eMedicine Specialties > Emergency Medicine > Hematology & Oncology

Hemophilia, Type A: Differential Diagnoses & Workup

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

Differential Diagnoses

Hemophilia, Type B

Other Problems to Be Considered

von Willebrand disease
Vitamin K and other factor deficiencies
Afibrinogenemia
Dysfibrinogenemia
Fibrinolytic defects
Platelet disorders

Workup

Laboratory Studies

  • Never delay indicated coagulation correction pending diagnostic testing.
  • Hemoglobin/hematocrit
    • Assess blood loss.
    • Expect normal range or low values.
  • Prothrombin time (PT)
    • Extrinsic coagulation pathway screen
    • Normal range expected
  • Activated partial thromboplastin time (aPTT)
    • Intrinsic pathway screen
    • Elevated values expected
    • May be normal range in mild disease
  • Platelet count
    • Assess bleeding.
    • Expect normal range.
  • Factor VIII level
    • Assess percentage activity (normal 50-150%).
    • Expect severe disease with less than 1%, moderate disease with 1-5%, and mild disease with greater than 5%.
  • Factor VIII inhibitors
    • Assess presence.
    • Assess anamnestic response to factor VIII.
    • Expect low titer (0-10 Bethesda U) or high titer (>10 Bethesda U).

Imaging Studies

  • Early and aggressive imaging is indicated, even with low suspicion for hemorrhage, after coagulation therapy is initiated.
  • Head CT scan (noncontrast): Assess spontaneous or traumatic hemorrhage.
  • Body CT scan
    • Perform with or without intravenous (IV) and/or oral contrast.
    • Perform as indicated by clinical suspicion and anatomical location.
    • Assess spontaneous or traumatic hemorrhage.
  • MRI
    • Head and spinal column
    • Further assessment of spontaneous or traumatic hemorrhage
  • X-ray for joint assessment
    • Of limited value in acute setting of hemarthrosis
    • Chronic degenerative joint disease often present
  • Special studies (as clinically indicated)
    • Angiography
    • Nucleotide bleeding scan

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

  1. Coagulation Factor VIIa Recombinant [package insert]. Denmark: Novo Nordisk; 2006. [Full Text].

  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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