Up to 18% of patients with acute myeloid leukemia (AML) present with a white blood cell (WBC) count of greater than 100,000/µL, a condition that is frequently referred to as hyperleukocytosis. Hyperleukocytosis has been associated with an adverse prognosis and a higher incidence of life-threatening complications such as leukostasis, disseminated intravascular coagulation (DIC), and tumor lysis syndrome (TLS). The molecular processes underlying hyperleukocytosis have not been fully elucidated yet. However, the interactions between leukemic blasts and endothelial cells leading to leukostasis and DIC as well as the processes in the bone marrow microenvironment leading to the massive entry of leukemic blasts into the peripheral blood are becoming increasingly understood. Leukemic blasts interact with endothelial cells via cell adhesion molecules such as various members of the selectin family which are upregulated via inflammatory cytokines released by leukemic blasts. Besides their role in the development of leukostasis, cell adhesion molecules have also been implicated in leukemic stem cell survival and chemotherapy resistance and can be therapeutically targeted with specific inhibitors such as plerixafor or GMI-1271 (uproleselan). However, in the absence of approved targeted therapies supportive treatment with the uric acid lowering agents allopurinol and rasburicase as well as aggressive intravenous fluid hydration for the treatment and prophylaxis of TLS, transfusion of blood products for the management of DIC, and cytoreduction with intensive chemotherapy, leukapheresis, or hydroxyurea remain the mainstay of therapy for AML patients with hyperleukocytosis.

中文翻译：

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急性髓性白血病中的高白细胞增多症和白细胞淤滞：更好地了解潜在分子病理生理学能否带来新的治疗方法？

多达 18% 的急性髓性白血病 (AML) 患者的白细胞 (WBC) 计数超过 100,000/µL，这种情况通常被称为白细胞增多症。白细胞增多症与不良预后和危及生命的并发症（如白细胞淤滞、弥散性血管内凝血 (DIC) 和肿瘤溶解综合征 (TLS)）的发生率较高有关。高白细胞增多症的分子过程尚未完全阐明。然而，白血病原始细胞和内皮细胞之间的相互作用导致白细胞淤滞和 DIC，以及导致白血病原始细胞大量进入外周血的骨髓微环境过程越来越被理解。白血病母细胞通过细胞粘附分子与内皮细胞相互作用，例如选择素家族的各种成员，这些分子通过白血病母细胞释放的炎性细胞因子上调。除了它们在白细胞淤滞发展中的作用外，细胞粘附分子还与白血病干细胞存活和化疗耐药性有关，并且可以用特定抑制剂如普乐沙福或 GMI-1271（uproleselan）进行治疗。然而，在没有批准的靶向治疗的情况下，使用降尿酸药物别嘌呤醇和拉布立酶以及积极静脉补液治疗和预防 TLS、输血治疗 DIC 和强化化疗的细胞减灭术支持治疗, 白细胞去除术,