Thrombosis in childhood acute lymphoblastic leukaemia: epidemiology, aetiology, diagnosis, prevention and treatment

Abstract

Acute lymphoblastic leukaemia (ALL) is the most common malignancy associated with venous thromboembolism (VTE) in children. The prevalence of symptomatic VTE ranges from 0% to 36%, and the variation can be explained, at least in part, by differences in chemotherapeutic protocols. The mechanism for increased risk of VTE is associated with alterations in the haemostatic system by use of L-asparaginase (ASP) alone or in combination with vincristine or prednisone, presence of central venous lines (CVLs) and/or inherited thrombophilia. The children at greatest risk are generally those receiving Escherichia coli ASP concomitant with prednisone. The majority of symptomatic VTEs occur in the central nervous system or in the upper venous system. In the majority of cases, asymptomatic VTEs are associated with CVLs. External CVLs are affected more often than internal CVLs. Evidence-based guidelines on prevention and treatment guidelines for ALL-related VTE are lacking, and carefully designed clinical trials are needed urgently.