Case number
|
Age
|
Sex
|
Symptoms
|
Coagulation tests performed at diagnosis
|
Immunosuppressive
|
Inhibitor
|
Outcome
|
---|
PT-INR
|
APTT (sec)
|
FVIII:C (%)
|
FVIII inhibitor (BU)
|
therapy
|
recurrence
|
---|
Case 1
|
82
|
Female
|
Bullae with bleeding, Nasal hemorrhage
|
1.09
|
80
|
0.7
|
5.4
|
PSLa
|
No
|
Alive at 32 M
|
Case 2
|
56
|
Male
|
Left shoulder joint pain, Difficulty walking
|
0.96
|
68
|
1.3
|
3.2
|
PSLa, CPAb, Rituximab
|
No
|
Alive at 31 M
|
Case 3
|
58
|
Female
|
Intramuscular hematoma in left leg, Difficulty walking
|
0.97
|
94
|
1.1
|
5.9
|
PSLa
|
No
|
Alive at 18 M
|
- The reference ranges for the PT-INR, APTT, and FVIII:C are 0.8-1.2, 25-35 sec, and 60-140%, respectively
- The lower limit of detection of the Bethesda assay was 0.1 BU.
- TEG Thromboelastography. PT-INR Prothrombin time-international normalized ratio; APTT Activated partial thromboplastin time
- FVIII:C Factor VIII coagulant activity, FVIII Factor VIII, BU Bethesda units, M Months
- aPSL Prednisolone, bCPA Cyclophosphamide