Alpha2-Plasmin Inhibitor Deficiency Follow-up

Prolonged therapy with FFP or PLAS+SD and antifibrinolytics may be needed in those who have alpha 2-plasmin inhibitor deficiency (alpha 2-PI deficiency, a2-PI deficiency), 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 alpha 2-plasmin inhibitor deficiency (alpha 2-PI deficiency, a2-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 alpha 2-plasmin inhibitor deficiency (alpha 2-PI deficiency, a2-PI deficiency).

Long-term maintenance therapy with oral antifibrinolytic agents has reduced the incidence of bleeding complications in alpha 2-plasmin inhibitor deficiency (alpha 2-PI deficiency, a2-PI deficiency). For dosing, please see the Medication section.

Avoidance of antiplatelet drugs is essential because these agents increase bleeding risk.

A patient with persistent bleeding in the postoperative period resulting from alpha 2-plasmin inhibitor deficiency (alpha 2-PI deficiency, a2-PI deficiency) may require transfer to a tertiary medical center if specialists with expertise are not available at the local community hospital.

Avoidance of trauma and the use of NSAIDs can minimize the frequency of bleeding complications.

Prevention is not feasible for the genetic defect that causes alpha 2-plasmin inhibitor deficiency (alpha 2-PI deficiency, a2-PI deficiency). Prenatal testing of a known defect may be attempted in a family in which members experience severe bleeding.

Immunization against HAV and HBV is useful in patients who require administration of plasma products. Although reports of blood-borne 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 hepatic failure. Therefore, immunizing patients against any form of hepatitis for which a vaccine is available is wise.^{[12, 16]} HAV vaccination conforms with recommendations of the National Hemophilia Foundation for patients receiving any kind of blood products on a recurrent basis.

Complications of alpha 2-plasmin inhibitor deficiency (alpha 2-PI deficiency, a2-PI deficiency) relate to the site at which excessive bleeding develops. Bleeding is aggravated by concomitant use of drugs that induce additional hemostatic dysfunction, such as acetylsalicylic acid (ASA) or other NSAIDs. Other complications include the following^{[12, 13, 16]} :

• Infection by HIV (including type 2 and HIV group O), HHV-8, acquired immunodeficiency syndrome (AIDS), hepatitis (hepatitis A-E viruses, hepatitis GB virus C, hepatitis G virus), parvovirus infection, and SEN viruses
• CJD and nvCJD resulting from prions: One review raised concerns about the transmission of CJD or its variant form (vCJD) from blood products.^[17] The FDA's Transmissible Spongiform Encephalopathies Advisory Committee has proposed excluding donors who have resided or traveled in Europe for more than 5 years starting in 1980 or donors who have resided in the United Kingdom for more than 3 months. The availability of a new test for vCJD is anticipated.
• Other complications may result from viral illnesses transmitted by human plasma.

People who are homozygous for alpha 2-plasmin inhibitor deficiency (alpha 2-PI deficiency, a2-PI deficiency) have a severe bleeding disorder, but if appropriate treatment is received, long-term survival is possible. The frequent need for plasma transfusions exposes the patient to the risks of virally transmitted illnesses, including HIV, hepatitis, parvovirus, TTV, nvCJD due to prions, and other pathogens.

People who are heterozygous for alpha 2-plasmin inhibitor deficiency (alpha 2-PI deficiency, a2-PI deficiency) have variable bleeding, generally mild or none. Cautious treatment is warranted to protect the patient from unneeded surgery with subsequent bleeding.

Educate patients with alpha 2-plasmin inhibitor deficiency (alpha 2-PI deficiency, a2-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 the eMedicine topic 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.