereafter, her renal function and ferritin levels remained in the normal ranges.Case 2 (the 5-year-old boy): The boy had a history of DBA at 2 months of the age. Hence, he received periodic transfusions. He showed resistance to an unspecified corticosteroid treatment. Thereafter, at the age of 2 years, he initiated treatment with deferasirox 10 mg/kg/day. Further, laboratory tests and MRI findings showed hepatic iron overload and ferritin levels of 500 ng/mL. Hence, the dose of deferasirox was increased to 30 mg/kg/day. However, he presented to the emergency department with abdominal pain, polyuria, poor general condition and vomiting. On the physical examination, moderate dehydration was noted. He was diagnosed with acute renal failure. He also had hypokalaemia, hypophosphataemia, hypouricaemia, metabolic acidosis and glycosuria, which were consisted of proximal tubule dysfunction. Hence, deferasirox was discontinued. After the stabilised condition, deferasirox was re-initiated with the lower dose of 5 mg/kg/day. It was reported that dehydration was occurred due to vomiting and polyuria secondary to tubulo­pathy [not all outcome stated; time to reactions onsets not stated].