eMedicine Specialties > Emergency Medicine > Hematology & Oncology

Hemophilia, Type B: Follow-up

Author: Brendan R Furlong, MD, Clinical Chief, Department of Emergency Medicine, Georgetown University Hospital
Coauthor(s): Mary A Furlong, MD, Associate Professor and Program/Residency Director, Department of Pathology, Georgetown University School of Medicine
Contributor Information and Disclosures

Updated: Oct 6, 2008

Follow-up

Further Inpatient Care

  • Continue further hemostatic (eg, splenectomy, posttrauma) and supportive care, as indicated.
  • The patient's disposition (ICU vs floor) is based on the severity of hemorrhage and potential for morbidity and death.
  • Choose attending service based on etiology of hemorrhage.
  • Hematology/blood bank/pathology consultation is mandatory.
  • Continue factor replacement and monitoring.
  • Administer epsilon aminocaproic acid, as indicated.
  • Consider inhibitor screening.

Further Outpatient Care

  • Minor hemorrhage (not life threatening): Continue hemostatic measures (eg, brief joint immobilization, bandage).
  • Hematologist or primary care physician follow-up care is indicated.
  • Continue factor replacement and monitoring.
  • Administer epsilon aminocaproic acid, as indicated.
  • Hepatitis A and B immunizations are recommended.

Transfer

  • The preference is to bring factor IX to the patient.
  • Transfer to a tertiary care center when indicated specialists are not available.
  • Transfer to a hemophilia treatment center for optimal hematologic management.

Deterrence/Prevention

  • Consider prophylactic or scheduled factor IX.
  • Optimize physical conditioning.
  • Avoid high-risk activities.
  • Gene therapy may be available in the future.

Complications

  • Ongoing hemorrhage with resulting morbidity and death
  • Inhibitor development
  • Exposure/infection from blood products (eg, HIV; hepatitis A, B, C; unknown viruses)

Prognosis

  • With appropriate education and treatment, patients with hemophilia can live full and productive lives.
  • Life expectancy was approaching age 60 years prior to HIV epidemic in the 1980s.
  • HIV-infected individuals are likely to succumb to HIV/AIDS-related complications.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • Delay of indicated coagulation correction pending diagnostic testing
  • Failure to monitor the results of treatments administered
  • Failure to administer prophylactic factor IX replacement, which is indicated prior to invasive procedures (eg, lumbar puncture [LP], tooth extraction)
  • Failure to recognize the remote risk of viral infection from blood products
  • Failure to consider the presence of inhibitors and treat accordingly.

Special Concerns

  • Inhibitors
    • Inhibitors are antibodies that neutralize factor IX and can render replacement therapy ineffective.
    • They are less common than factor VIII inhibitors and are found in only 3% of those with severe disease.
    • Low-titer inhibitors and, occasionally, high-titer inhibitors can be overcome with high doses of factor IX.
    • The relatively low frequency of this problem has lead to a dearth of experience in its treatment.
    • Recombinant human coagulation factor VIIa (rFVIIa) is indicated for the treatment of patients with bleeding episodes and for the prevention of bleeding in surgical interventions or invasive procedures in patients with hemophilia A or B with inhibitors to factor VIII or factor IX.
    • Recent treatments with the anti-inhibitor coagulant complex factor VIII inhibitor bypassing activity (FEIBA) have shown promise.
    • Immune-tolerance strategies in those with identified inhibitors have been successful as well.
    • Early hematology consultation for these patients is essential.
  • Cryoprecipitate contains no factor IX and is not appropriate for factor IX therapy.
  • DDAVP is not a treatment for factor IX deficiency.
 


More on Hemophilia, Type B

Overview: Hemophilia, Type B
Differential Diagnoses & Workup: Hemophilia, Type B
Treatment & Medication: Hemophilia, Type B
Follow-up: Hemophilia, Type B
References
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References

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  2. Adamson S, Charlebois T, O'Connell B, Foster W. Viral safety of recombinant factor IX. Semin Hematol. Apr 1998;35(2 Suppl 2):22-7. [Medline].

  3. Bell B, Canty D, Audet M. Hemophilia: an updated review. Pediatr Rev. Aug 1995;16(8):290-8. [Medline].

  4. Bolan CD, Alving BM. Pharmacologic agents in the management of bleeding disorders. Transfusion. Jul-Aug 1990;30(6):541-51. [Medline].

  5. Brettler DB, Levine PH. Clinical manifestations and therapy of inherited coagulation factor deficiencies. In: Hemostasis and Thrombosis: Basic Principles and Clinical Practice, Third Edition. 1994:169-83.

  6. Brinkhous KM, Sigman JL, Read MS, et al. Recombinant human factor IX: replacement therapy, prophylaxis, and pharmacokinetics in canine hemophilia B. Blood. Oct 1 1996;88(7):2603-10. [Medline].

  7. DiMichele D, Neufeld EJ. Hemophilia. A new approach to an old disease. Hematol Oncol Clin North Am. Dec 1998;12(6):1315-44. [Medline].

  8. Dunn AL, Abshire TC. Recent advances in the management of the child who has hemophilia. Hematol Oncol Clin North Am. Dec 2004;18(6):1249-76, viii. [Medline].

  9. Furie B, Limentani SA, Rosenfield CG. A practical guide to the evaluation and treatment of hemophilia. Blood. Jul 1 1994;84(1):3-9. [Medline].

  10. Goldsmith JC. Hemophilia: Current Medical Management. The National Hemophilia Foundation; 1994:1-30.

  11. Ludlam CA. Treatment of haemophilia. Br J Haematol. May 1998;101 Suppl 1:13-4. [Medline].

  12. Medical and Scientific Advisory Council (MASAC) of the National Hemophilia Found. Recommendations concerning prophylaxis. Medical Bulletin #193. 1994;1-3.

  13. Medical and Scientific Advisory Council (MASAC) of the National Hemophilia Found. Recommendations regarding the use of recombinant factor VIII in the treatment of hemophilia A. Medical Bulletin # 232. 1995;1-2.

  14. Medical and Scientific Advisory Council (MASAC) of the National Hemophilia Found. Revised recommendations regarding hepatitis A vaccination in individuals with hemophilia and other congenital bleeding disorders. Medical Advisory # 277. 1997;1-2.

  15. Soucie JM, Evatt B, Jackson D. Occurrence of hemophilia in the United States. The Hemophilia Surveillance System Project Investigators. Am J Hematol. Dec 1998;59(4):288-94. [Medline].

  16. Thompson A. Recombinant factor IX for the treatment of hemophilia B. Introduction. Semin Hematol. Apr 1998;35(2 Suppl 2):1-3. [Medline].

  17. White G, Shapiro A, Ragni M. Clinical evaluation of recombinant factor IX. Semin Hematol. Apr 1998;35(2 Suppl 2):33-8. [Medline].

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Further Reading

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Keywords

hemophilia type B, hemophilia type B, blood disorder, deficiency of factor IX, factor IX deficiency, dysfunctional factor IX, factor IX inhibitors, hemorrhage, bleeding, bleeding disorder, factor IX activity, plasma coagulation

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Contributor Information and Disclosures

Author

Brendan R Furlong, MD, Clinical Chief, Department of Emergency Medicine, Georgetown University Hospital
Brendan R Furlong, MD is a member of the following medical societies: American College of Emergency Physicians and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Mary A Furlong, MD, Associate Professor and Program/Residency Director, Department of Pathology, Georgetown University School of Medicine
Mary A Furlong, MD is a member of the following medical societies: United States and Canadian Academy of Pathology
Disclosure: Nothing to disclose.

Medical Editor

William G Gossman, MD, Associate Clinical Professor of Emergency Medicine, Creighton University School of Medicine; Consulting Staff, Department of Emergency Medicine, Creighton University Medical Center
William G Gossman, MD is a member of the following medical societies: American Academy of Emergency Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Jeffrey L Arnold, MD, FACEP, Chairman, Department of Emergency Medicine, Santa Clara Valley Medical Center
Jeffrey L Arnold, MD, FACEP is a member of the following medical societies: American Academy of Emergency Medicine and American College of Physicians
Disclosure: Nothing to disclose.

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

Steven C Dronen, MD, FAAEM, Director of Emergency Services, Director of Chest Pain Center, Department of Emergency Medicine, Ft Sanders Sevier Medical Center
Steven C Dronen, MD, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

 
 
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