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[高白细胞性白血病所致呼吸窘迫综合征]

[Respiratory distress syndrome due to hyperleukocytic leukemias].

作者信息

Valdovinos Mahave M C, Salvador Osuna C, del Agua C, Lanau Arilla M P, Vicente Cámara M P

机构信息

Servicio de Medicina Intensiva, Hospital Miguel Servet, Zaragoza.

出版信息

An Med Interna. 1999 Jul;16(7):359-60.

Abstract

Hyperleukocytic leukemias are a small proportion of leukemias that have white blood cell count > 100 x 10(9)/l, most of them are leukemic blast cells. These leukemias have a grave prognosis because they can develop a leukostasis syndrome which describes: the acute onset of pulmonary failure and, often, neurologic deficits and disseminated intravascular coagulation (DIC). The leukostasis is produced by the mechanical obstruction of vascular bed by blast cells, which can be induced by the spontaneous tumor lysis or as a side effect of cytotoxic drugs. So, hyperleukocytic leukemias require early and vigorous measures to decrease the white blood cell count, using leukapheresis and/or chemotherapy, before pulmonary failure exists. Then, it is possible to reverse the lesions. We report two cases of acute myeloblastic leukemia with a white blood count > 100 x 10(9)/l, that developed a respiratory distress syndrome and died. The postmortem examination has been done in one of the cases.

摘要

高白细胞性白血病是一小部分白细胞计数>100×10⁹/L的白血病,其中大多数是白血病原始细胞。这些白血病预后严重,因为它们可发展为白细胞淤滞综合征,该综合征表现为:急性呼吸衰竭,常伴有神经功能缺损和弥散性血管内凝血(DIC)。白细胞淤滞是由原始细胞对血管床的机械性阻塞所致,这可由自发性肿瘤溶解或作为细胞毒性药物的副作用诱发。因此,高白细胞性白血病需要在呼吸衰竭发生前尽早采取积极措施,通过白细胞单采术和/或化疗降低白细胞计数。这样,才有可能逆转病变。我们报告两例急性髓细胞白血病患者,其白细胞计数>100×10⁹/L,发生了呼吸窘迫综合征并死亡。其中一例进行了尸检。

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