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心血管崩溃和 McConnell 征作为白细胞淤滞的早期表现。

Cardiovascular collapse and McConnell's sign as early manifestations of leucostasis.

机构信息

Critical Care Medicine, Stanford Health Care, Stanford, California, USA

Emergency Medicine, Stanford Health Care, Stanford, California, USA.

出版信息

BMJ Case Rep. 2024 Feb 17;17(2):e257330. doi: 10.1136/bcr-2023-257330.

Abstract

A man in his late 70s with chronic myelomonocytic leukaemia presented for evaluation of acute leukaemic transformation and initiation of cytoreductive therapy after being found to have asymptomatic hyperleucocytosis. Within 24 hours, the patient developed vasopressor-refractory shock, severe lactic acidosis and multiorgan failure. Serial echocardiographic assessments revealed interval enlargement of the right ventricle with development of the McConnell's sign, and abdominal CT showed diffuse bowel wall thickening, likely due to ischaemia. CT angiography excluded pulmonary embolism or occlusion of intra-abdominal arteries. Despite aggressive care, the patient died from cardiovascular collapse within 8 hours of the onset of hypotension. An autopsy revealed extensive infiltration of early myeloid cells in pulmonary, myocardial, hepatic and intestinal microvasculature. This case illustrates different mechanisms by which leucostasis causes acute cardiovascular collapse and stresses the emergent nature of this diagnosis.

摘要

一位 70 多岁的男性,患有慢性髓单核细胞白血病,因无症状性白细胞增多而就诊,随后发现白血病转化,开始进行细胞减少治疗。在 24 小时内,患者出现升压药抵抗性休克、严重乳酸性酸中毒和多器官衰竭。连续的心超评估显示右心室间隔扩大,出现 McConnell 征,腹部 CT 显示弥漫性肠壁增厚,可能是由于缺血所致。CT 血管造影排除了肺栓塞或腹内动脉闭塞。尽管进行了积极治疗,但患者从低血压发作到心血管崩溃仅 8 小时内死亡。尸检显示早期髓样细胞在肺、心肌、肝和肠道微血管中广泛浸润。该病例说明了白细胞淤滞导致急性心血管崩溃的不同机制,并强调了这种诊断的紧急性质。

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本文引用的文献

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How I treat hyperleukocytosis in acute myeloid leukemia.我如何治疗急性髓系白血病中的白细胞增多症。
Blood. 2015 May 21;125(21):3246-52. doi: 10.1182/blood-2014-10-551507. Epub 2015 Mar 16.

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