Transfusion Medicine Division, Department of Pathology and Laboratory Medicine, Henry Ford Hospital, Detroit, Michigan, USA.
Division of Hematology and Oncology, Department of Internal Medicine, Henry Ford Hospital, Detroit, Michigan, USA.
Transfusion. 2021 Nov;61(11):3277-3280. doi: 10.1111/trf.16682. Epub 2021 Sep 26.
Essential thrombocythemia (ET) is associated with increased risk of bleeding secondary to acquired von Willebrand syndrome (AVWS). Bleeding in ET requires urgent platelet reduction by cytoreductive therapy such as hydroxyurea or thrombocytapheresis. We report on the efficacy and safety of thrombocytapheresis in managing AVWS in a patient with ET and multivisceral transplantation.
The patient was a 51-year-old female who underwent multivisceral transplantation. Her postoperative course was complicated by bleeding from oral cavity, IV lines, gastrointestinal and upper respiratory tracts as well as vaginal bleeding, which coincided with ET flare with a platelet count of 1512 × 10 /L. Coagulation studies including von Willebrand factor (vWF) antigen and activity, vWF propeptide antigen, and vWF multimer analysis were consistent with AVWS. Hydroxyurea was initiated. However, due to major bleeding, rapidly increasing platelet count, and uncertainty of response to hydroxyurea being given through the enteral tube, thrombocytapheresis was initiated for rapid platelet reduction. The patient tolerated the procedure well. Platelet count was reduced from 1636 × 10 /L to 275 × 10 /L with rapid cessation of bleeding. The patient's condition stabilized over the next few days; however, bleeding recurred with increasing platelet count, which required a second thrombocytapheresis 8 days after the first one. The patient was maintained on hydroxyurea 500 mg twice/day. At 11-month follow-up, she had a normal platelet count and no recurrence of bleeding.
Thrombocytapheresis is safe and efficient in managing postoperative bleeding due to ET/AVWS in solid organ transplant patients. The procedure can be an adjunct to bridging therapy before response to hydroxyurea is achieved.
特发性血小板增多症（ET）与获得性血管性血友病（AVWS）相关的出血风险增加有关。ET 引起的出血需要通过细胞减少治疗（如羟基脲或血小板分离术）迅速降低血小板计数。我们报告了血小板分离术在管理 ET 伴多器官移植患者的 AVWS 中的疗效和安全性。
患者为 51 岁女性，行多器官移植术。术后发生口腔、静脉输液管、胃肠道和上呼吸道以及阴道出血，同时伴有 ET 发作，血小板计数为 1512×10 /L。凝血研究包括血管性血友病因子（vWF）抗原和活性、vWF 前肽抗原和 vWF 多聚体分析均符合 AVWS。开始使用羟基脲。然而，由于大量出血、血小板计数迅速增加以及不确定通过肠内管给予羟基脲的反应，因此开始进行血小板分离术以迅速降低血小板计数。患者耐受良好。血小板计数从 1636×10 /L 降至 275×10 /L，出血迅速停止。接下来的几天，患者病情稳定；然而，随着血小板计数的增加，出血再次发生，需要在第一次血小板分离术 8 天后进行第二次。患者每天服用 500mg 羟基脲，分两次服用。在 11 个月的随访中，她的血小板计数正常，没有再次出血。
血小板分离术在管理实体器官移植患者 ET/AVWS 术后出血方面是安全有效的。该程序可作为羟基脲起效前桥接治疗的辅助手段。
