TABLE 1.

Summary of indications for treatment with desmopressin in primary hematological disorders.

Bleeding disorder	Indications	Utility
Mild congenital hemophilia A	Surgical prophylaxis and acute bleeding	Evidence to support use36–43
Mild acquired hemophilia A	Acute bleeding episodes	Evidence to support use if inhibitor titers are low42,45,48,49
Hemophilia B	Acute bleeding episodes	No Evidence to support use Factor IX not in storage pools50
FXI deficiency	Surgical prophylaxis	Systematic review with 16 case reports and no adverse bleeding51–54
FXI deficiency	Acute bleeding episodes	More research needed-case reports in patients with dual coagulopathies and improvement from vWF release54,55
Type 1 vWDa	Surgical prophylaxis and acute bleeding episode	Evidence to support use56–58
Type 2A vWDa	Acute bleeding episodes	Generally avoided due to defective multimer formation and release59
Type 2B vWD	Acute bleeding episodes	Contraindicated due to bleeding and thrombocytopenia60
Type 2M vWDa	Acute bleeding episodes	Generally avoided due to defective multimer formation and release59,61
Type 2N vWDa	Acute bleeding episodes	Case report level evidence to suggest some benefit61

^aIt is recommended that all patients undergo a desmopressin trial to determine whether this agent can be used to treat bleeding.