Green David
Division of Hematology/Oncology, Department of Medicine, Feinberg School of Medicine of Northwestern University, Chicago, Illinois.
Semin Thromb Hemost. 2007 Jun;33(4):427-34. doi: 10.1055/s-2007-976178.
Persons with hematologic malignancies bleed for a variety of reasons, including alterations in platelet function and numbers, clotting factor deficiencies, circulating anticoagulants, and defects in vascular integrity. The management of bleeding begins with a full characterization of the hemostatic defect. Vitamin K deficiency always should be considered and excluded by clinical history and laboratory tests. Localized bleeding is treated by packing, topical hemostatic agents, dressings, vessel ligation, laser beam coagulation, or embolization. Platelet transfusions are administered for hemorrhage secondary to severe platelet dysfunction or thrombocytopenia, but usually are not indicated if there is no bleeding, even though platelets may be as low as 10,000/microL. Bleeding due to thrombocytopenia that is refractory to random-donor platelets may respond to cross-matched compatible platelets, or to recombinant factor VIIa (rFVIIa). Fresh frozen plasma is indicated infrequently; bleeding due to coagulopathies is better managed with cryoprecipitate if fibrinogen is low, or with clotting factor concentrates appropriate for the specific clotting factors found to be deficient. rFVIIa or activated prothrombin complex concentrate usually controls hemorrhage due to autoantibodies directed against factor VIII, and acquired von Willebrand's disease may be responsive to desmopressin or intravenous gamma globulin infusion. Antifibrinolytic agents often enhance other hemostatic therapies, but should be withheld if there is genitourinary bleeding or evidence of disseminated intravascular coagulation. Finally, plasmapheresis and immunoadsorption to remove paraproteins may be helpful when other measures fail to curb bleeding.
血液系统恶性肿瘤患者出血的原因多种多样,包括血小板功能和数量改变、凝血因子缺乏、循环抗凝物质以及血管完整性缺陷。出血的处理首先要全面明确止血缺陷。应始终考虑维生素K缺乏,并通过临床病史和实验室检查予以排除。局部出血可通过填塞、局部止血剂、敷料、血管结扎、激光束凝固或栓塞治疗。对于严重血小板功能障碍或血小板减少继发的出血,可输注血小板,但即使血小板计数低至10,000/微升,若无出血通常也不主张输注。随机供者血小板难治性血小板减少所致出血,可能对交叉配型相容的血小板或重组凝血因子VIIa(rFVIIa)有反应。新鲜冰冻血浆很少使用;纤维蛋白原低时,凝血障碍所致出血用冷沉淀治疗更好,若发现特定凝血因子缺乏,则用相应的凝血因子浓缩物治疗。rFVIIa或活化的凝血酶原复合物浓缩物通常可控制因针对凝血因子VIII的自身抗体所致的出血,获得性血管性血友病可能对去氨加压素或静脉输注丙种球蛋白有反应。抗纤溶药物常可增强其他止血治疗,但如果有泌尿生殖道出血或弥散性血管内凝血证据则应停用。最后,当其他措施无法控制出血时,血浆置换和免疫吸附去除副蛋白可能会有帮助。