eMedicine Specialties > Pediatrics: General Medicine > Hematology

Antithrombin III Deficiency: Follow-up

Author: James L Harper, MD, Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center
Contributor Information and Disclosures

Updated: Jul 10, 2009

Follow-up

Complications

  • Most thrombotic complications involve the venous circulation; venous insufficiency is a common complication.
  • In children more than in adults, antithrombin III (ATIII) deficiency may cause arterial occlusion resulting in stroke or other ischemic injury.

Prognosis

Prognosis depends on 3 variables: the degree of the deficiency, the nature of the observed clot, and the number of clots seen.

  • Prognosis for homozygous-deficient antithrombin III deficiency is grim. Typically, severe thrombotic complications arise in neonates. Prognosis for survival into adulthood is good for patients who are heterozygous deficient. The peak risk for thrombotic complications does not arise until the third and fourth decades of life.
  • The nature of the observed clot is an important variable. A clot that occurs in the mesenteric circulation or other central vein carries a more ominous prognosis than clots in the peripheral circulation. Patients with clots in the mesenteric circulation are often best treated with indefinite anticoagulation, even after a single episode.
  • Clots that recur, particularly those that recur either in the central or mesenteric circulation, are of important prognostic significance. Patients with such clots are much more likely to continue to experience thrombotic episodes and eventually to suffer either a life-threatening clot or suffer needlessly from the complications of the thrombi. One thrombotic event may be treated successfully with limited warfarin therapy, particularly if no other prothrombotic condition is present. Patients who experience multiple thrombi are often best treated with indefinite warfarin therapy.

Patient Education

  • Education regarding signs and symptoms of thrombi, as well as the risks associated with warfarin therapy, is the cornerstone of management.
  • Given the teratogenic potential of warfarin, carefully instruct female patients on the importance of avoiding pregnancy. These patients should alert their hematology team regarding any pregnancy or intention to become pregnant so that alternative anticoagulation strategies may be explored in a timely manner.
  • For excellent patient education resources, visit eMedicine's Circulatory Problems Center. Also, see eMedicine's patient education article Blood Clot in the Legs.
  • Medlineplus.gov has a continually updated listing of patient information for antithrombin deficiency.

Miscellaneous

Medicolegal Pitfalls

  • Complications of undertreated antithrombin III (ATIII) deficiency comprise an important pitfall, as do the complications of warfarin therapy. Careful follow-up care and monitoring of the patient's degree of anticoagulation and general clinical course are important.
  • Physicians who practice in rural environments where laboratory studies may be performed at a referring hospital must take care to ensure that data are accurate and reliable. Use of the international normalized ratio (INR) for comparison of the prothrombin time (PT) data is essential to treat patients of these physicians.
  • Parents of a newborn that is found to have a congenital anticoagulation factor deficiency or a thrombotic event in the neonatal period are themselves at an increased risk of thrombotic disease and should be referred for evaluation of their own coagulation status.

Special Concerns

  • Warfarin is potentially teratogenic. All female patients of childbearing potential, and their parents, should be strongly counseled regarding the importance of avoiding unplanned pregnancies. They should keep their hematologist informed of any pregnancy.
  • Lovenox requires daily subcutaneous infusions that may be problematic for families of sick neonates and children. Home health care should be set up prior to discharge to assist families in getting started at home. Home health care should also be available in case families are unable to administer the medication; the short half life of the medication will affect its efficacy, if doses are missed.
 


More on Antithrombin III Deficiency

Overview: Antithrombin III Deficiency
Differential Diagnoses & Workup: Antithrombin III Deficiency
Treatment & Medication: Antithrombin III Deficiency
Follow-up: Antithrombin III Deficiency
Multimedia: Antithrombin III Deficiency
References
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References

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Further Reading

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Keywords

antithrombin III deficiency, acquired antithrombin deficiency, congenital antithrombin deficiency, AT-III deficiency, ATIII deficiency, AT III deficiency, heterozygous antithrombin deficiency, heparin, low molecular weight heparin, thrombin disorder, anticoagulation, anti-coagulation, venous thrombosis, arterial thrombosis, clotting disorder, blood clots, hematologic disorder, increased thrombogenesis, inappropriate activation of the clotting system, inappropriate coagulation, coagulopathy, disseminated intravascular coagulation, DIC, microangiopathic hemolytic anemias due to endothelial damage, hemolytic-uremic syndrome, veno-occlusive disease, venoocclusive disease, VOD, protein C deficiency, protein S deficiency, liver disease, nephrotic syndrome, bone marrow transplantation, treatment, diagnosis

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Contributor Information and Disclosures

Author

James L Harper, MD, Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center
James L Harper, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society
Disclosure: Nothing to disclose.

Medical Editor

Gary R Jones, MD, Associate Medical Director, Clinical Development, Berlex Laboratories
Gary R Jones, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Gary D Crouch, MD, Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Associate Professor, Uniformed Services University of the Health Sciences
Gary D Crouch, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Hematology
Disclosure: Nothing to disclose.

CME Editor

David Pallares, MD, Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville
David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology
Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD, King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine
Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

 
 
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