Inpatient & Outpatient Medications
Prolonged therapy with fresh frozen plasma (FFP) or solvent/detergent-treated plasma (SDP) or antifibrinolytic agents may be needed in patients with alpha2–plasmin inhibitor (alpha2-PI), depending on the clinical circumstance.
Continuation of oral antifibrinolytic therapy on an outpatient basis is warranted, particularly if the drug was effective in controlling bleeding, as in persons with hemophilia following oral surgical procedures. Only a brief period of therapy is recommended for acquired disorders of alpha2-PI deficiency. Monitor patients closely, and determine the appropriate duration of therapy by clinical observation of the patient.
For prophylactic care, long-term oral therapy with antifibrinolytics has successfully reduced the incidence of bleeding in patients with inherited alpha2-PI deficiency.
Avoidance of antiplatelet drugs is essential because these agents increase bleeding risk.
Deterrence/Prevention
To minimize the frequency of bleeding complications, patients with alpha2–plasmin inhibitor (alpha2-PI) deficiency should avoid contact sports and other activities with a significant risk of trauma, and should not take nonsteroidal anti-inflammatory drugs (NSAIDs).
Prevention is not feasible for the genetic defect that causes alpha2-PI deficiency. Prenatal testing of a known defect may be attempted in a family whose members experience severe bleeding.
Immunization against hepatitis A and hepatitis B is useful in patients who require administration of plasma products. Although reports of blood-borne hepatitis A virus (HAV) infection resulting from tainted donations are sporadic only, the superimposition of acute HAV infection on chronic hepatitis (which may exist in patients with repeated exposure to blood products) clearly puts patients at higher risk of liver failure. Therefore, immunizing patients against any form of hepatitis for which a vaccine is available is wise. HAV vaccination conforms with recommendations of the National Hemophilia Foundation for patients receiving any kind of blood products on a recurrent basis.
Prognosis
Persons who are homozygous for alpha2–plasmin inhibitor (alpha2-PI) deficiency have a severe bleeding disorder, but if appropriate treatment is received, long-term survival is possible. However, the frequent need for plasma transfusions exposes the patient to the risks of transfusion-transmitted diseases.
Persons who are heterozygous for alpha 2-PI deficiency have variable bleeding, generally mild or none. Cautious treatment is warranted to protect the patient from unneeded surgery with subsequent bleeding.
Patient Education
Educate patients with alpha2–plasmin inhibitor (alpha2-PI) deficiency on a continuing basis, and encourage them to seek appropriate information, which will strengthen their ability to deal with this inherited disorder.
Discuss the potential thrombotic risk of antifibrinolytic agents. This author's practice is to request the pharmacist to provide the patient and family with package inserts for special drugs.
If plasma is used, discuss the potential risks of blood product use. No source of plasma is 100% safe. Moreover, the risks of transmission of viral illnesses vary according to the country of source of the plasma (see Factor VIII for a discussion of these issues, as well as Transfusion-Transmitted Diseases).
The National Hemophilia Foundation provides information and support for patients with bleeding disorders and their families.
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The role of alpha2-plasmin inhibitor (alpha2-antiplasmin) in fibrinolysis.
