Lizurej Wojciech, Mazurkiewicz Łukasz, Kowalski Michał, Szydłowska Sylwia, Wyrzykowski Michał, Lewandowski Krzysztof
Department of Hematology and Bone Marrow Transplantation, Poznan University of Medical Sciences, Poznan, Poland.
Department of Diagnostic Imaging, Poznan University of Medical Sciences, Poznan, Poland.
Thromb J. 2023 Nov 16;21(1):117. doi: 10.1186/s12959-023-00561-9.
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.
与成人其他急性白血病相比，混合表型急性白血病（MPAL）的总生存期更差。缺乏明确的治疗指南使得治疗具有挑战性。类似急性淋巴细胞白血病的诱导和巩固治疗，随后进行异基因造血干细胞移植（allo-HSCT）是首选的一线治疗方法。我们报告一例36岁女性，诊断为伴有KMT2A重排的MPAL（EGIL骨髓/ B型），采用PALG-ALL-7方案（包括聚乙二醇化天冬酰胺酶）进行治疗。在诱导治疗开始后的第25天，观察到肢体麻木和头晕。因此，进行了影像学检查（CT和MRI），确诊为大脑上矢状窦血栓形成。常规进行的凝血检查显示活化部分凝血活酶时间（APTT）和凝血酶原时间（PT）延长，抗凝血酶III活性降低，游离蛋白S浓度降低。开始使用低分子肝素（LMWH）治疗和抗凝血酶III替代治疗，3天后血栓相关症状显著减轻，神经状态改善。诱导和巩固治疗后，患者获得完全血液学缓解，可测量残留病为阴性。诊断后6个月，成功进行了异基因造血干细胞移植。在4个月的随访期间，患者仍保持残留病阴性且无血栓形成症状。据我们所知，我们的交流是首例关于在接受含聚乙二醇化天冬酰胺酶方案治疗的成人MPAL患者中出现这种并发症的报告。我们建议对出现任何轻微神经症状的患者提高警惕，并尽早决定进行MRI检查。
