Patient
Age (years), Gender
Inherited bleeding disorder
Lab findings
Imaging findings
Treatment for bleed
Treatment for CSVT
Outcome of bleed and CSVT
1
5, F
Mild Hemophilia A
Baseline: Factor VIII level: 28% (normal range: 50-242%) Following recombinant FVIII therapy: FVIII level: Trough: 85% Peak: 160% Following enoxaparin therapy: Anti-Xa level: 0.43 IU/mL CT: Extra axial subdural hematoma of the left parieto-occipital region CTV: nearly complete occlusion of the left sigmoid venous sinus and left upper internal jugular consistent with CSVT
IV Recombinant FVIII at 50 u/kg every 8 hours for 14 days followed by long acting FVIII prophylaxis of 65 u/kg weekly for 6 weeks
Subcutaneous Enoxaparin therapy twice daily for 6 weeks
Complete resolution of subdural hematoma and CSVT, 8 weeks after presentation
2
2, F
Mild Hemophilia A
Baseline: Factor VIII level: 24% (normal range: 50-242%) aPTT: 42 seconds (normal range: 22.9-35.8 seconds) Pre-bleed: Anti-Xa level: 0.53 IU/mL Following abdominal bleed: Hemoglobin: 5.3 g/dl Platelets: 298,000/microliter PT: 16.1 seconds aPTT: 39.8 seconds INR :1.38 Anti-Xa level: 0.38 IU/mL Following bleeding intervention: Hemoglobin: 8.6 g/dL aPTT: 29.6 seconds CTV: Left sigmoid sinus and internal jugular vein thrombosis Abdominal US and CT: Significant free fluid in the abdomen and pelvis indicative of an abdominal bleed Repeat abdominal US (1week after presentation): hematoma at the root of the mesentery
Protamine sulfate, IV vitamin K (0.3 mg/kg), 15 ml/kg of packed red blood cells
UFH followed by twice daily subcutaneous enoxaparin. Enoxaparin held following discovery of acute abdominal bleed 3 days after starting
Complete resolution of the left sigmoid sinus and IJ vein thrombosis at 4 months after presentation
3
8, M
Von Willebrand Disease, Type 3
Baseline: Factor VIII level: 10% (normal range: 50-242%) Von Willebrand factor (VWF) antigen: 8% (normal range: 22.9-35.8%) Ristocetin co-factor: 7%. (normal range: 40-130%) Following initiation of VWF concentrate treatment (trough level): VWF antigen: 32% Ristocetin cofactor: 21% FVIII level: 45% CT: Linear left parietal fracture with small subarachnoid contrecoup bleeding CTV (5 days after fall): New left epidural and left sagittal vein thrombosis, resolution of bleeding
IV VWF concentrate three times a week, discontinued following development of thrombus
None
Complete resolution of subarachnoid bleeding and CSVT, 1 month after presentation
7, M
Factor XI deficiency
aPTT: 82 seconds FXI level: 4% (normal range: 50-200%) CT: Transverse sinus thrombosis and epidural bleed CTV (48 hours after fall): Hematoma mass effect without clear intraluminal thrombosis
Watchful waiting
None
Complete resolution of epidural hematoma and CSVT 1 month after presentation