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Delayed hemolytic transfusion reaction/hyperhemolysis syndrome in children with sickle cell disease
Julie-An M Talano1, Cheryl A Hillery, Jerome L Gottschall
1Medical College of Wisconsin, Department of Pediatric Hematology/Oncology, MACC Fund Research Center, Milwaukee, WI 53226, USA. jtalano@mail.mcw.edu
Insights
Delayed hemolytic transfusion reaction/hyperhemolysis (DHTR/H) syndrome is a serious complication in pediatric sickle cell disease patients following red blood cell transfusions. This condition presents with pain, fever, and hemoglobinuria, often mimicking a pain crisis and leading to severe complications.
Area of Science:
- Hematology
- Transfusion Medicine
- Pediatric Sickle Cell Disease
Background:
- Alloimmunization affects 5-36% of sickle cell disease (SCD) patients.
- Delayed hemolytic transfusion reaction/hyperhemolysis (DHTR/H) syndrome occurs in 11% of alloimmunized patients.
- DHTR/H in SCD patients presents with hemolysis, pain, and anemia 1 week post-transfusion, often with negative DAT and no new antibodies.
Purpose of the Study:
- To describe clinical and laboratory findings of DHTR/H syndrome in pediatric SCD patients.
- To highlight the diagnostic challenges and complications of DHTR/H in this population.
- To emphasize the need for including DHTR/H in the differential diagnosis for SCD patients with transfusion reactions.
Main Methods:
- Retrospective chart review of SCD patients with transfusion reactions over 11 years.
- Defined DHTR/H by specific criteria: accelerated hemolysis 4-10 days post-transfusion with Hb fall, elevated LDH, bilirubin, and hemoglobinuria.
- Excluded patients with acute transfusion reactions.
Main Results:
- Seven pediatric patients experienced 9 episodes of DHTR/H syndrome 6-10 days post-RBC transfusion.
- Common symptoms included fever, hemoglobinuria, and pain (mimicking vaso-occlusive crisis).
- Direct antiglobulin test (DAT) was positive in only 2 episodes; 8 episodes showed Hb below pretransfusion levels. Severe complications included acute chest syndrome, pancreatitis, heart failure, and renal failure.
Conclusions:
- DHTR/H syndrome in pediatric SCD patients typically manifests ~1 week post-transfusion with pain, fever, and hemoglobinuria, potentially mistaken for pain crisis.
- Negative DAT and reticulocytopenia are common, with Hb often dropping below pretransfusion levels.
- DHTR/H is a serious, potentially life-threatening complication in pediatric SCD, necessitating avoidance of further transfusions if possible and inclusion in differential diagnoses.
Objective:
Alloimmunization in patients with sickle cell disease (SCD) has a reported incidence of 5% to 36%. One complication of alloimmunization is delayed hemolytic transfusion reaction/hyperhemolysis (DHTR/H) syndrome, which has a reported incidence of 11%. In patients with SCD, clinical findings in DHTR/H syndrome occur approximately 1 week after the red blood cell (RBC) transfusion and include the onset of increased hemolysis associated with pain and profound anemia. The hemoglobin (Hb) often drops below pretransfusion levels. In many reported adult cases, the direct antiglobulin test (DAT) remains negative and no new alloantibody is detected as the cause for these transfusion reactions. To date, few pediatric cases have been reported with this phenomenon. The objective of this study was to describe the clinical and laboratory findings of a case series in children who had SCD and experienced a DHTR/H syndrome at our institution.
Methods:
An 11-year retrospective chart review of patients with discharge diagnosis of SCD and transfusion reaction was performed. DHTR/H syndrome was defined as the abrupt onset of signs and symptoms of accelerated hemolysis evidenced by an unexplained fall in Hb, elevated lactic dehydrogenase, elevated bilirubin above baseline, and hemoglobinuria, all occurring between 4 and 10 days after an RBC transfusion. Patient characteristics, time from transfusion, symptoms, reported DAT, new autoantibody or alloantibody formation, laboratory abnormalities, and complications were recorded. Patients with acute transfusion reactions were excluded.
Results:
We encountered 7 patients who developed 9 episodes of DHTR/H syndrome occurring 6 to 10 days after RBC transfusion. Each presented with fever and hemoglobinuria. All but 1 patient experienced pain initially ascribed to vaso-occlusive crisis. The DAT was positive in only 2 of the 9 episodes. The presenting Hb was lower than pretransfusion levels in 8 of the 9 events. Severe complications were observed after the onset of DHTR/H: acute chest syndrome, n = 3; pancreatitis, n = 1; congestive heart failure, n = 1; and acute renal failure, n = 1.
Conclusions:
DHTR/H syndrome occurs in pediatric SCD patients, typically 1 week posttransfusion, and presents with back, leg, or abdominal pain; fever; and hemoglobinuria that may mimic pain crisis. Hb is often lower than it was at the time of original transfusion, suggesting the hemolysis of the patient's own RBCs in addition to hemolysis of the transfused RBCs; a negative DAT and reticulocytopenia are often present. Severe complications including acute chest syndrome, congestive heart failure, pancreatitis, and acute renal failure were associated with DHTR/H syndrome in our patients. DHTR/H in the pediatric sickle cell population is a serious and potentially life-threatening complication of RBC transfusion. It is important to avoid additional transfusions in these patients, if possible, because these may exacerbate the hemolysis and worsen the degree of anemia. DHTR/H syndrome must be included in the differential of a patient who has SCD and vaso-occlusive crisis who has recently had a transfusion.