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成人急性白血病患者血液学急症的处理方法。

How I treat hematologic emergencies in adults with acute leukemia.

机构信息

Rambam Health Care Campus and Technion, Israel Institute of Technology, Haifa, Israel.

出版信息

Blood. 2012 Sep 6;120(10):1993-2002. doi: 10.1182/blood-2012-04-424440. Epub 2012 Jun 13.

Abstract

Acute myeloid leukemia and acute lymphoblastic leukemia remain devastating diseases. Only approximately 40% of younger and 10% of older adults are long-term survivors. Although curing the leukemia is always the most formidable challenge, complications from the disease itself and its treatment are associated with significant morbidity and mortality. Such complications, discussed herein, include tumor lysis, hyperleukocytosis, cytarabine-induced cellebellar toxicity, acute promyelocytic leukemia differentiation syndrome, thrombohemorrhagic syndrome in acute promyelocytic leukemia, L-asparaginase-associated thrombosis, leukemic meningitis, neutropenic fever, neutropenic enterocolitis, and transfussion-associated GVHD. Whereas clinical trials form the backbone for the management of acute leukemia, emergent clinical situations, predictable or not, are common and do not readily lend themselves to clinical trial evaluation. Furthermore, practice guidelines are often lacking. Not only are prospective trials impractical because of the emergent nature of the issue at hand, but clinicians are often reluctant to randomize such patients. Extensive practical experience is crucial and, even if there is no consensus, management of such emergencies should be guided by an understanding of the underlying pathophysiologic mechanisms.

摘要

急性髓系白血病和急性淋巴细胞白血病仍然是毁灭性的疾病。只有大约 40%的年轻患者和 10%的老年患者能够长期生存。尽管治愈白血病始终是最具挑战性的问题,但疾病本身及其治疗的并发症与显著的发病率和死亡率相关。本文讨论的此类并发症包括肿瘤溶解、高白细胞血症、阿糖胞苷诱导的小脑毒性、急性早幼粒细胞白血病分化综合征、急性早幼粒细胞白血病血栓性出血综合征、L-天冬酰胺酶相关血栓形成、白血病性脑膜炎、中性粒细胞减少性发热、中性粒细胞减少性结肠炎和输血相关性移植物抗宿主病。虽然临床试验是急性白血病治疗的基础,但紧急的临床情况,无论是否可预测,都很常见,且不易进行临床试验评估。此外,实践指南往往也缺乏。由于手头问题的紧急性质,前瞻性试验不仅不切实际,而且临床医生通常也不愿意对这些患者进行随机分组。广泛的实践经验至关重要,即使没有共识,此类紧急情况的管理也应基于对潜在病理生理机制的理解。

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