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Danila Sergeyevich Kuroedov , Jaime Pamplona , Carla Conceicao, Joao Lopes Dos Reis

Neuroradiology Department, Hospital Dona Estefânia e Hospital São José, Centro Hospitalar Universitário de Lisboa Central, Lisboa

- 43rd ESNR Annual Meeting, 5 a 9 Outubro 2020
- Neuroradiology (2020) 62 (Suppl 1): S51. DOI: 10.1007/s00234-020-02534-5

Introduction: Cerebral venous thrombosis is a recognized complication in hematological malignancies like acute lymphoblastic leukemia and acute lymphoblastic lymphoma (ALL). While it may be related to the disease itself by activation of blood coagulation via procoagulant substances or by impairment of fibrinolytic or anticoagulant pathways, it may also be related to the chemotherapy instituted, particularly Lasparaginase.
Method: We present four selected cases from our Department’s Neuroradiology database, with illustrative CT and MRI imaging showing sinovenous thrombosis (CSVT) following therapy with L-asparaginase in patients suffering from hematological malignancies.
Discussion: L-asparaginase is an enzyme that catalyzes the conversion of L-asparagine, necessary for lymphoblasts and other neoplastic cells, decreasing it’s plasmatic concentration, leading to inhibition of RNA and DNA synthesis with the subsequent blastic cell apoptosis. The incidence of thrombotic complications during L-asparaginase therapy varies from 1% to 37%, being cerebral venous thrombosis its leading form of presentation, reported in 1-3% of treated patients. Clinical features of headache, altered consciousness, focal neurological deficit, and seizures developing during or immediately after treatment with Lasparaginase should alert the treating physician and prompt to a correct evaluation of the patient.
Conclusion: The key management of this condition is early diagnosis by imaging as delayed institution of anticoagulation may in some cases be futile. Rapid replenishment of coagulation factors may be achieved with fresh frozen plasma although antithrombin concentrates are preferred for this. For therapeutic anticoagulation, low molecular weight heparin is given initially and this may be continued or it may be substituted by oral anticoagulants for 3–6 months, leaving endovascular treatment as last resort gateway. We conclude that, diagnosis of CSVT in ALL patients being treated with L-asparaginase requires a high index of clinical suspicion in the presence of seizures, focal neurological deficit, and features of raised intracranial pressure. Early diagnosis demands a low threshold for imaging, and MRI should complement CT study. Identification of relevant findings such as venous infarcts, the empty delta sign, and absent flow in the dural sinuses on CT and MR venography enables proper diagnosis and management.

Palavras Chave: Sinovenous Thrombosis, L-Asparaginase, hematological malignancies, chemotherapy, Asparaginase-induced deficiency of antithrombin III