Ganapule Abhijeet, Jain Punit, Abubacker Fouzia N, Korula Anu, Abraham Aby, Mammen Joy, George Biju, Mathews Vikram, Srivastava Alok, Viswabandya Auro
aDepartment of Haematology bDepartment of Immunohaematology and Transfusion Medicine, Christian Medical College, Vellore, Tamil Nadu, India.
Blood Coagul Fibrinolysis. 2017 Mar;28(2):171-175. doi: 10.1097/MBC.0000000000000524.
Glanzmann's thrombasthenia is a rare platelet function disorder with an autosomal recessive pattern of inheritance. Achieving haemostasis in such patients who undergo surgical procedures always poses a significant challenge. Herein we report six cases of Glanzmann's thrombasthenia, who underwent nine surgeries under the cover of platelet-rich concentrates with or without recombinant activated factor VII . Of these, five were major surgeries such as thyroidectomy, laparotomy, Hartmann's procedure, reversal of Hartmann's procedure and a complete dental extraction. All five procedures were successfully done without any major bleeding. The major cost incurred in these procedures is due to the large number of blood products used and recombinant activated factor VII if used.
血小板无力症是一种罕见的血小板功能障碍，呈常染色体隐性遗传模式。对于接受外科手术的此类患者，实现止血始终是一项重大挑战。在此，我们报告6例血小板无力症患者，他们在富含血小板浓缩物（有或无重组活化因子VII）的支持下接受了9次手术。其中，5例为大手术，如甲状腺切除术、剖腹术、哈特曼手术、哈特曼手术的逆转术以及一次完整的拔牙手术。所有这5例手术均成功完成，未出现任何大出血情况。这些手术产生的主要费用是由于使用了大量血液制品以及（若使用的话）重组活化因子VII。
