Kalk E, Goede A, Rose P
Departments of Haematology and Surgery, Warwick Hospital, Warwick, UK.
Clin Lab Haematol. 2003 Aug;25(4):267-70. doi: 10.1046/j.1365-2257.2003.00526.x.
Localized large vessel thrombosis in acute leukaemia is rare, haemorrhagic complications being more common.
We present a patient with acute promyelocytic leukaemia (APL) presenting with an acutely ischaemic lower limb. Large vessel thrombosis is a rare presentation of APL. We reviewed the literature on the coagulopathy of APL and discuss the pathology and current treatment options.
Disordered haemostasis is typical of acute promyelocytic leukaemia (FAB M3) and relates to the intrinsic properties of the blast cells as well as thrombocytopenia from bone marrow involvement. Expression of procoagulants, stimulation of cytokines and alterations in endothelial cell anticoagulant properties initiate a disseminated intravascular coagulation (DIC) resulting in the typical clinical and laboratory findings in APL. The promyelocytes are characterized by the balanced reciprocal translocation between chromosomes 15 and 17. All-trans-retinoic acid (ATRA) induces differentiation in these cells, revolutionizing the treatment of APL.
Unexpected limb ischaemia in a young, apparently healthy patient might be the presenting symptom of an underlying haematological disorder such as APL. A thorough haematological investigation should be performed prior to contemplating surgery. New treatment strategies based on knowledge of the molecular biology of APL has improved the prognosis of patients suffering from APL.
急性白血病中局部大血管血栓形成较为罕见，出血并发症更为常见。
我们报告一例急性早幼粒细胞白血病（APL）患者，其表现为急性下肢缺血。大血管血栓形成是APL的一种罕见表现。我们回顾了关于APL凝血异常的文献，并讨论其病理及当前的治疗选择。
止血功能紊乱是急性早幼粒细胞白血病（FAB M3型）的典型特征，与原始细胞的内在特性以及骨髓受累导致的血小板减少有关。促凝剂的表达、细胞因子的刺激以及内皮细胞抗凝特性的改变引发弥散性血管内凝血（DIC），导致APL典型的临床和实验室检查结果。早幼粒细胞的特征是15号和17号染色体之间的平衡易位。全反式维甲酸（ATRA）诱导这些细胞分化，彻底改变了APL的治疗方式。
年轻、看似健康的患者出现意外的肢体缺血可能是潜在血液系统疾病如APL的首发症状。在考虑手术之前应进行全面的血液学检查。基于APL分子生物学知识的新治疗策略改善了APL患者的预后。
