Rarely a hereditary deficiency may affect 2 or more different coagulation factors. This may be due to multiple mutations or a single mutation that impacts multiple factors.
These disorders affect siblings or other close family members.
Patients may have a severe bleeding disorder.
V and VIII
PT and aPTT
VIII and IX
II, VII, IX, X
proteins S and C
VII and VIII
VIII, IX and XI
IX and XI
VIII and X
• All of the deficient elements in Type III are vitamin K dependent.
• 5 of the 7 types involve Factor VIII.
• 5 of the 7 types involve 2 factors, 1 type involves 3 and 1 type involves 4.
Differential diagnosis - acquired disorder affecting multiple factors:
(1) vitamin K deficiency
(2) consumptive coagulopathy (DIC, other)
(3) mixed single hereditary factor deficiency and acquired deficiency
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Specialty: Hematology Oncology, Clinical Laboratory