Medical Care
Initial treatment of hypoprothrombinemia is aimed at controlling hemorrhage. Numerous products that provide prothrombin are available. Frozen plasma contains about 1 U/mL of prothrombin. It is readily available and contains other factors that may be useful if the hypoprothrombinemia is associated with multiple factor deficiencies. Concentrates of prothrombin complex (eg, Proplex T, Konyne 80, Bebulin VH, Kcentra, Beriplex P/N, ThromboPlex, Prothrombinex-HT) are concentrated sources of prothrombin. However, these products also contain other vitamin K–dependent factors in high concentration, and use of these products at high doses has been associated with thromboembolic complications. Prothrombin-complex concentrates may contain activated clotting factors, and their use has been associated with thromboembolic complications. No pure concentrate of prothrombin factor is available.
When lupus anticoagulant-hypoprothrombinemia syndrome (LAHS) is associated with systemic lupus erythematosus, treatment with steroids, intravenous immunoglobulin, fresh-frozen plasma, or azathioprine has been successful in reducing lupus anticoagulant levels, in increasing prothrombin levels, and in controlling bleeding. However, prothrombin levels have decreased in some patients when the drugs were tapered.
In vitamin K deficiency and warfarin overdose, vitamin K is the treatment of choice unless clinically significant bleeding is present and quick correction of the coagulopathy is desired. In these cases, use either frozen plasma or a prothrombin-complex concentrate.
The question of prophylactic treatment in patients with hypoprothrombinemia is controversial. No replacement protocols are standard. Prophylaxis has been reserved for patients who have had severe, recurrent episodes of bleeding. Case reports of patients with severe hypoprothrombinemia who were treated weekly with prothrombin-complex concentrates described a reduction in hemorrhagic episodes and improved quality of life.
Surgical Care
Surgery could result in clinically significant bleeding in patients with hypoprothrombinemia. Avoid surgery whenever possible. Use concentrates of prothrombin complex in patients with factor II deficiency who require surgery. If an inhibitor is present, attempt to decrease the inhibitor titer before the surgical procedure, if possible.
Activity
Patients with hypoprothrombinemia must avoid activities and situations that could result in clinically significant trauma, especially head trauma.
Several variables determine a child's risk of bleeding. Among them are the nature and severity of the trauma, the severity of the bleeding disorder, and the speed at which treatment can be administered.
Risk of bleeding during athletic activity increases as the level of competition increases and as the likelihood of collision at top running speed increases.
The National Hemophilia Foundation has published guidelines regarding athletic activity in people with bleeding disorders.
