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慢性粒单核细胞白血病中轴内出血性病变上方的慢性硬膜下积液:病例报告及文献复习

Chronic subdural collection overlying an intra-axial hemorrhagic lesion in chronic myelomonocytic leukemia: special report and review of the literature.

作者信息

Bernat Anne-Laure, Priola Stefano Maria, Elsawy Ahmad, Farrash Faisal, Taslimi Shervin, Gentili Fred

机构信息

Department of Neurosurgery, Toronto Western Hospital, University of Toronto, Toronto, Canada.

出版信息

Expert Rev Neurother. 2018 May;18(5):371-377. doi: 10.1080/14737175.2018.1464391. Epub 2018 Apr 19.

Abstract

: Chronic myelomonocytic leukaemia (CMML) is a clonal hematopoietic stem cell disorder characterized by the presence of an absolute monocytosis in the peripheral blood (>1 x 10/L) and the presence of myelodysplastic and myeloproliferative features in the bone marrow. Involvement of the central nervous system (CNS) is uncommon in CMML.: Herein described is a case report of a CMML patient who presents with symptomatic chronic subdural collection overlying a haemorrhagic brain lesion, along with diffuse dural infiltration, after two cycles of azacytidine. Surgical intervention was performed to alleviate the mass effect on the brain, and obtain a tissue sample for diagnosis. Histopathological report confirmed brain infiltration with myeloid leukemic cells.: Despite its rarity, cerebral dissemination should be considered even in patients with CMML. A multidisciplinary approach, lead by a hematologist, is mandatory in order to correct the underlying haematological disorder, with specific attention to the coagulation profile. Surgical intervention is necessary for symptomatic patients, and should be performed once an improvement of clinical conditions has been achieved. Despite appropriate surgical and medical therapy, the prognosis remains poor with high risk of perioperative complications, such as rebleeding, and progressive systemic involvement.

摘要

慢性粒单核细胞白血病(CMML)是一种克隆性造血干细胞疾病,其特征为外周血中存在绝对单核细胞增多(>1×10⁹/L),且骨髓中存在骨髓发育异常和骨髓增殖特征。中枢神经系统(CNS)受累在CMML中并不常见。本文报道了1例CMML患者,在接受两个周期阿扎胞苷治疗后,出现有症状的慢性硬膜下积液,其下方为出血性脑病变,并伴有弥漫性硬脑膜浸润。进行了手术干预以减轻对脑的占位效应,并获取组织样本进行诊断。组织病理学报告证实脑内有髓系白血病细胞浸润。尽管罕见,但即使是CMML患者也应考虑脑转移。必须采取以血液科医生为主导的多学科方法来纠正潜在的血液系统疾病,尤其要关注凝血状况。有症状的患者需要进行手术干预,且应在临床状况有所改善后进行。尽管采取了适当的手术和药物治疗,但预后仍然很差,围手术期并发症(如再出血)和进行性全身受累的风险很高。

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