백혈구증가증을 동반하는 소아 급성백혈병에 의한 백혈구울혈의 병태생리, 임상증상 및 응급처치
Leukostasis by hyperleukocytic childhood acute leukemia: pathophysiology, clinical presentation, and emergency care
남기룡, 백경윤, 전인상,
소속 상세정보
남기룡 ( Nam Ki-Lyong )
가천대학교 의과대학 길병원 응급의학교실
백경윤 ( Baek Gyeong-Yoon )
가천대학교 의과대학 길병원 소아과학교실
전인상 ( Jeon In-Sang )
가천대학교 의과대학 길병원 소아과학교실
Abstract
Hyperleukocytosis (HL), defined by a peripheral white blood cell (WBC) count exceeding 100,000/mm3, is occasionally observed in childhood acute leukemia. The increased viscosity in the micro-circulation by HL and the interaction between the leukemic blasts and endometrium of blood vessels sometimes result in leukostasis. Leukostasis can incur life-threatening manifestations, such as respiratory distress, brain infarction and hemorrhage, and renal failure, needing an emergency care. Although early stage of leukostasis is difficult to detect due to nonspecific manifestations, an emergency care is mandatory because leukostasis can proceed to a fatal course. Initial management includes an aggressive fluid therapy that can reduce WBC count, and prevent other metabolic complications implicated by HL. Packed red blood cells should be judiciously transfused because it increases blood viscosity. Conversely, transfusion of platelet concentrates or fresh frozen plasma, which does not affect blood viscosity, is recommended for prevention of hemorrhage. To reduce tumor burden, leukapheresis or exchange transfusion is commonly performed. However, the efficacy is still controversial, and technical problems are present. Leukapheresis or exchange transfusion is recommended if WBC count is 200,000-300,000/mm3 or more, especially in acute myelocytic leukemia, or manifestations of leukostasis are present. In addition, early chemotherapy is the ultimate management of leukostasis.
키워드
Disease Management; Emergency Service, Hospital; Leukemia; Leukocyte Disorders; Leukocytosis; Leukostasis
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