Background:
Blood transfusion is a key treatment of sickle cell disease (SCD) complications. Delayed hemolytic transfusion reaction (DHTR) is a delayed reaction, that occurs days to weeks following a transfusion, characterized by mild anemia and/or hyperbilirubinemia and is one of the serious complications of blood transfusion. The symptoms of DHTR resemble those of vaso-occlusive crisis secondary to SCD leading to difficulty or delaying in diagnosis of DHTR. DHTR may lead to multiple organ failure and death.
Case report:
A 31-year-old female patient with a known case of SCD presented to our ER in King Fahad hospital Hofuf in the Kingdom of Saudi Arabia, with a history of generalized body ache, exertional dyspnoea, headache and easy fatigability for a few days on a background history of episodic hospital admissions for SCD, but she was admitted 3 times over the previous 6 months and received 6 units of packed red blood cells (PRBCs). The last blood transfusion was 18 days earlier. She was sick and her Hb level was 4.5 g/dL with positive Coombs test and positive alloantibodies, diagnosed as DHTR. We treated her with prednisolone tablets 1 mg/kg daily, intravenous immunoglobulins, 0.4 gm/kg daily for 5 days, and rituximab 500 mg IV every week for 4 weeks. Her Hb level raised up to 8.2 g/dL and she was discharged in good condition.
Conclusion:
Identifying risk factors for DHTR by history and presentation is urgently needed in order to risk stratify the transfusion regimen. It is important to avoid additional transfusions in these patients if possible because these may exacerbate the hemolysis and worsen the degree of anemia.