1
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5, F
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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
|