Neurosurgical bleeding in platelet storage pool disorder: a case report

Area of Science:

• Hematology
• Platelet Disorders
• Inherited Bleeding Disorders

Background:

• Dense-granule deficiency (DGD) is an inherited platelet disorder characterized by the absence of dense granules, crucial for platelet activation.
• Diagnosis can be challenging as standard hemostasis tests, like PFA closure times, may appear normal despite a severe platelet function disorder.

Observation:

• A case report detailing a patient with a lifelong history of mucocutaneous bleeding and excessive hemorrhage during vestibular Schwannoma resection.
• Intraoperative bleeding resembled that seen with antiplatelet drug use, prompting further hematologic investigation.

Findings:

• The patient was diagnosed with a severe platelet function disorder, specifically dense-granule deficiency.
• Literature on managing intracranial neurosurgery in patients with inherited platelet disorders is limited.

Implications:

• Successful management involved meticulous hemostatic planning, including empiric platelet transfusions and tranexamic acid (TXA), to prevent recurrent bleeding during and after intracranial tumor surgery.
• This case highlights the importance of tailored management strategies for patients with DGD undergoing major surgical procedures, including neurosurgery and radiosurgery.