가족성 가성고칼륨혈증의 헌혈 혈액 및 수혈 결과에 대한 영향

Abstract

Familial pseudohyperkalemia (FP) is a dominantly inherited condition caused by variants in the gene ABCB6 resulting in red blood cell (RBC) membrane protein defects. FP is generally asymptomatic. However, FP RBCs have an increased permeability to monovalent cations when stored below 37°C. Transfusion of RBC components donated by FP individuals can induce hyperkalemia and may be causally related to transfusion-associated hyperkalemic cardiac arrest, particularly in neonates and infants. Therefore it is necessary to accurately evaluate the frequency of FP occurrence in the Korean population and assess whether FP RBCs have significantly higher supernatant potassium levels. Efforts should be made to recognize the effects of blood products collected from FP donors on blood transfusion recipients to reduce the risk of hyperkalemia, especially in fetuses, infants, and patients at risk of this condition. (Korean J Blood Transfus 2023;34:87-91)