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Adverse Occurrence type:
Case reports, known to occur in patients with sickle cell disease.
Time to detection:
4 - 6 days
Alerting signals, symptoms, evidence of occurrence:
Delayed hemolysis after transfusion therapy that are strikingly similar and can be mistaken for sickle-cell pain crisis. All four patients described in this publication developed features of painful crisis 6 days after transfusion therapy.
Demonstration of imputability or root cause:
All three previously transfused units were typed Jka and the patient’s RBCs were Jk(a-b+). Antibody identification performed by ID-DiaMed Gel Techique (GT) showed the presence of anti-Jka with dosage phenomenon.
Suggest new keywords:
Sickle cell crisis
hemolytic transfusion reaction
sickle cell hemolytic transfusion reaction
- Diamond, W. J., Brown, F. L., Jr., Bitterman, P., Klein, H. G., Davey, R. J. and Winslow, R. M. (1980). Delayed hemolytic transfusion reaction presenting as sickle-cell crisis. Ann Intern Med 93(2):231-234.; - Petz LD, Calhoun L, Shulman IA, et al. The sickle cell hemolytic transfusion reaction syndrome. Transfusion. 1997;37:382–392. - Garratty G, Severe reactions associated with transfusion of patients with sickle cell disease. Transfusion. 1997;37:357–361.
Add the following references Petz LD, Calhoun L, Shulman IA, et al. The sickle cell hemolytic transfusion reaction syndrome. Transfusion. 1997;37:382–392. Garratty G, Severe reactions associated with transfusion of patients with sickle cell disease. Transfusion. 1997;37:357–361.
Expert comments for publication:
Incidence of delayed hemolytic transfusion reactions (DHTR) are much higher in patients with sickle cell disease compared to random patients who have received transfusions. DHTR may present with a number of distinctive features. In addition to the usual laboratory manifestations of hemolysis, patients may develop symptoms characteristic of a sickle cell pain crisis (as first described in this publication), and life-threatening anemia may develop as a result of drops in hemoglobin (Hb) and hematocrit (Hct) to levels markedly lower than those before transfusion. These conditions were labeled as sickle cell hemolytic transfusion reactions by Petz et al (Transfusion 1997). Others have also described hyperhemolysis after transfusion therapy, wherein in addition to the allo-red cells, patients own red cells are hemolysed.