A 42-year-old man, farmer, with no relevant background, consulted several times for 3 months of headache evolution with a feeling of heaviness and dizziness without response to common analgesics.
The patient developed ataxia and right atrium.
One week prior to admission, the patient presented with right endophoria, diplopia, and bedridden vomiting.
A simple CT scan of the skull showed a mass in the posterior fossa and subarachnoid hemorrhage, so the patient was admitted to a tertiary hospital.
The patient was admitted to the emergency department, conscious and oriented, with headache, diplopia and inability to walk due to verdict.
Her physical examination showed only right endophoria, gait disturbance and right atrium.
A simple contrast-enhanced MRI showed an image compatible with hemangioblastoma.
On admission tests, a platelet count of 10,000/mL was observed, with no other alterations in red or white series, which led to a surgical difference and management studies began.
The autoimmune profile (ANAS, ENAS, Coombs, immunoglobulins, lupus anticoagulant and anticardiolipin) infectious (HIV, hepatitis B and C), coagulation tests (PT, PTT) were normal.
The presence of suggestive symptoms of coagulopathy was not considered.
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The patient presented neurological deterioration so it was decided to carry out emergency surgery prior platelet transfusion support for resection of the mass in CNS.
Through right occipital trepanation, an external ventricular derivation catheter was passed to the ipsilateral lateral ventricle, obtaining pressure CSF.
A tumor was identified in the posterior fossa, draining a tumor cyst appearance of hemangioblastoma, highly vascularized, with total macroscopic resection.
The pathology report was compatible with hemangioblastoma.
Given the association with Von Hippel Lindau syndrome, additionally, a search for other abdominal tumors with ultrasound was considered negative.
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It is linked to a critical care unit with excellent neurological response without resolution of thrombocytopenia – between 5,000 and 169,000/mL – often requiring transfusion support seeking counts > 100,000/mL.
Tomographic follow-up (Day 2 and 7 post-operative) revealed subdural hematoma with signs of herniation in resolution.
She underwent a bone marrow study (myogram, biopsy, karyotype) all normal and with suspected purpura thrombotic thrombocytopenicum 2 days (6 mg), glucocorticoids were indicated (h/6 4 mg dexamethasone).
In view of the risk of intracerebral bleeding, it was decided to apply IV immunoglobulin 1 g/kg for two doses administered one week plus prednisone at 1 mg/kg/day without clinical improvement, which was scheduled for neurocerebral surgery 20.
The procedure develops without complications and presents platelet count of 165,000/mL in the immediate postoperative period, with histologically normal spleen.
Forty-eight hours later, the patient presented new neurological deterioration requiring readmission to the ICU.
Platelet count 126,000/mL, thromboelastography with high MA suggestive of platelet hyperaggregability and CT scan of the skull with subacute right subdural hematoma with mass effect and tendency to emergency cerebral ischemia all
Subsequent CT showed no significant changes.
With these findings, sedation is withdrawn from the patient, 2 days later he presents with hypernatremia and fever, after two assessments he declares himself dead.
