| Autor: |
Pierre C; Neurosurgery, Swedish Neuroscience Institute, Seattle, WA, USA., Alcorn KW; Transfusion, Bloodworks Northwest, Seattle, WA, USA., Chen D; Division of Hematopathology, Mayo Clinic, Rochester, MN, USA., Fesler J; Hematology, Swedish Center for Blood Disorders and Stem Cell Transplantation, Seattle, WA, USA., Landis D; Radiology, Swedish Radiosurgery Center, Seattle, WA, USA., Litvack ZN; Neurosurgery, Swedish Neuroscience Institute, Seattle, WA, USA., Konkle BA; Division of Hematology, and Washington Center for Bleeding Disorders, University of Washington, Seattle, WA, USA., Hegerova L; Division of Hematology, and Washington Center for Bleeding Disorders, University of Washington, Seattle, WA, USA. |
| Abstrakt: |
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. |
