Alpha2-Plasmin Inhibitor Deficiency Workup

  • Author: Olga Kozyreva, MD; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Jan 6, 2012
 

Laboratory Studies

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)
  • Factor II, V, VII, and X levels if PT is prolonged
  • Platelet function
  • Screening for factor XIII deficiency using a urea or monochloroacetic acid solubility test
  • Thrombin time

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.
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Imaging Studies

Use computed tomography (CT) scanning, magnetic resonance imaging (MRI), or ultrasonography as needed for objective documentation of the size and resolution of bleeds.

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Procedures

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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Contributor Information and Disclosures
Author

Olga Kozyreva, MD  Attending Physician, Division of Hematology-Oncology, St Elizabeth's Medical Center; Assistant Professor, Tufts University School of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Samer A Bleibel, MD  Staff Physician, Department of Internal Medicine, Wayne State University School of Medicine, St John's Hospital and Medical Centers

Samer A Bleibel, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Sarah K May, MD  Consulting Staff, Department of Hematology-Oncology, Caritas Carney Hospital, Commonwealth Hematology-Oncology PC

Disclosure: Nothing to disclose.

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Jeanine Walenga, PhD  Co-Director, Hemostasis Research Laboratory, Professor, Departments of Thoracic-Cardiovascular Surgery and Pathology, Loyola University Medical Center

Jeanine Walenga, PhD is a member of the following medical societies: American Association for Clinical Chemistry, American Heart Association, American Society for Clinical Pathology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Specialty Editor Board

S Gerald Sandler, MD, FACP, FCAP  Professor of Medicine and Pathology, Director, Transfusion Medicine, Department of Laboratory Medicine, Georgetown University Hospital

S Gerald Sandler, MD, FACP, FCAP is a member of the following medical societies: American Association of Blood Banks, College of American Pathologists, and International Society of Blood Transfusions

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Marcel E Conrad, MD  Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group

Disclosure: No financial interests None None

Timothy D Rice, MD  Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, St Louis University School of Medicine

Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

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The role of alpha2-plasmin inhibitor (alpha2-antiplasmin) in fibrinolysis.
 
 
 
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