A case of delayed hemolytic transfusion reaction in sickle cell disease patient

Abstract

Sickle cell disease (SCD) is autosomal recessive, genetically transmitted hemoglobinopathy responsible for considerable morbidity and mortality. It is prevalent in many parts of India including Central India, where the prevalence in different communities has ranged from 9.4% to 22%. Perioperative management may include transfusion of red blood cells. Hemolytic transfusion reactions can occur, and these can be either acute or delayed. We present a case of delayed hemolytic transfusion reaction in a patient with SCD.

Introduction

Sickle cell disease (SCD) affects approximately 9.4–22% of individuals of central India.[1] Perioperative management may include transfusion of red cells. Other indications in which sickle cell patients require blood are:

1. Management of severe anemia

   1. Acute splenic sequestration
   2. Transient red cell aplasia
   3. Hyperhemolysis associated with infection
   4. Acute chest syndrome

2. Management of sudden severe illness

   1. Acute chest syndrome
   2. Stroke
   3. Sepsis
   4. Acute multiorgan failure.

This treatment is not without risk, and serious complications may arise ranging from acute or delayed hemolytic reaction. We present a case of delayed hemolytic transfusion reaction.[2]

Case Report

A 14-year-old girl with a history of aseptic necrosis at the right femur head [Figure 1] and fistula of long bones presented at Orthopaedics Department of Dhiraj Hospital, SBKSMIRC, Waghodia, Vadodara, Gujarat. She was to be taken for sequestrectomy. On admission, she was severely ill. The patients baseline full blood count revealed Hb of 4g/dl. Hemoglobin electrophoresis done at the time of admission shows Hb A – 0%, Hb F – 29%, Hb A2 – 5.1%, and Hb S – 65.9%. The pattern was consistent with SCD. She had a history of 3 bony crises per year and received about 10 transfusions in the past. Now, she was given 4 red blood cell (RBC) transfusions preoperatively over a course of a fortnight to achieve hemoglobin S % age of <30%. Blood transfused was screened for irregular antibodies, and it was negative. Direct Coombs test was negative before transfusion. She had no apparent ill effect at the time of transfusion, and her Hb was reported 8 after 4 blood transfusions. After 1 week of 4^th transfusion, she presented with jaundice, malaise, and weakness. Her Hb concentration was 5.4 g/dl with fall of about 3.6 g/dl of Hb, bilirubin level was 13.65 mg/dl (unconjugated 9.5 mg/dl), and LDH level was 3910 U/L. Direct Coombs test was positive. Her red cell serology identified anti-K and anti-Jk^b antibodies by AHG gel cards. A diagnosis of delayed hemolytic transfusion reaction was made. On the day of surgery, she was transfused 2 antigen negative blood units found by antigen typing. Postoperatively, she was comfortable. Her full blood count showed a value of 7.4 g/dl and her Hb concentration was stabilized. Patient was discharged after 7 days. On follow-up clinic, her Hb was 9.1 g/dl. No new alloantibodies had been detected in her serum.

Figure 1.

Aseptic necrosis of right femur head

Discussion

Delayed hemolytic transfusion reaction/hemolysis occurs typically 1 week posttransfusion and presents with back, leg, abdominal pain, fever, and hemoglobinuria.[3] It is due to stimulation of alloantibody by exposure to a foreign surface antigen on the transfused RBCs.[4] Delayed hemolytic transfusion reaction leads to fall in Hb levels.[5] In addition to delayed hemolytic transfusion reaction, patient may exhibit symptoms of pain crisis, marked reticulocytopenia, and worsening of anemia (due to fall in Hb) following transfusion.[6] To prevent delayed hemolytic transfusion reactions, it is essential to obtain prior transfusion records because alloantibodies identified may no longer be detectable but the patients should be supplied blood that is negative for corresponding antigens. Many institutions have a program to provide at least partially phenotypically matched blood for sickle cell patients who have developed multiple alloantibodies. This is clinically important in patients with SCD who may develop a complication called as sickle cell hemolytic transfusion reaction (bystander hemolysis) where autologous cells are destroyed as well as allogeneic cells.[7]

Our patient had baseline Hb of 4 g/dl preoperatively and her lowest Hb 1 week later after 4 transfusions was 5.4 g/dl, a fall of about 3.6 g/dl of Hb occurred after 4 transfusions. Once she was provided with antigen negative blood, her Hb concentration improved.

Conclusion

Our patient of SCD received blood transfusion preoperatively. As these patients are multitransfused and risk of alloimmunization is high so antibody screening and identification should be regularly carried out in this population and only antigen negative blood should be transfused.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.