Aragona M, Aragona F
Istituto di Clinica Oncologica e di Ricerca sui Tumori, Policlinico Universitario, Padiglione H, Università degli Studi, Messina.
Minerva Med. 2000 Oct;91(10):229-37.
Four cases of unexpected death due to acute myeloid leukemia have been studied: myeloblastic M1 (case I and II), promyelocytic M3 (case III), myelomonocytic M4 (case IV). Diagnosis was performed before death only in case III. Histological leukostasis was the most evident feature in all cases, particularly at the lung level, where tumor-like aspects were evident and widely diffused in case II. The last three cases were pregnant at the third trimester. Serious hemorrhagic complications were evident in all cases: CID in the former 3 cases and primary hyperfibrinolysis in the 4th, as demonstrated by the absence of intravascular cloths in many organs and especially in lung and kidney; abundant, on the contrary, in the former three cases. Leukostasis was not associated to hyper-leucocytosis in all 4 cases, but associated to normal number of peripheral white cells (below normal level in case III). This phenomenon, that disagrees with literature data on acute or chronic reactivated myeloid leukemia leukostasis, may be due to the primary confination of the immature granuloblasts in the vascular bed, because of the hemoreologic effects connected with their deficient plasticity, that is essential for normal granulocytes to flow easily in the capillary bed. In all cases death was caused by breathing and cardiocirculatory insufficiency with the associated unceasing hemorrhages.
对4例急性髓系白血病意外死亡病例进行了研究：髓母细胞性M1（病例I和II）、早幼粒细胞性M3（病例III）、粒单核细胞性M4（病例IV）。仅病例III在死亡前做出了诊断。组织学上的白细胞淤滞是所有病例中最明显的特征，尤其是在肺部，在病例II中肿瘤样表现明显且广泛分布。后3例在妊娠晚期。所有病例均有明显的严重出血并发症：前3例为消耗性凝血障碍，第4例为原发性纤溶亢进，许多器官尤其是肺和肾内无血管内血栓形成可证明这一点；相反，前3例血栓形成丰富。4例中白细胞淤滞均与白细胞增多无关，而是与外周白细胞数量正常有关（病例III低于正常水平）。这种现象与急性或慢性再激活髓系白血病白细胞淤滞的文献数据不一致，可能是由于未成熟粒细胞在血管床的原发性滞留，这是由于与其缺乏可塑性相关的血液学效应，而可塑性是正常粒细胞在毛细血管床中轻松流动所必需的。所有病例均死于呼吸和心血管循环功能不全以及相关的持续出血。
