Alpha2-Plasmin Inhibitor Deficiency Workup
- Author: Olga Kozyreva, MD; Chief Editor: Perumal Thiagarajan, MD more...
Appropriate testing methodology (ie, functional vs antigenic, biologic vs chromogenic substrate assays) is an important consideration during the workup of patients with alpha 2-plasmin inhibitor deficiency (alpha 2-PI deficiency, a2-PI deficiency).
Testing blood during acute bleeding events may show reduced levels of factors, which may rise to reference range levels when patients are stable. Therefore, testing patients repeatedly when they are in a stable state is important to confirm the diagnosis.
The functional and antigenic levels of alpha 2-plasmin inhibitor (alpha 2-PI, a2-PI) are reduced to a similar extent in most patients with severe alpha 2-plasmin inhibitor deficiency. Patients with a dysfunctional molecule who have reduced functional activity with reference antigen values for the inhibitor have also been described.
Initial routine workup should include testing, as follows:
Activated partial thromboplastin time (aPTT)
Prothrombin time (PT)
Thrombin-coagulable fibrinogen levels
Euglobulin lysis time
Whole blood clot lysis time
Platelet counts and bleeding times (only if patient has not had antiplatelet drugs in the preceding 5-7 d)
Screening for factor XIII deficiency using a urea or monochloroacetic acid solubility test
Specialized laboratory tests are as follows:
Alpha 2-plasmin inhibitor levels: Evaluate alpha 2-plasmin inhibitor (alpha 2-PI, a2-PI) levels with the use of antigenic and functional assays. Perform functional assays with both biologic and chromogenic tests. In addition, evaluate for a genetic defect in collaboration with a specialized laboratory.
t-PA antigen and activity levels
Plasminogen functional activity levels
Levels of other inhibitors: These include alpha 2-macroglobulin, alpha 1-antitrypsin, alpha 1-chymotrypsin inhibitor, C1 inactivator of complement, and antithrombin.
Use computed tomography (CT) scanning, magnetic resonance imaging (MRI), or ultrasonography as needed for objective documentation of the size and resolution of bleeds.
Essential surgical procedures in those with alpha 2-plasmin inhibitor deficiency (alpha 2-PI deficiency, a2-PI deficiency) should be performed only after re-evaluating the level of alpha 2-PI and after deciding on the need for plasma and administration of aminocaproic acid (Amicar).
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