Clinical features of recessively inherited coagulation disorders
Deficient factor . | Main clinical symptoms* . | Hemostatic levels . | Plasma half-life . |
|---|---|---|---|
| Fibrinogen | Umbilical cord, joint, and mucosal tract bleeding; recurrent miscarriages, rarely thrombosis | 50 mg/dL | 2-4 d |
| Prothrombin | Umbilical cord, joint, and mucosal tract bleeding | 20%-30% | 3-4 d |
| V | Mucosal tract bleeding | 15%-20% | 36 h |
| VII | Mucosal tract, joint, and muscle bleeding | 15%-20% | 4-6 h |
| X | Umbilical cord, joint, and muscle bleeding | 15%-20% | 40-60 h |
| XI | Posttraumatic bleeding | 15%-20% | 40-70 h |
| XIII | Umbilical cord, intracranial, and joint bleeding; recurrent miscarriages, impaired wound healing | 2%-5% | 11-14 d |
| V + VIII | Mucosal tract bleeding | 15%-20% | 36 h for factor V and 10-14 h for factor VIII |
| Vitamin K—dependent, multiple deficiency | Umbilical cord and intracranial bleeding | 15%-20% | See corresponding factors |
Deficient factor . | Main clinical symptoms* . | Hemostatic levels . | Plasma half-life . |
|---|---|---|---|
| Fibrinogen | Umbilical cord, joint, and mucosal tract bleeding; recurrent miscarriages, rarely thrombosis | 50 mg/dL | 2-4 d |
| Prothrombin | Umbilical cord, joint, and mucosal tract bleeding | 20%-30% | 3-4 d |
| V | Mucosal tract bleeding | 15%-20% | 36 h |
| VII | Mucosal tract, joint, and muscle bleeding | 15%-20% | 4-6 h |
| X | Umbilical cord, joint, and muscle bleeding | 15%-20% | 40-60 h |
| XI | Posttraumatic bleeding | 15%-20% | 40-70 h |
| XIII | Umbilical cord, intracranial, and joint bleeding; recurrent miscarriages, impaired wound healing | 2%-5% | 11-14 d |
| V + VIII | Mucosal tract bleeding | 15%-20% | 36 h for factor V and 10-14 h for factor VIII |
| Vitamin K—dependent, multiple deficiency | Umbilical cord and intracranial bleeding | 15%-20% | See corresponding factors |
Excessive bleeding after invasive procedures carried out without replacement therapy is a symptom common to all RICDs.