Q: 22 years old Afro-American male with a known history of sickle cell disease (SCD) - recently discharged from the hospital - is admitted back to ICU with sickle cell crisis and severe anemia. Hemoglobin (Hb) is reported as 3 g/dL Two weeks ago, on discharge, his Hb was 8 g/dL after he received two units of pRBC. Hematology fellow wrote the diagnosis of bystander hemolysis. What is bystander hemolysis?
Answer: Patients with sickle cell disease (SCD) often develop a delayed hemolytic transfusion reaction, after a week or two of pRBC infusion. The term was introduced by Drs. Petz and Garratty in 1997 as: " immune hemolysis of RBCs that are negative for the antigen against which the relevant antibody is directed." Allegedly activation of complement system is involved. The treatment is threefolds: avoidance of transfusion, steroids, and enhancing erythropoiesis. Recently, immune-modulating medications such as rituximab have been tried in cases of Delayed Hemolytic Transfusion Reactions (DHTR).
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References:
1. Scheunemann LP, Ataga KI. Delayed hemolytic transfusion reaction in sickle cell disease. Am J Med Sci. 2010;339(3):266-269. doi:10.1097/MAJ.0b013e3181c70e14
2. Petz LD, Calhoun L, Shulman IA, et al. The sickle cell hemolytic transfusion reaction syndrome. Transfusion. 1997;37:382–392.
3. Garratty G. Severe reactions associated with transfusion of patients with sickle cell disease. Transfusion. 1997;37:357–361.
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