2 cases of pegylated asparaginase-associated cerebral venous sinus thrombosis in children: clinical analysis and literature review.

BACKGROUND

Cerebral venous sinus thrombosis (CVST) is a serious complication in children with acute lymphoblastic leukemia (ALL) undergoing chemotherapy with pegylated asparaginase (PEG-Asp). This study aims to investigate its risk factors, clinical features, and prognosis.

METHODS

We retrospectively analyzed the clinical manifestations, laboratory findings, imaging characteristics, treatment, and outcomes of two pediatric ALL patients who developed CVST during induction remission therapy at our center. Additionally, a systematic literature review was conducted to contextualize our findings.

RESULTS

Both children were treated per the CCCG-ALL-2020 protocol and developed CVST approximately two weeks after the first PEG-Asp dose, presenting with generalized tonic-clonic seizures. Notably, platelet counts were normal at presentation. Coagulation tests primarily showed decreased fibrinogen (FIB) and antithrombin III (AT-III). Head magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) confirmed venous sinus thrombosis with hemorrhage. Management included fresh frozen plasma and fibrinogen infusion, mannitol for intracranial pressure reduction, and rivaroxaban for anticoagulation. Both patients recovered without recurrence of thrombosis upon subsequent PEG-Asp exposure.

CONCLUSION

During ALL chemotherapy involving PEG-Asp, regular monitoring of coagulation parameters (especially FIB and AT-III) is crucial. Unexplained neurological symptoms should raise suspicion for CVST, warranting prompt MRI + MRV. Anticoagulation with rivaroxaban was effective in these cases, yielding favorable outcomes. CVST complicating the induction phase of ALL requires heightened clinical awareness for early prevention, diagnosis, and treatment.