eMedicine Specialties > Emergency Medicine > Hematology & Oncology

Hemophilia, Type A: 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.
  • Disposition (ICU vs floor) should be based on 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 DDAVP/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 DDAVP/epsilon aminocaproic acid as indicated.
  • Hepatitis A and B immunizations are recommended.

Transfer

  • The preference is to bring factor VIII to the patient.
  • Transfer to a tertiary care center when indicated specialists (eg, neurosurgery) are not available.
  • Transfer to a hemophilia treatment center for optimal hematologic management.

Deterrence/Prevention

  • Consider prophylactic or scheduled factor VIII.
  • Optimize physical conditioning.
  • Avoid high-risk activities.
  • Gene therapy is a possibility 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 60 years prior to HIV epidemic in the 1980s.
  • HIV-infected individuals are likely to die of that disease than from hemophilia.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • Failure to administer indicated coagulation correction promptly pending diagnostic testing
  • Failure to monitor the results of treatments administered
  • Failure to administer prophylactic factor VIII 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 VIII and can render replacement therapy ineffective.
    • They are found more commonly in patients with moderate to severe hemophilia (up to 30% of those with severe disease) who have received significant amounts of replacement therapy.
    • Inhibitors can rarely develop in individuals without hemophilia (eg, elderly persons, pregnant women) and occasionally are responsive to immunosuppressive therapy (eg, prednisone).
    • Immune tolerance strategies in those with identified inhibitors also have been successful.
    • Assuming no anamnestic response, low-titer inhibitors occasionally can be overcome with high doses of factor VIII.
    • 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.
    • High-titer inhibitors have been treated with variable success using porcine factor VIII, factor IX complex concentrates, recombinant factor VIII, and exchange plasma pheresis.
    • Early hematology consultation for management of this type of patient is essential.
 


More on Hemophilia, Type A

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

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

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

  4. Brettler DB, Levine PH. Clinical manifestations and therapy of inherited coagulation factor deficiencies. In: Hemostasis and Thrombosis: Basic Principles and Clinical Practice. 3rd ed. 1994:169-83.

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

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

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

  8. Luck JV, Silva M, Rodriguez-Merchan EC, et al. Hemophilic arthropathy. J Am Acad Orthop Surg. Jul-Aug 2004;12(4):234-45. [Medline].

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

  10. Manco-Johnson M. Hemophilia management: optimizing treatment based on patient needs. Curr Opin Pediatr. Feb 2005;17(1):3-6. [Medline].

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

  12. 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.

  13. 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.

  14. Mudad R, Kane WH. DDAVP in acquired hemophilia A: case report and review of the literature. Am J Hematol. Aug 1993;43(4):295-9. [Medline].

  15. Schneiderman J, Nugent DJ, Young G. Sequential therapy with activated prothrombin complex concentrate and recombinant factor VIIa in patients with severe haemophilia and inhibitors. Haemophilia. Jul 2004;10(4):347-51. [Medline].

  16. 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].

  17. von Depka M. Immune tolerance therapy in patients with acquired hemophilia. Hematology. Aug 2004;9(4):245-57. [Medline].

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Further Reading

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Keywords

hemophilia type A, hemophilia A, deficiency of functional plasma coagulation factor VIII, factor VIII deficiency, dysfunctional factor VIII, factor VIII inhibitors, disruption of the normal intrinsic coagulation cascade

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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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