RT Book, Section A1 Lichtman, Marshall A. A1 Kaushansky, Kenneth A1 Prchal, Josef T. A1 Levi, Marcel M. A1 Burns, Linda J. A1 Linch, David C. SR Print(0) ID 1189336574 T1 Inherited Deficiencies of Coagulation Factors II, V, VII, X, XI, and XIII and Combined Deficiencies of Factor V and VIII and of Vitamin K–Dependent Factors T2 Williams Manual of Hematology, 10e YR 2022 FD 2022 PB McGraw-Hill Education PP New York, NY SN 9781264269204 LK hemonc.mhmedical.com/content.aspx?aid=1189336574 RD 2024/03/29 AB Inherited deficiencies of coagulation factors other than factor VIII (hemophilia A) and factor IX (hemophilia B) are rare bleeding disorders that occur in most populations.Patients are usually homozygotes or compound heterozygotes.Factor XI and factor VII deficiency are rare but occur relatively frequently compared to other factor deficiencies (apart from hemophilia A and B), whereas the other deficiencies are very rare (Table 82–1).The severity of the bleeding disorder usually relates to the severity of the factor deficiency.All may be caused by decreased synthesis of a specific coagulation factor, by synthesis of a dysfunctional form of the coagulation factor, or both.Inherited deficiency of a coagulation factor does not protect patients from thrombosis.Rare bleeding disorders are often caused by mutations unique for each kindred and scattered throughout the genes.The molecular diagnosis is based on the mutation search in the genes encoding the corresponding coagulation factor (Table 82–2).