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Superior sagittal sinus thrombosis in the course of mixed phenotype acute leukaemia treated with acute lymphoblastic leukaemia-like therapy—a case report
Thrombosis Journal ( IF 3.1 ) Pub Date : 2023-11-16 , DOI: 10.1186/s12959-023-00561-9
Wojciech Lizurej 1 , Łukasz Mazurkiewicz 1 , Michał Kowalski 1 , Sylwia Szydłowska 1 , Michał Wyrzykowski 2 , Krzysztof Lewandowski 1
Affiliation  

Mixed phenotype acute leukaemia (MPAL) is associated with worse overall survival, compared with other acute leukaemias in adults. Lack of clear treatment guidelines makes the therapy challenging. ALL-like induction and consolidation treatment followed by allo-HSCT is the preferred first-line treatment. We present a case of a 36-year-old woman diagnosed with MPAL (EGIL Myelo/B) with KMT2A rearrangement, treated with the PALG-ALL-7 (including PEG-asparaginase) protocol. On day 25 after the induction therapy initiation, numbness of limbs and dizziness were observed. Therefore, the imaging studies (CT and MRI) were performed and a diagnosis of thrombosis of superior sagittal sinus of the brain was established. Routinely performed blood coagulation tests showed prolonged APTT and PT, decreased antithrombin III activity and decreased free protein S concentration. LMWH treatment and substitutional therapy with antithrombin III were started, which resulted in a significant reduction in the thrombosis associated symptoms and improvement of the neurological status after 3 days. After induction and consolidation therapy, the patient obtained complete haematological remission and negative measurable residual disease. Six months after the diagnosis, allo-HSCT was successfully performed. During the 4 months follow-up, the patient remained MRD negative and thrombotic symptoms free. To the best of our knowledge, our communication has been the first report of such complication in an MPAL patient treated with PEG-asparaginase containing protocol in adults. We recommend increased vigilance in patients manifesting any mild neurological symptoms and early decision about the MRI study performance.

中文翻译:

混合表型急性白血病急性淋巴细胞白血病样治疗过程中上矢状窦血栓形成一例报告

与成人的其他急性白血病相比,混合表型急性白血病(MPAL)与较差的总体生存率相关。缺乏明确的治疗指南使得治疗具有挑战性。类 ALL 诱导和巩固治疗,然后进行异基因造血干细胞移植 (allo-HSCT) 是首选的一线治疗。我们介绍了一名 36 岁女性的病例,她被诊断患有 MPAL (EGIL Myelo/B) 并伴有 KMT2A 重排,并接受 PALG-ALL-7(包括 PEG-天冬酰胺酶)方案治疗。诱导治疗开始后第25天,观察到四肢麻木和头晕。因此,进行了影像学检查(CT和MRI)并确定了脑上矢状窦血栓形成的诊断。常规进行的凝血测试显示 APTT 和 PT 延长、抗凝血酶 III 活性降低以及游离蛋白 S 浓度降低。开始LMWH治疗和抗凝血酶III替代治疗,3天后血栓相关症状显着减轻,神经状态改善。经过诱导和巩固治疗后,患者获得了完全的血液学缓解和可测量的残留病灶呈阴性。诊断六个月后,成功进行了allo-HSCT。在 4 个月的随访期间,患者保持 MRD 阴性且无血栓症状。据我们所知,我们的通讯是首次报告在成人中接受含 PEG-天冬酰胺酶方案治疗的 MPAL 患者出现此类并发症。我们建议对出现任何轻微神经系统症状的患者提高警惕,并尽早决定 MRI 研究的表现。
更新日期:2023-11-16
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