Key concepts

Intravenous factor replacement with recombinant or plasma-derived products to treat or prevent bleeding is the primary treatment of hemophilia.

Type 1 patients unresponsive to desmopressin, patients with types 2 and 3 von Willebrand's disease (vWD), and major surgery patients require replacement therapy with plasma-derived intermediate-and high-purity factor VIII, virus-inactivated factor VIII concentrates containing von Willebrand's factor (vWF).

The primary treatment of recessively inherited coagulation disorders (RICDs) is single-donor fresh-frozen plasma (FFP) that contains all coagulation factors.

The cornerstone of the management of disseminated intravascular coagulation (DIC) is aggressive treatment of the underlying primary illness. Supportive measures may be used as necessary; however, owing to the heterogeneity of the DIC etiology, treatment should be guided by predominant symptoms (bleeding or clotting).

O The treatment of immune thrombocytopenic purpura (ITP) is determined by the symptom severity. In some cases, no therapy is needed.

The present standard of treatment for thrombotic thrombocytopenic purpura (TTP) is urgent plasma exchange (PEX). If PEX is unavailable, treatment with plasma infusion and glucocorticoids is indicated until PEX is available.

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