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Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017
| Contributor Information
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Image courtesy of Martin Oeggerli | Visuals Unlimited.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
Blood coagulation occurs after primary hemostasis and reinforces the platelet plug. Bleeding disorders can involve deficiencies in specific coagulation factors, including factor VIII (FVIII; hemophilia A), factor IX (FIX; hemophilia B), factor XI (FXI; hemophilia C), factor XII (FXII or Hageman factor), and factor XIII (FXIII or fibrinogen stabilizing factor).[1] Deficiency of von Willebrand factor (vWF), a multimeric glycoprotein that binds to FVIII, can lead to von Willebrand disease (vWD).[2]
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
During the initial evaluation, a patient with a history of bleeding should be evaluated for a blood disorder. Obtaining a complete family history may alert the physician to the possibility of an inherited coagulation disorder, such as hemophilia A, hemophilia B, or vWD.[3,4]
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Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
vWD is the most common inherited clotting disorder in humans. It involves disruptions in normal processing of primary and secondary hemostatic events[5] and is associated with delayed-onset bleeding, such as may occur after surgical procedures.[2]
Hereditary vWD is divided into type 1 (partial quantitative vWF deficiency), type 2 (qualitative vWF deficiency), and type 3 (total vWF deficiency), with type 2 being further divided into subtypes 2A, 2B, 2N, and 2M on the basis of the characteristics of the dysfunctional vWF. The pseudo or platelet form of vWD is not included in these three types but is also a hereditary disorder.
Image courtesy of Wikimedia Commons.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
Type I vWD is inherited in an autosomal dominant manner.[6] It commonly presents as unusually heavy menorrhagia or increased bleeding during minor procedures (eg, a tooth extraction).[7,8] As a hereditary disorder, it may be contrasted with acquired causes of bleeding, such as an acquired inhibitor of FVIII or FIX. Drugs such as penicillin are capable of causing acquired FXIII inhibition.
Image courtesy of Medscape.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
vWD also exists in an acquired form.[9-11] The slide shows some of the findings that may be associated with this form.
Image courtesy of Kirill Zdorov | Dreamstime.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
A 45-year-old man presents to the hospital with heavy bleeding after a cholecystectomy. Prothrombin time (PT) is 13 sec, partial thromboplastin time (PTT) is 30 sec, and hemoglobin concentration, hematocrit, and platelet counts are all within normal limits.
Which of the following deficiencies is this patient most likely to have?
FVIII deficiency
FIX deficiency
FX deficiency
FXI deficiency
FXII deficiency
FXIII deficiency
Image courtesy of Rob3000 | Dreamstime.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
Answer: F. FXIII deficiency.
FXIII is the last part of the common coagulation cascade. Deficiency of this coagulation factor leads to defective crosslinking of fibrin. The clot lysis assay is used to help confirm the diagnosis. As in Glanzmann thrombasthenia, a patient can present with normal PT and PTT and still have a bleed.
Image courtesy of Roger Asbury | Dreamstime.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
The patient in the slide was diagnosed as having FXIII deficiency. Which of the following can effectively treat this disorder?
Cryoprecipitate
Fresh frozen plasma (FFP)
FXIII concentrate
Plasmapheresis, if an inhibitor is present
All of the above
Image courtesy of Dreamstime | ibreakstock.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
Answer: E. All of the above.
FXIII concentrate is commonly given for acute bleeding episodes; however, cryoprecipitate and FFP are also potential treatments for FXIII deficiency. If an FXIII inhibitor is present, the use of plasmapheresis may be considered, in addition to medications that ramp down the inhibitor (eg, steroids, intravenous [IV] immunoglobulin [IVIG], cyclophosphamide, or rituximab).
Image courtesy of Stuart Monk | Dreamstime (left).
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
A 39-year-old woman presents with bleeding after a full-term vaginal delivery. Her PT is 18 sec, and her PTT is 30 sec.
Which of the following is least likely to be responsible for this patient's bleeding disorder?
FVII deficiency
Anticoagulation with warfarin
Vitamin K deficiency
Liver cirrhosis
FXIII deficiency
Image courtesy of Medscape.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
Answer: E. FXIII deficiency.
FXIII deficiency is typically associated with a normal PT and a normal PTT and thus is unlikely in this case. The other potential sources of bleeding mentioned are all possibilities in this patient.
Image courtesy of Medscape / photo Sam Shlomo Spaeth.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
A 28-year-old man undergoes routine blood screening by his primary care physician and is found to have a PTT of 102 sec. He has no history of previous bleeding issues or events and no family history of bleeding disorders. He is otherwise asymptomatic.
Which of the following is most likely to be responsible for this patient's elevated PTT?
FVIII deficiency
FIX deficiency
FX deficiency
FXI deficiency
FXII deficiency
Image courtesy of Medscape by Sam Shlomo Spaeth.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
Answer: E. FXII deficiency.
This patient's presentation is most plausibly explained by a deficiency of FXII (ie, Hageman factor), a condition that is common among Azhkenazi Jews. FXII deficiency carries only a minimal risk of significant bleeding and need not be corrected.
Image courtesy of Halina Valiushka | Dreamstime (left).
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
A 68-year-old woman has a past medical history that is significant for diabetes mellitus type 2, hypertension, chronic obstructive pulmonary disease (COPD), myocardial infarction (MI) with several stent placements, and end-stage renal disease (ESRD) with hemodialysis. She has missed her past five hemodialysis sessions and presents with bleeding after a fall from an ambulance, during which she sustained trauma to her knee.
Which of the following treatments is most likely to be effective in reversing this patient's bleeding?
Administration of desmopressin
Administration of vitamin K
Administration of IV iron
Immediate plasmapheresis
Iron chelation therapy
Image courtesy of Medscape.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
Answer: A. Administration of desmopressin.
This patient probably has uremia-related bleeding. Treatment with desmopressin would raise vWF levels, and this would help stem the bleeding. The other treatment options listed would not help with the current bleeding episode.
Image courtesy of Iofoto | Dreamstime.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
A 32-year-old woman with a history of bleeding after minor procedures (eg, tonsillectomy) and events (eg, finger cuts) presents to the emergency department with a complaint of ongoing bleeding during menses. Her PTT is found to be elevated. The patient's mother had a long-standing bleeding history and had uterine atony during her pregnancy, as well as epistaxial bleeding.
Which of the following is the best test to order at this time?
Platelet function test
vWF antigen (vWF:Ag) level
Inhibitor screening test
FVIII level
FIX level
Image courtesy of Medscape by Sam Shlomo Spaeth.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
Answer: B. vWF antigen (vWF:Ag) level.
The most likely diagnostic possibility in this patient is vWD, especially in view of her family and personal history. Accordingly, the best next option is assessment of the vWF:Ag level, which serves as a screening test for vWD. Hemophilia is a less likely diagnosis in this scenario, in that it involves bleeding of the deeper tissues and joints and less mucocutaneous bleeding. Therefore, assessment of the FVIII level is not the optimal test choice.
Image courtesy of Medscape.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
A 42-year-old man experiences moderate bleeding during a minor surgical procedure (mole removal). A complete blood count (CBC) reveals a decreased platelet count (95,000/µL). A vWF multimer test shows an absence of large multimers.
Which of the following treatments is contraindicated for this patient?
FVIII/vWF
Aminocaproic acid
Tranexamic acid
Hydroxyurea
Desmopressin
Image courtesy of Medscape.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
Answer: E. Desmopressin.
This patient has type 2B vWD, and thus, desmopressin should not be used. Desmopressin can cause a transient drop in platelet counts, and historically, it has been either contraindicated or used only with extreme caution in this setting.
Image courtesy of Alexander Raths | Dreamstime.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
Which of the following treatments should be considered for a patient with newly diagnosed vWD?
Hydroxyurea, if the patient has vWD related to essential thrombocythemia
Desmopressin
FVIII/vWF replacement
Antifibrinolytics (eg, tranexamic acid)
All of the above
Image courtesy of Wikimedia Commons.
Nonhemophilia Bleeding Disorders: The Coagulation Factor Factor
Roman Leonid Kleynberg, MD; Sameer Kabir, BS | April 13, 2017 | Contributor Information
Acquired Hemophilia: Control the Bleeding, Eliminate the Inhibitor
Acquired hemophilia is a rare but life-threatening bleeding disorder caused by the development of autoantibodies directed against a plasma coagulation factor. Do you know how to manage the acute bleeding and eradicate the inhibiting autoantibody?Slideshows, February 2017
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Contributor Information
Authors
Roman Leonid Kleynberg, MD
University of California, Irvine Medical Center
Chao Family Comprehensive Cancer Center
Department of Hematology/ Oncology
Orange, CA
Disclosure: Roman Leonid Kleynberg, MD, has disclosed no relevant financial relationships.
Sameer Kabir, BS
Bachelor of Science in Bioengineering
University of California, Berkeley
Disclosure: Sameer Kabir has disclosed no relevant financial relationships.
Goodeve A et al. von Willebrand disease. Pagon RA et al, eds. GeneReviews® [Internet]. Seattle, WA: University of Washington; 2014 Jul 24. Available at: https://www.ncbi.nlm.nih.gov/books/NBK7014.
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