Factor XIII deficiency

XIII hver, megakaryocytes 95-120 1-2 1-2 factor XIII deficiency, <0.5 AR ... 

XIII Factor XIII deficiency 5% 6 days FFP Cryoprecipitate... 

Factor XIII deficiency. There is no expected effect on any of the clotting time measurements. The susceptibility to plasmin suggests an unstabilized clot. The solubility in urea is diagnostic for un-cross-linked fibrin. 

Chemoprotective agent for cisplatin and cyclophosphamide Hypercalcemia of malignancy Hemophilia A and B Congenital factor XIII deficiency Lennox-Gastaut syndrome Colonic adenomatous polyps Treatment of hepatitis B Corneal ulcers Myelodysplastic syndrome Diagnosing allergy to fire ant Non-Hodgkin s lymphoma Hyperbilirubinemia in newborns unresponsive to phototherapy... 

A (very rare) genetic deficiency in the production of factor XIII also results in impaired clotting efficacy in affected persons. In this case, covalent links that normally characterize transformation of a soft clot into a hard clot are not formed. Factor XIII preparations, partially purified from human blood, are used to treat individuals with this condition to date, no recombinant version of the product has been commercialized. 

Congenital deficiency of Factor XIII is inherited as an autosomal recessive trait and is frequendy recognized at birth because of delayed persistent hemorrhage from the umbilicus. In Factor XHI-deficient people wound healing is defective and wound dehiscence is common. 

Factor deficiencies include disorders of fibrinogen such as afibrinogenemia and dysfibrinogenemias, prothrombin deficiency, factor V VII, X, XI, XII, and XIII deficiency, prekallikrein and high-molecular-weight kininogen deficiency, combined factor deficiencies, a2 anti-plasmin deficiency, a] antitrypsin Pittsburgh, and protein Z deficiency. 

A deficiency in factor XIII of autoimmune origin associated with the administration of isoniazid has been described [50]. 

Recessively inherited coagulation disorders (RICDs) refer to relatively rare deficiencies in factor II, V, VII, and X-XIII resulting in either decreased clotting factor production or production of a dysfunctional molecule with reduced activity.19 The clinical severity of bleeding varies and generally is poorly correlated with the factor blood levels. Table 64-6 illustrates these clotting factor deficiencies and some of their characteristics. 

Fresh frozen plasma contains the components of the coagulation system and is indicated for the replacement of deficient coagulation factors II, V, VII, X, XI, and XIII. Factor VIII and IX deficiencies are treated with specific factor concentrates. Fresh frozen plasma is also used for the rapid reversal of warfarin anticoagulation and in the treatment of disseminated intravascular coagulation. Thrombotic thrombocytopenic purpura is treated by means of therapeutic plasma exchange with fresh frozen plasma as the replacement fluid. Cryo-precipitate, which contains factor VIII, von Willebrand s factor, and fibrinogen, is indicated for the treatment of von Willebrand s disease that does not respond to desmopressin acetate, and for fibrinogen replacement (see Chap. 100). 

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