eMedicine Specialties > Hematology > Coagulation, Hemostasis, and Disorders

Factor VIII: Multimedia

Author: Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and 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, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Contributor Information and Disclosures

Updated: Aug 13, 2009

Multimedia

Obituary in the March 22, 1796, <i>Salem Gazette<...Media file 1: Obituary in the March 22, 1796, Salem Gazette (Massachusetts) for a 19-year-old man who bled to death after suffering a foot injury. Also detailed are the deaths of 5 brothers by various minor injuries.
Obituary in the March 22, 1796, <i>Salem Gazette<...

Obituary in the March 22, 1796, Salem Gazette (Massachusetts) for a 19-year-old man who bled to death after suffering a foot injury. Also detailed are the deaths of 5 brothers by various minor injuries.

The hemostatic pathway: role of factor VIII.Media file 2: The hemostatic pathway: role of factor VIII.
The hemostatic pathway: role of factor VIII.

The hemostatic pathway: role of factor VIII.

Structural domains of human factor VIII. Adapted ...Media file 3: Structural domains of human factor VIII. Adapted from: Stoilova-McPhie S, Villoutreix BO, Mertens K, Kemball-Cook G, Holzenburg A. 3-Dimensional structure of membrane-bound coagulation factor VIII: modeling of the factor VIII heterodimer within a 3-dimensional density map derived by electron crystallography. Blood. Feb 15 2002;99(4):1215-23 45; Roberts HR, Hoffman M. Hemophilia A and B. In: Beutler E, Lichtman MA, Coller BS, et al, eds. Williams Hematology. 6th ed. NY: McGraw-Hill; 2001:1639-57 34; and Roberts HR. Thoughts on the mechanism of action of FVIIa. Presented at: Second Symposium on New Aspects of Haemophilia Treatment; 1991; Copenhagen, Denmark. 46
Structural domains of human factor VIII. Adapted ...

Structural domains of human factor VIII. Adapted from: Stoilova-McPhie S, Villoutreix BO, Mertens K, Kemball-Cook G, Holzenburg A. 3-Dimensional structure of membrane-bound coagulation factor VIII: modeling of the factor VIII heterodimer within a 3-dimensional density map derived by electron crystallography. Blood. Feb 15 2002;99(4):1215-23 45; Roberts HR, Hoffman M. Hemophilia A and B. In: Beutler E, Lichtman MA, Coller BS, et al, eds. Williams Hematology. 6th ed. NY: McGraw-Hill; 2001:1639-57 34; and Roberts HR. Thoughts on the mechanism of action of FVIIa. Presented at: Second Symposium on New Aspects of Haemophilia Treatment; 1991; Copenhagen, Denmark. 46

Cell surface–directed hemostasis (adapted f...Media file 4: Cell surface–directed hemostasis (adapted from: Hoffman M, Monroe DM 3rd. A cell-based model of hemostasis. Thromb Haemost. Jun 2001;85(6):958-65. 47). Initially, a small amount of thrombin is generated on the surface of the tissue factor–bearing cell. Following amplification, the second burst generates a larger amount of thrombin, leading to fibrin (clot) formation.
Cell surface–directed hemostasis (adapted f...

Cell surface–directed hemostasis (adapted from: Hoffman M, Monroe DM 3rd. A cell-based model of hemostasis. Thromb Haemost. Jun 2001;85(6):958-65. 47). Initially, a small amount of thrombin is generated on the surface of the tissue factor–bearing cell. Following amplification, the second burst generates a larger amount of thrombin, leading to fibrin (clot) formation.

Possible genetic outcomes in individuals carrying...Media file 5: Possible genetic outcomes in individuals carrying the hemophilic gene.
Possible genetic outcomes in individuals carrying...

Possible genetic outcomes in individuals carrying the hemophilic gene.

Photograph of a teenage boy with bleeding into hi...Media file 6: Photograph of a teenage boy with bleeding into his right thigh as well as both knees and ankles.
Photograph of a teenage boy with bleeding into hi...

Photograph of a teenage boy with bleeding into his right thigh as well as both knees and ankles.

Photograph of the right knee in an older man with...Media file 7: Photograph of the right knee in an older man with a chronically fused, extended knee following open drainage of knee bleeding that occurred many years previously.
Photograph of the right knee in an older man with...

Photograph of the right knee in an older man with a chronically fused, extended knee following open drainage of knee bleeding that occurred many years previously.

Photograph depicting severe bilateral hemophilic ...Media file 8: Photograph depicting severe bilateral hemophilic arthropathy and muscle wasting. The 3 punctures made into the left knee joint were performed in an attempt to aspirate recent aggravated bleeding.
Photograph depicting severe bilateral hemophilic ...

Photograph depicting severe bilateral hemophilic arthropathy and muscle wasting. The 3 punctures made into the left knee joint were performed in an attempt to aspirate recent aggravated bleeding.

Radiograph depicting advanced hemophilic arthropa...Media file 9: Radiograph depicting advanced hemophilic arthropathy of the knee joint. These images show chronic severe arthritis, fusion, loss of cartilage, and joint space deformities.
Radiograph depicting advanced hemophilic arthropa...

Radiograph depicting advanced hemophilic arthropathy of the knee joint. These images show chronic severe arthritis, fusion, loss of cartilage, and joint space deformities.

Radiograph depicting advanced hemophilic arthropa...Media file 10: Radiograph depicting advanced hemophilic arthropathy of the elbow. This image shows chronic severe arthritis, fusion, loss of cartilage, and joint space deformities.
Radiograph depicting advanced hemophilic arthropa...

Radiograph depicting advanced hemophilic arthropathy of the elbow. This image shows chronic severe arthritis, fusion, loss of cartilage, and joint space deformities.

Photograph of a hemophilic knee at surgery, with ...Media file 11: Photograph of a hemophilic knee at surgery, with synovial proliferation caused by repeated bleeding; synovectomy was required.
Photograph of a hemophilic knee at surgery, with ...

Photograph of a hemophilic knee at surgery, with synovial proliferation caused by repeated bleeding; synovectomy was required.

Large amount of vascular synovium removed at surg...Media file 12: Large amount of vascular synovium removed at surgery (same knee as in Image 11).
Large amount of vascular synovium removed at surg...

Large amount of vascular synovium removed at surgery (same knee as in Image 11).

Microscopic appearance of synovial proliferation ...Media file 13: Microscopic appearance of synovial proliferation and high vascularity. If stained with iron, diffuse deposits would be demonstrated; iron-laden macrophages are present.
Microscopic appearance of synovial proliferation ...

Microscopic appearance of synovial proliferation and high vascularity. If stained with iron, diffuse deposits would be demonstrated; iron-laden macrophages are present.

Large pseudocyst involving the left proximal femu...Media file 14: Large pseudocyst involving the left proximal femur.
Large pseudocyst involving the left proximal femu...

Large pseudocyst involving the left proximal femur.

Transected pseudocyst (following disarticulation ...Media file 15: Transected pseudocyst (following disarticulation of the left lower extremity due to vascular compromise, nerve damage, loss of bone, and nonfunctional limb). This photo shows black-brown old blood, residual muscle, and bone.
Transected pseudocyst (following disarticulation ...

Transected pseudocyst (following disarticulation of the left lower extremity due to vascular compromise, nerve damage, loss of bone, and nonfunctional limb). This photo shows black-brown old blood, residual muscle, and bone.

Dissection of a pseudocyst.Media file 16: Dissection of a pseudocyst.
Dissection of a pseudocyst.

Dissection of a pseudocyst.

Transected pseudocyst with chocolate brown-black ...Media file 17: Transected pseudocyst with chocolate brown-black old blood.
Transected pseudocyst with chocolate brown-black ...

Transected pseudocyst with chocolate brown-black old blood.

Photograph of a patient who presented with a slow...Media file 18: Photograph of a patient who presented with a slowly expanding abdominal and flank mass, as well as increasing pain, inability to eat, weight loss, and weakness of his lower extremity.
Photograph of a patient who presented with a slow...

Photograph of a patient who presented with a slowly expanding abdominal and flank mass, as well as increasing pain, inability to eat, weight loss, and weakness of his lower extremity.

Plain radiograph of the pelvis showing a large ly...Media file 19: Plain radiograph of the pelvis showing a large lytic area.
Plain radiograph of the pelvis showing a large ly...

Plain radiograph of the pelvis showing a large lytic area.

Intravenous pyelogram showing extreme displacemen...Media file 20: Intravenous pyelogram showing extreme displacement of the left kidney and ureter by a pseudocyst.
Intravenous pyelogram showing extreme displacemen...

Intravenous pyelogram showing extreme displacement of the left kidney and ureter by a pseudocyst.

Photograph depicting extensive spontaneous abdomi...Media file 21: Photograph depicting extensive spontaneous abdominal wall hematoma and thigh hemorrhage in an older, previously unaffected man with an acquired factor VIII inhibitor.
Photograph depicting extensive spontaneous abdomi...

Photograph depicting extensive spontaneous abdominal wall hematoma and thigh hemorrhage in an older, previously unaffected man with an acquired factor VIII inhibitor.

Magnetic resonance image of an extensive spontane...Media file 22: Magnetic resonance image of an extensive spontaneous abdominal wall hematoma and thigh hemorrhage in an older, previously unaffected man with an acquired factor VIII inhibitor (same patient as in Image 21).
Magnetic resonance image of an extensive spontane...

Magnetic resonance image of an extensive spontaneous abdominal wall hematoma and thigh hemorrhage in an older, previously unaffected man with an acquired factor VIII inhibitor (same patient as in Image 21).

Image depicting the 28q region of the X chromosom...Media file 23: Image depicting the 28q region of the X chromosome. Adapted from: Kazazian HH Jr, Tuddenham EGD, Antonarakis SE. Hemophilia A and parahemophilia: deficiencies of coagulation factors VIII and V. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic and Molecular Bases of Inherited Disease. 7th ed. New York, NY: McGraw-Hill; 1995:3241-67 4; Reitsma PH. Genetic principles underlying disorders of procoagulant and anticoagulant proteins. In: Coleman RW, Hirsh J, Marder VJ, et al, eds. Hemostasis and Thrombosis: Basic Principles & Clinical Practice. 4th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2001:59-87 5; Roberts HR, Monroe DM III, Hoffman M. Molecular biology and biochemistry of the coagulation factors and pathways of hemostasis. In: Beutler E, Beutler E, Lichtman MA, et al, eds. Williams Hematology. 6th ed. New York: McGraw-Hill, 2001:1409-34 17; and Roberts HR, Hoffman M. Hemophilia A and B. In: Beutler E, Lichtman MA, et al, eds. Williams Hematology. 6th ed. New York: McGraw-Hill, 2001:1639-57. 34
Image depicting the 28q region of the X chromosom...

Image depicting the 28q region of the X chromosome. Adapted from: Kazazian HH Jr, Tuddenham EGD, Antonarakis SE. Hemophilia A and parahemophilia: deficiencies of coagulation factors VIII and V. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic and Molecular Bases of Inherited Disease. 7th ed. New York, NY: McGraw-Hill; 1995:3241-67 4; Reitsma PH. Genetic principles underlying disorders of procoagulant and anticoagulant proteins. In: Coleman RW, Hirsh J, Marder VJ, et al, eds. Hemostasis and Thrombosis: Basic Principles & Clinical Practice. 4th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2001:59-87 5; Roberts HR, Monroe DM III, Hoffman M. Molecular biology and biochemistry of the coagulation factors and pathways of hemostasis. In: Beutler E, Beutler E, Lichtman MA, et al, eds. Williams Hematology. 6th ed. New York: McGraw-Hill, 2001:1409-34 17; and Roberts HR, Hoffman M. Hemophilia A and B. In: Beutler E, Lichtman MA, et al, eds. Williams Hematology. 6th ed. New York: McGraw-Hill, 2001:1639-57. 34

Quality of life! A child with hemophilia at summe...Media file 24: Quality of life! A child with hemophilia at summer camp.
Quality of life! A child with hemophilia at summe...

Quality of life! A child with hemophilia at summer camp.

Photograph depicting the application of a Velcro ...Media file 25: Photograph depicting the application of a Velcro tourniquet, followed by self-infusion of concentrate used for in-home therapy (see Image 26).
Photograph depicting the application of a Velcro ...

Photograph depicting the application of a Velcro tourniquet, followed by self-infusion of concentrate used for in-home therapy (see Image 26).

Self-infusion of concentrate used for in-home the...Media file 26: Self-infusion of concentrate used for in-home therapy (same patient as in Image 25).
Self-infusion of concentrate used for in-home the...

Self-infusion of concentrate used for in-home therapy (same patient as in Image 25).

More on Factor VIII

Overview: Factor VIII
Differential Diagnoses & Workup: Factor VIII
Treatment & Medication: Factor VIII
Follow-up: Factor VIII
Multimedia: Factor VIII
References

References

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Further Reading

Keywords

factor VIII, hemophilia, von Willebrand, von Willebrand disease, von Willebrands, hemophilia A, factor 8, blood coagulation factor VIII, factor VIII coagulant activity, F8, coagulation factor VIII, F VIII-C, FVIII-C, F VIII clotting activity, FVIII clotting activity, antihemophilic globulin, blood disease, blood disorder, hematologic disorder, bleeding disorder, hemostatic system, plasma coagulation system, hemophilia B, haemophilia, hemorrhagic disorder, coagulation disorder, clotting disorder, blood clotting, coagulopathies, coagulopathy, cryoprecipitate, plasma concentrate, F8A gene, F8B gene, ERGIC53 gene, combined deficiency of FV and FVIII, PLAS+SD, Advate, DDAVP, Stimate, EACA, AMCA, Cyklokapron

Contributor Information and Disclosures

Author

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and 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, PhD 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, Southwest Medical Consultants, SC, Department of Medicine, 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

Charles S Greenberg, MD, Director of Thrombosis and Transglutaminase Research Laboratory, Professor, Departments of Pathology and Medicine, Division of Hematology/Oncology, Duke University Medical Center
Charles S Greenberg, MD is a member of the following medical societies: American Society of Hematology and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Pharmacy Editor

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

Managing Editor

Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center
Ronald A Sacher, MB, BCh, MD, FRCPC 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, 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: Abbott Grant/research funds Speaking and teaching; Genzyme Honoraria Consulting; Amgen Honoraria Speaking and teaching; Ortho Biotech Honoraria Speaking and teaching

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 Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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