Pediatr Emerg Med J.  2019 Dec;6(2):35-41. 10.22470/pemj.2019.00150.

Leukostasis by hyperleukocytic childhood acute leukemia: pathophysiology, clinical presentation, and emergency care

• Nam KL
• Baek GY
• Jeon IS

• KMID: 2469716
• DOI: http://doi.org/10.22470/pemj.2019.00150

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 definite treatment of leukostasis.

Keyword

Disease Management; Emergency Service, Hospital; Leukemia; Leukocyte Disorders; Leukocytosis; Leukostasis

MeSH Terms

Blood Platelets
Blood Vessels
Blood Viscosity
Brain Infarction
Disease Management
Drug Therapy
Emergencies*
Emergency Medical Services*
Emergency Service, Hospital
Endometrium
Erythrocytes
Female
Fluid Therapy
Hemorrhage
Leukapheresis
Leukemia*
Leukemia, Myeloid, Acute
Leukocyte Disorders
Leukocytes
Leukocytosis
Leukostasis*
Plasma
Renal Insufficiency
Tumor Burden
Viscosity