Bleeding disorder resident survival guide
Bleeding disorder Resident Survival Guide |
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Overview |
Causes |
Diagnosis |
Management |
Do's |
Don'ts |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Javaria Anwer M.D.[2]
Synonyms and keywords: Coagulopathy, platelet disorders, bleeding
Overview
The bleeding disorder can be due to coagulopathy, platelet dysfunction, or vessel pathology. They can be genetic or acquired. History of the site, duration, associated symptoms, and a thorough physical exam to evaluate the type of bleeding (subcutaneous vs deep tissue) are essential to diagnosis. Screening tests include CBC with peripheral smear, platelet count, PT, aPTT, PFA-100. Management depends upon the type of disorder, severity, and active bleeding. Plasma exchange, fresh frozen plasma, and factor replacement are the main treatment options available. The initial clinical impression based on the baseline screening tests should direct specialized laboratory tests to save the time, effort and money.
Causes
Common causes of bleeding (bleeding disorders) are enlisted below.[1][2][3][4]
Causes of bleeding disorders | |||||||||||||||||||||||||||||||||||||||||||||||
Platelet disorders | Coagulopathy | Vessel/ Supporting tissue defect | |||||||||||||||||||||||||||||||||||||||||||||
Acquired | Genetic | ❑Aging ❑Corticosteroid use | |||||||||||||||||||||||||||||||||||||||||||||
❑ Myeloproliferative disorders ❑ Uremia | |||||||||||||||||||||||||||||||||||||||||||||||
Genetic | Acquired | Prothrombotic | |||||||||||||||||||||||||||||||||||||||||||||
❑ Prohemorrhagic liver diseases ❑ Vitamin K deficiency | |||||||||||||||||||||||||||||||||||||||||||||||
Hemorrhagic disorders | Hypercoaguable disease | ||||||||||||||||||||||||||||||||||||||||||||||
❑ Factor VIII deficiency (Hemophilia A) ❑ Factor IX Deficiency (Hemophilia B) | |||||||||||||||||||||||||||||||||||||||||||||||
Diagnosis
The algorithm illustrates the approach to the diagnosis of bleeding disorder.[1][5][6][7][8]
Abbreviations: HEENT: Head, Eyes, Ears. Nose, and Throat exam; CBC: Complete blood count; APTT Partial thromboplastin time; CMP: Comprehensive metabolic panel; LFTs:Liver function tests
Boxes in red signify that an urgent management is needed.
History ❑ Demographics: Patient age, gender,and race to screen for inherited disorders.
❑ Past medical history: For the underlying disease. History of blood or blood components transfusion. Childhood history of epistaxis, bleeding post-circumcision, and umbilical stump bleeding may suggest an inherited bleeding disorder. | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Physical exam Appearance of the patient Petechie, bruises, or hemorrhages ❑ Vital signs: Temperature; heart rate (tachycardia with regular pulse may demonstrate hypovolemia); respiratory rate, blood pressure (hypotension); and oxygen saturation may be low due to anemia. | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Screening Labs ❑ CBC with differential | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Soft tissue hematoma, deep internal hemorrhage, hemarthrosis | Superficial cutaneous or mucous membrane bleeding | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
PT normal, aPTT prolonged | PT prolonged, aPTT normal | PT prolonged, aPTT prolonged | Low platelet count | Normal platelet count | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
❑ Factor VIII, factor IX, factor XI, factor XII, and prekallikrien deficiency ❑ Von Willebrand Disease | ❑ Check thrombin time | Check PFA-100 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Prolonged | Normal | Prolonged closure time | Normal | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
High clinical suspician | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Management
The following tables illustrates the treatment to common bleeding disorders.[9][10][11][12][13][14][15][16][17][18]
Disorder | Labs | Treatment |
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Factor VII deficiency |
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Hemophilia A and Hemophilia B |
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Factor X deficiency |
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Hemophilia C |
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Von Willebrand disease |
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Microangiopathic hemolytic anemia |
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Glanzmann's thrombasthenia |
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Bernard-Soulier syndrome |
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Thrombocytopenia of pregnancy |
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SLE | ||
Liver disease | ||
Antithrombin III deficiency |
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Protein C deficiency |
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Protein S deficiency |
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Do's
- A study by Wahlberg et al. demonstrated that the patient's perception of his/her own bleeding may be understated or exaggerated, so labs vital in the assessment of bleeding disorders.[20]
- The initial clinical impression based on the baseline screening tests should direct specialized laboratory tests to save the time, effort and money.[21]
Don'ts
- It is important to not miss the genetic workup for a diagnosed bleeding disorder.
- A bleeding disorder may present as anemia so common signs/ complaints should not be missed.
References
- ↑ 1.0 1.1 Bashawri LA, Ahmed MA (May 2007). "The approach to a patient with a bleeding disorder: for the primary care physician". J Family Community Med. 14 (2): 53–8. PMC 3410146. PMID 23012146.
- ↑ George JN (April 2000). "Platelets". Lancet. 355 (9214): 1531–9. doi:10.1016/S0140-6736(00)02175-9. PMID 10801186.
- ↑ Al-Fawaz IM, Gader AM, Bahakim HM, Al-Mohareb F, Al-Momen AK, Harakati MS (May 1996). "Hereditary bleeding disorders in Riyadh, Saudi Arabia". Ann Saudi Med. 16 (3): 257–61. doi:10.5144/0256-4947.1996.257. PMID 17372424.
- ↑ Bick, Rodger (2002). Disorders of thrombosis and hemostasis : clinical and laboratory practice. Philadelphia: Lippincott Williams & Wilkins. ISBN 978-0397516902.
- ↑ Hayward CP (2005). "Diagnosis and management of mild bleeding disorders". Hematology Am Soc Hematol Educ Program: 423–8. doi:10.1182/asheducation-2005.1.423. PMID 16304414.
- ↑ Blanchette VS, Sparling C, Turner C (April 1991). "Inherited bleeding disorders". Baillieres Clin Haematol. 4 (2): 291–332. doi:10.1016/s0950-3536(05)80162-3. PMID 1912663.
- ↑ Favaloro, Emmanuel J. (2002). "Clinical application of the PFA-100®". Current Opinion in Hematology. 9 (5): 407–415. doi:10.1097/00062752-200209000-00004. ISSN 1065-6251.
- ↑ Harrison, Paul (2005). "The role of PFA-100R testing in the investigation and management of haemostatic defects in children and adults". British Journal of Haematology. 130 (1): 3–10. doi:10.1111/j.1365-2141.2005.05511.x. ISSN 0007-1048.
- ↑ Powell JS (April 2009). "Recombinant factor VIII in the management of hemophilia A: current use and future promise". Ther Clin Risk Manag. 5 (2): 391–402. doi:10.2147/tcrm.s4412. PMC 2697540. PMID 19536318.
- ↑ Kozek-Langenecker SA, Afshari A, Albaladejo P, Santullano CA, De Robertis E, Filipescu DC, Fries D, Görlinger K, Haas T, Imberger G, Jacob M, Lancé M, Llau J, Mallett S, Meier J, Rahe-Meyer N, Samama CM, Smith A, Solomon C, Van der Linden P, Wikkelsø AJ, Wouters P, Wyffels P (June 2013). "Management of severe perioperative bleeding: guidelines from the European Society of Anaesthesiology". Eur J Anaesthesiol. 30 (6): 270–382. doi:10.1097/EJA.0b013e32835f4d5b. PMID 23656742.
- ↑ Gopinath R, Sreekanth Y, Yadav M (September 2014). "Approach to bleeding patient". Indian J Anaesth. 58 (5): 596–602. doi:10.4103/0019-5049.144664. PMC 4260306. PMID 25535422.
- ↑ Meeks SL, Batsuli G (December 2016). "Hemophilia and inhibitors: current treatment options and potential new therapeutic approaches". Hematology Am Soc Hematol Educ Program. 2016 (1): 657–662. doi:10.1182/asheducation-2016.1.657. PMC 6142469. PMID 27913543.
- ↑ Napolitano M, Siragusa S, Mariani G (March 2017). "Factor VII Deficiency: Clinical Phenotype, Genotype and Therapy". J Clin Med. 6 (4). doi:10.3390/jcm6040038. PMC 5406770. PMID 28350321.
- ↑ Hayward CP, Pai M, Liu Y, Moffat KA, Seecharan J, Webert KE, Cook RJ, Heddle NM (April 2009). "Diagnostic utility of light transmission platelet aggregometry: results from a prospective study of individuals referred for bleeding disorder assessments". J Thromb Haemost. 7 (4): 676–84. doi:10.1111/j.1538-7836.2009.03273.x. PMID 19143930.
- ↑ Uprichard, James; Perry, David J. (2002). "Factor X deficiency". Blood Reviews. 16 (2): 97–110. doi:10.1054/blre.2002.0191. ISSN 0268-960X.
- ↑ Bertina RM, Broekmans AW, Krommenhoek-van Es C, van Wijngaarden A (February 1984). "The use of a functional and immunologic assay for plasma protein C in the study of the heterogeneity of congenital protein C deficiency". Thromb. Haemost. 51 (1): 1–5. PMID 6547008.
- ↑ Faioni EM, Franchi F, Asti D, Sacchi E, Bernardi F, Mannucci PM (1993). "Resistance to activated protein C in nine thrombophilic families: interference in a protein S functional assay". Thromb Haemost. 70 (6): 1067–71. PMID 8165605.
- ↑ Brown, D. L.; Kouides, P. A. (2008). "Diagnosis and treatment of inherited factor X deficiency". Haemophilia. 14 (6): 1176–1182. doi:10.1111/j.1365-2516.2008.01856.x. ISSN 1351-8216.
- ↑ Zauber NP, Stark MW (May 1986). "Successful warfarin anticoagulation despite protein C deficiency and a history of warfarin necrosis". Ann. Intern. Med. 104 (5): 659–60. PMID 3754407.
- ↑ Wahlberg T, Blombäck M, Hall P, Axelsson G (October 1980). "Application of indicators, predictors and diagnostic indices in coagulation disorders. I. Evaluation of a self-administered questionnaire with binary questions". Methods Inf Med. 19 (4): 194–200. PMID 7432180.
- ↑ Bick, Rodger (2002). Disorders of thrombosis and hemostasis : clinical and laboratory practice. Philadelphia: Lippincott Williams & Wilkins. ISBN 978-0397516902.