DELAYED HAEMOLYTIC TRANSFUSION REACTIONS (DHTR): A MASQUERADE OF SICKLE CELL COMPLICATIONS
(Abstract release date: 05/18/17)
EHA Library. Fernandez-Leyva H. 05/18/17; 182864; PB2151
Harberth Fernandez-Leyva
Contributions
Contributions
Abstract
Abstract: PB2151
Type: Publication Only
Background
Patients with sickle cell disease (SCD) may require repeated red blood cells (RBCs) transfusion, putting them at risk from minor blood group alloimmunization and the development of delayed haemolytic transfusion reactions.
Aims
Reported a prevalence of recognized DHTR syndrome in patients with SCD.
Methods
We reviewed the cases of (DHTR) in SCD patients in a 5-year period (2010- 2016). A total of 10 patients had a clinical picture compatible with DHTR and underwent treatment with high dose steroids, intravenous immunoglobulins (IVIG) or erythropoietin. Any patient received Rituximab.
Results
The most common indications for transfusion were anemia due to vasococlusive sickle cell crisis or preoperative anaemia optimization. The cohort received partial exchange transfusion and phenotypically matched RBCs. Before transfusion the median of Hb level was 69 g/L (baseline range 80g/L) and the nadir at haemolysis episode was 38 g/L, Ht was 21.9%, WBC was 17.3 × 109 cells/L and mean LDH 1290 IU/L).
The median time to develop DHTR was seven days after the transfusion and approximately 6 days after the surgical interventions (range: 4–12 days) and all cases presented with symptoms of anaemia, jaundice, tiredness and tachycardia. The median age was 29 years with female predominance (6:4). Blood cultures were negative in 80% of patients and only positive in 2 cases. 30% of patients tested positive for viral infection on PCR. Mortality rate in our series was low (zero).
Pain episodes and other complications associated with DHTR was treated as required and four cases were successfully monitored in HDU. One patient required noninvasive ventilations and inotropic support. Two patients received RBCs transfusion (median 1.5 unit of packed RBCs). Possibly as their presentation mimics an acute vaso-oclusive crisis. In all cases haemoglobin stabilized and improved, symptoms resolved and patients were discharged on small course of oral antibiotics (median admission 6 days).
Conclusion
The symptoms of DHTR can easily be mistaken for other SCD complications, including infection and vaso-occlusive crisis.
The diagnosis of DHRT is based on clinical suspicion, when there is a rapid Hb drop after a recent RBC transfusion with clinical signs of haemolysis. To support the diagnosis, laboratory tests (serial FBCs, haemolysis screen, DAT, measurement of Hb S levels) and exclusion of other aetiologies are useful.
Whenever a DHTR is suspected, further RBC transfusion should be withheld unless absolutely necessary, as it may precipitate acceleration of the hemolytic reaction. Patients in whom the diagnosis of DHTR is missed may receive repeat transfusions, which may contribute to the complications associated with SCD.
The use of more extensive phenotypic matching of blood and minimizing RBC transfusion help to prevent DHTR.
The present series emphasize the importance of early recognition of symptoms and signs in correlation with a recent history of RBC transfusions, as DHTR can be a potentially life-threatening complication.
Session topic: 25. Sickle cell disease
Keyword(s): transfusion, sickle cell disease, Hemolysis
Abstract: PB2151
Type: Publication Only
Background
Patients with sickle cell disease (SCD) may require repeated red blood cells (RBCs) transfusion, putting them at risk from minor blood group alloimmunization and the development of delayed haemolytic transfusion reactions.
Aims
Reported a prevalence of recognized DHTR syndrome in patients with SCD.
Methods
We reviewed the cases of (DHTR) in SCD patients in a 5-year period (2010- 2016). A total of 10 patients had a clinical picture compatible with DHTR and underwent treatment with high dose steroids, intravenous immunoglobulins (IVIG) or erythropoietin. Any patient received Rituximab.
Results
The most common indications for transfusion were anemia due to vasococlusive sickle cell crisis or preoperative anaemia optimization. The cohort received partial exchange transfusion and phenotypically matched RBCs. Before transfusion the median of Hb level was 69 g/L (baseline range 80g/L) and the nadir at haemolysis episode was 38 g/L, Ht was 21.9%, WBC was 17.3 × 109 cells/L and mean LDH 1290 IU/L).
The median time to develop DHTR was seven days after the transfusion and approximately 6 days after the surgical interventions (range: 4–12 days) and all cases presented with symptoms of anaemia, jaundice, tiredness and tachycardia. The median age was 29 years with female predominance (6:4). Blood cultures were negative in 80% of patients and only positive in 2 cases. 30% of patients tested positive for viral infection on PCR. Mortality rate in our series was low (zero).
Pain episodes and other complications associated with DHTR was treated as required and four cases were successfully monitored in HDU. One patient required noninvasive ventilations and inotropic support. Two patients received RBCs transfusion (median 1.5 unit of packed RBCs). Possibly as their presentation mimics an acute vaso-oclusive crisis. In all cases haemoglobin stabilized and improved, symptoms resolved and patients were discharged on small course of oral antibiotics (median admission 6 days).
Conclusion
The symptoms of DHTR can easily be mistaken for other SCD complications, including infection and vaso-occlusive crisis.
The diagnosis of DHRT is based on clinical suspicion, when there is a rapid Hb drop after a recent RBC transfusion with clinical signs of haemolysis. To support the diagnosis, laboratory tests (serial FBCs, haemolysis screen, DAT, measurement of Hb S levels) and exclusion of other aetiologies are useful.
Whenever a DHTR is suspected, further RBC transfusion should be withheld unless absolutely necessary, as it may precipitate acceleration of the hemolytic reaction. Patients in whom the diagnosis of DHTR is missed may receive repeat transfusions, which may contribute to the complications associated with SCD.
The use of more extensive phenotypic matching of blood and minimizing RBC transfusion help to prevent DHTR.
The present series emphasize the importance of early recognition of symptoms and signs in correlation with a recent history of RBC transfusions, as DHTR can be a potentially life-threatening complication.
Session topic: 25. Sickle cell disease
Keyword(s): transfusion, sickle cell disease, Hemolysis
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