eMedicine Specialties > Hematology > Coagulation, Hemostasis, and Disorders

Factor XIII: Follow-up

Author: Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Coauthor(s): Elzbieta Klujszo, MD, Head of Department of Dermatology, Wojewodzki Szpital Zespolony, Kielce; Pere Gascon, MD, PhD, Professor and Director, Division of Medical Oncology, Institute of Hematology and Medical Oncology, IDIBAPS, University of Barcelona Faculty of Medicine, Spain; Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Contributor Information and Disclosures

Updated: Sep 26, 2007

Follow-up

Further Inpatient Care

Patients should be hospitalized for serious complications, such as severe bleeding, or for major surgical procedures, all which require complex interdisciplinary care including pharmacy and laboratory support. Constant clinical evaluation and laboratory monitoring ensure adequacy of product replacement, pain relief, and other supportive care. The hematologist must be centrally involved to coordinate care.

Further Outpatient Care

  • Clinic-supervised outpatient care is an extremely important part of treatment.
    • Complete annual physical examinations and laboratory testing for inhibitors, hepatitis, and HIV infection, as well as other tests, should be performed as needed.
    • As in persons without hemophilia, routine care should be provided to patients with FXIII deficiency, including examination of stool for blood, rectal examination, colonoscopy, prostate-specific antigen determinations, mammography, and dental care.
    • Physical therapy may be needed for the long-term care of affected joints.
    • Prophylactic care includes vaccination for HAV105 and HBV and other routine vaccines.
  • Notify the local chapter of the National Hemophilia Foundation regarding the patient so that proper statistics can be provided to the appropriate agencies for adequate federal and state funding of patient care.
  • Provide counseling and classes to encourage questions and to help solve problems, such as possible ways to avoid transmission of HIV to an uninfected spouse and to children.106
  • An appointment with a psychosocial worker at the time of counseling ensures that other psychological, social, and economic support is provided.

Inpatient & Outpatient Medications

Patients should avoid acetylsalicylic acid, NSAIDs, and any over-the-counter herbal medications that can precipitate bleeding.

Transfer

  • If a qualified hematologist and laboratory personnel with expertise are available, patients may be cared for in a setting close to home. Laboratory testing provided by community hospitals has been improved by the existence of commercial referral laboratories.
  • Federal and state funding for programs may be available through a medical center. Costs of care are much higher at tertiary medical centers.

Deterrence/Prevention

  • Avoidance of high-risk activities (eg, boxing, motorbike riding) and NSAIDs reduces the frequency of bleeding.
  • Avoidance of alcohol helps protect liver function in patients with hepatitis.
  • Primary prophylaxis is the best way to prevent recurrent CNS bleeding, recurrent miscarriages or, rarely, recurrent joint bleeding. Joint replacement may be needed in the older patient with severe arthropathy.
  • HAV and HBV vaccines should be administered. Other routine vaccinations, such as those for influenza and pneumonia, should be provided as in other persons.

Complications

  • Recurrent CNS bleeding is a major problem requiring prophylactic transfusions. Infections, particularly HIV, AIDS, and chronic hepatitis, can lead to death. Interferon alfa has been used to treat chronic viral hepatitis. Multidrug cocktails are used to treat HIV/AIDS, but protease inhibitors can increase risk of bleeding. Some over-the-counter herbal remedies increase the risk of bleeding.
  • Viral safety in products derived from plasma is ensured through several techniques, ie, heating, pasteurization, SD treatment, and monoclonal antibody purification. These procedures currently free products from HIV and HCV (lipid-enveloped viruses) but do not solve the problem of transmission of non–lipid-enveloped viruses such as HAV, parvovirus B19, and TTV. Even with recombinant products, a possibility exists of contamination with pathogens previously unknown, including new murine viruses. A recent report shows the presence of TTV in first-generation recombinant products, which is due to the use of human serum albumin that is contaminated with TTV.103 Thus, virus-induced illnesses of concern include hepatitis viruses A-E, GB virus C (or hepatitis G virus), the SEN family of viruses,102 and human herpesvirus 8,101 all of which constitute emerging pathogens related to transfusion-transmitted illnesses.
  • A recent review reports concerns about the transmission of CJD or its variant form (vCJD) in recipients of blood products. The Transmissible Spongiform Encephalopathies Advisory Committee of the FDA has suggested limiting the donor pool and excluding donors who have lived or traveled for 5 years or longer in Europe or donors who have lived in the United Kingdom for a total of 3 months or longer. The development of new tests to check for vCJD is anticipated.107,101
  • The presence of inhibitors adds another layer of complexity when alloantibodies develop as a consequence of transfusion of blood products. Spontaneous disappearance is a typical feature of autoantibodies, presumably as a response to removal of the antigenic stimulus. Bleeding associated with inhibitors can be life threatening and requires complex care.
  • Severe economic and emotional problems occur as a result of the recurrent nature of the bleeding.
  • Complex psychiatric issues arise in the treatment of patients with HIV/AIDS.106

Prognosis

  • Prognosis depends on the types of complications that develop, on the type of replacement product the patient has received, and on the viral infections that the patient has accumulated over the years. Newly diagnosed patients should, whenever possible, receive purer products to ensure maximum safety.
  • The presence of inhibitors in patients poses a serious therapeutic challenge, and, currently, surgery should be considered only as a lifesaving measure.

Patient Education

  • Encourage patients to register with the local chapter of the National Hemophilia Foundation and to attend educational seminars.
  • Provide one-on-one discussions of issues with patients and family members.
  • Early and complete genetic testing can help families plan future pregnancies.
  • For excellent patient education resources, visit eMedicine's Pregnancy and Reproduction Center. Also, see eMedicine's patient education article Miscarriage.

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose FXIII deficiency, to provide the correct product replacement or appropriate and adequate therapy, and to prevent inordinate delays in replacing factor
  • Failure to diagnose HIV infection
  • Failure to discuss short- and long-term consequences of the use of specific products with patients, whenever feasible

Special Concerns

Genetic testing to determine the type of defect in the family is important; the information then is available for prenatal testing in appropriate families. Prenatal testing provides a family with more choices. Consequences of genetic testing of the propositus and family should be discussed before testing is implemented. During pregnancy, labor, and delivery, treat the deficiency with approximately once-a-month replacement therapy. FXIII levels should be monitored.

 


More on Factor XIII

Overview: Factor XIII
Differential Diagnoses & Workup: Factor XIII
Treatment & Medication: Factor XIII
Follow-up: Factor XIII
Multimedia: Factor XIII
References
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Further Reading

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Keywords

plasma transglutaminase, fibrin stabilizing factor, transligase, factor XIII deficiency, fibrin stabilizing factor deficiency, FXIII, FXIII deficiency, rFXIII, FXIIIa, hemophilia, bleeding diathesis, autosomal blood disorder, blood disorder, congenital hemorrhagic diathesis, coagulation disorder, tissue transglutaminase, thrombin

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Contributor Information and Disclosures

Author

Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Elzbieta Klujszo, MD, Head of Department of Dermatology, Wojewodzki Szpital Zespolony, Kielce
Disclosure: Nothing to disclose.

Pere Gascon, MD, PhD, Professor and Director, Division of Medical Oncology, Institute of Hematology and Medical Oncology, IDIBAPS, University of Barcelona Faculty of Medicine, Spain
Pere Gascon, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, New York Academy of Medicine, New York Academy of Sciences, and Sigma Xi
Disclosure: Nothing to disclose.

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, 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.

Medical Editor

Paul Schick, MD, Emeritus Professor, Department of Internal Medicine, Thomas Jefferson University Medical College; Research Professor, Department of Internal Medicine, Drexel University College of Medicine
Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Hematology, International Society on Thrombosis and Haemostasis, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Ronald A Sacher, MD, Director of the Hoxworth Blood Center, Professor, Departments of Internal Medicine and Pathology, University of Cincinnati Medical Center
Ronald A Sacher, MD is a member of the following medical societies: American Society of Hematology
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

CME Editor

Rebecca J Schmidt, DO, FACP, FASN, Clinical Associate Professor of Medicine, West Virginia School of Osteopathic Medicine; Professor of Medicine, Section Chief, Department of Medicine, Section of Nephrology, West Virginia University School of Medicine
Rebecca J Schmidt, DO, FACP, FASN is a member of the following medical societies: American College of Osteopathic Internists, American College of Physicians, American Medical Association, American Society of Nephrology, International Society of Nephrology, National Kidney Foundation, Renal Physicians Association, and West Virginia State Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, 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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