Cerebral venous sinus thrombosis in children with acute lymphoblastic leukemia carrying the MTHFR TT677 genotype and further prothrombotic risk factors.
BACKGROUND
The present study was designed to prospectively evaluate the role of prothrombotic risk factors in leukemic children treated according to the ALL-BFM 90/95 study protocols with respect to the onset of cerebral venous sinus thrombosis.


METHODS
317 consecutive leukemic children aged 6 months to 18 years were enrolled in this study. 288 of the 317 patients were available for thrombosis-free survival analysis.


RESULTS
In 17 (5.9%) of these 288 patients cerebral venous sinus thrombosis occurred. The overall event-free survival of thrombosis in the central nervous system in patients with at least one prothrombotic defect (n = 12) was significantly reduced compared with patients without a prothrombotic defect (p < 0.0001). 15 patients showed acute clinical symptoms at onset of cerebral venous sinus thrombosis, two were asymptomatic. Three of the 17 patients affected (17.6%) died directly associated with the thrombotic event during induction therapy, the remaining 14 patients did not show prolonged clinical symptoms.


CONCLUSIONS
Prothrombotic risk factors should be included in a screening program in ALL children treated according to the BFM study protocols. Further prospective studies are recommended to establish adequate prophylactic anticoagulant treatment during ALL (BFM) polychemotherapy.
Wermes
C
C
Pediatric Hematology/Oncology, University Hospitals Bonn, Germany.
Fleischhack
G
G
Junker
R
R
Schobess
R
R
Schwabe
D
D
Sykora
K W
KW
Nowak-Göttl
U
U
D000293:Adolescent; D002648:Child; D018572:Disease-Free Survival; D005260:Female; D005838:Genotype; D006801:Humans; D007020:Hypoprothrombinemias / Q000235:genetics*; D015994:Incidence; D008297:Male; D042965:Methylenetetrahydrofolate Reductase (NADPH2); D000587:Oxidoreductases Acting on CH-NH Group Donors / Q000235:genetics*; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications* / Q000188:drug therapy / Q000235:genetics*; D011446:Prospective Studies; D012307:Risk Factors; D012851:Sinus Thrombosis, Intracranial / Q000453:epidemiology* / Q000209:etiology* / Q000401:mortality / Q000517:prevention & control; D015996:Survival Rate
D000587:Oxidoreductases Acting on CH-NH Group Donors; D042965:Methylenetetrahydrofolate Reductase (NADPH2)