Coagulation disorders acquired

ACQUIRED COAGULATION DISORDERS DISSEMINATED INTRAVASCULAR COAGULATION (DIC)... 

Staudinger T, Locker GJ, Frass M. Management of acquired coagulation disorders in emergency and intensive-care medicine. Semin Thromb Hemost 1996 22 93-104. 

In a prospective study of 23 children several coagulation disorders were associated with valproate thrombocytopenia n = 2), acquired von Willebrand s disease (6), a significant fall in fibrinogen concentrations (12), and a reduction in factor XIII (4) [373 ]. Thrombelastography showed altered platelet function in 11 and prothrombin time was significantly prolonged, with many other coagulation defects. 

Several disorders of coagulation and fibrinolysis have been identified that lead to thrombosis or bleeding. These include von Willebrand disease (vWD), hemophilia, coagulation factor deficiencies, acquired/congenital inhibitors of coagulation, and antiphospolipid syndrome. 

Thrombosis is clearly die most common cause of death in the United States. About two million individuals die each year from an arterial or venous thrombosis or the consequences of these disorders. About 80% to 90% of all causes of dirombosis can now be defined with respect to cause. Of these, over 50% of all patients harbor a congenital or acquired blood coagulation protein or platelet defect... 

A (congenital or acquired) and type 1 von Willebrand disease, in which the VWF protein structure is normal but the plasma concentration is reduced (1). By contrast with conventional coagulation factor concentrates, desmopressin is cheap and is free from the risk of transmission of viral infections, which have proved such a problem in the past. It is also very useful in the treatment of carriers of hemophilia A, many of whom have significant reductions in the baseline concentration of factor VIII. By contrast, desmopressin has no effect on the concentration of factor IX, and is thus of no value in hemophilia B (Christmas disease). It is also of little value in type 2 (abnormal VWF structure) von Willebrand s disease, which accounts for about 15-20% of all cases. The administration of desmopressin to patients with type 2B von Willebrand s disease can be hazardous, as it is likely to cause thrombocytopenia (2). The use of desmopressin in bleeding disorders has been reviewed (3). Tachyphylaxis develops if desmopressin is used for prolonged periods to control bleeding disorders, because desmopressin causes release of stored factor VIII and von Willebrand factor, after which it takes time for them to accumulate again. 

Agranulocytosis, thrombocytopenia, hemolytic anemia, sideroblastic anemia (17), pure red cell aplasia (18), methemoglobinemia, and eosinophilia can occur exceptionally during isoniazid treatment (SEDA-9, 268) (19). An acquired coagulation factor XIII inhibitor developed in a patient taking isoniazid and resulted in a bleeding disorder (20). 

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