Abstract：
Dense-granule deficiency (DGD) is an inherited platelet disorder due to the absence of dense granules essential for activation of platelets in the event of vascular injury. Decreased platelet dense granules can be detected by electron microscopy, while other tests of hemostasis, including platelet function analyzer (PFA®) closure times, may be normal. The present case report describes a patient with a lifelong history of mucocutaneous bleeding and excessive hemorrhage with resection of vestibular Schwannoma. After hemostasis was obtained the case was aborted and the neurosurgeon noted bleeding resembled as if patient was on an antiplatelet drug. Subsequent hematologic workup revealed a severe platelet function disorder. There is a paucity of literature on management of intracranial neurosurgery in patients with inherited platelet disorders. Patients undergoing major surgical procedures often receive tranexamic acid (TXA), desmopressin, and/or human-leukocyte antigen (HLA)-matched platelet transfusions. We review the clinical management of intracranial tumor surgery, as well as Cyberknife radiosurgery, in our patient with DGD. After diagnosis was known, thoughtful hemostatic planning with empiric platelet transfusions and TXA prevented recurrent bleeding.
Platelet disorders that affect platelet function require high index of suspicion and special laboratory evaluation for diagnosis. We provide a case report of storage pool deficiency with management of bleeding in Schwannoma resection and radiosurgery. This case report adds to the limited literature to guide treatment of platelet function disorder in neurosurgery.
Platelet disorders that affect platelet function require high index of suspicion and special laboratory evaluation for diagnosis. We provide a case report of storage pool deficiency with management of bleeding in Schwannoma resection and radiosurgery. This case report adds to the limited literature to guide treatment of platelet function disorder in neurosurgery.
摘要：
致密颗粒缺乏症（DGD）是一种遗传性血小板疾病，因为在血管损伤的情况下缺乏对活化血小板至关重要的致密颗粒。减少的血小板致密颗粒可以通过电子显微镜检测，而其他止血测试，包括血小板功能分析仪（PFA®）关闭时间，可能是正常的。本病例报告描述了一名患有皮肤粘膜出血和出血过多的终生病史，并切除前庭神经鞘瘤的患者。止血后，该病例中止，神经外科医生注意到出血类似于患者服用抗血小板药物。随后的血液学检查显示严重的血小板功能紊乱。关于遗传性血小板疾病患者的颅内神经外科治疗的文献很少。接受大型外科手术的患者通常会接受氨甲环酸（TXA），去氨加压素,和/或人白细胞抗原（HLA）匹配的血小板输注。我们回顾了颅内肿瘤手术的临床处理，以及射波刀放射外科,我们的DGD患者.确诊后,经过深思熟虑的止血计划，经验性血小板输注和TXA可预防复发性出血。
影响血小板功能的血小板疾病需要高度怀疑和特殊的实验室评估来诊断。我们提供了一例神经鞘瘤切除术和放射外科手术中存储池不足并处理出血的病例报告。该病例报告增加了有限的文献，以指导神经外科中血小板功能紊乱的治疗。
影响血小板功能的血小板疾病需要高度怀疑和特殊的实验室评估来诊断。我们提供了一例神经鞘瘤切除术和放射外科手术中存储池不足并处理出血的病例报告。该病例报告增加了有限的文献，以指导神经外科中血小板功能紊乱的治疗。
