Bleeding diathesis

In medicine (hematology), bleeding diathesis is an unusual susceptibility to bleed (hemorrhage) mostly due to hypocoagulability (a condition of irregular and slow blood clotting), in turn caused by a coagulopathy (a defect in the system of coagulation). Therefore, this may result in the reduction of platelets being produced and leads to excessive bleeding.[1] Several types of coagulopathy are distinguished, ranging from mild to lethal. Coagulopathy can be caused by thinning of the skin (Cushing's syndrome), such that the skin is weakened and is bruised easily and frequently without any trauma or injury to the body.[2] Also, coagulopathy can be contributed by impaired wound healing or impaired clot formation.[3]

Bleeding diathesis
Other namesHaemorrhagic diathesis, Hemorrhagic diathesis
A bleeding wound in the finger
SpecialtyHematology Edit this on Wikidata

Signs and symptoms
SymptomDisorders
Petechiae (red spots)
Purpura and ecchymoses
  • Acute leukemia[5]
  • Chronic leukemia[5]
  • Vitamin K deficiency[6]
Blood in stool
Bleeding gingiva (gums)
  • Wiskott–Aldrich syndrome[4]
  • Acute leukemia[5]
  • Chronic leukemia[5]
Prolonged nose bleeds
  • Wiskott–Aldrich syndrome[4]

Complications

Following are some complications of coagulopathies, some of them caused by their treatments:

ComplicationDisorders
Soft tissue bleeding, e.g. deep-muscle bleeding, leading to swelling, numbness or pain of a limb.
Joint damage, potentially with severe pain and even destruction of the joint and development of arthritis
Retinal bleeding
  • Acute leukemia[5]
Transfusion transmitted infection, from blood transfusions that are given as treatment.
Adverse reactions to clotting factor treatment.
Anemia
Exsanguination (bleeding to death)
Cerebral hemorrhage

Causes

While there are several possible causes, they generally result in excessive bleeding and a lack of clotting.

Acquired

Acquired causes of coagulopathy include anticoagulation with warfarin, liver failure, vitamin K deficiency and disseminated intravascular coagulation. Additionally, the hemotoxic venom from certain species of snakes can cause this condition, for example Bothrops, rattlesnakes and other species of viper. Viral hemorrhagic fevers include dengue hemorrhagic fever and dengue shock syndrome. Leukemia may also cause coagulopathy. Furthermore, cystic fibrosis has been known to cause bleeding diathesis, especially in undiagnosed infants, due to malabsorption of fat soluble vitamins like vitamin K.

Autoimmune causes of acquired coagulation disorders

There are autoimmune causes of coagulation disorders. They include acquired antibodies to coagulation factors, termed inhibitors of coagulation. The main inhibitor is directed against clotting factor VIII. Another example is antiphospholipid syndrome, an autoimmune, hypercoagulable state.

Causes other than coagulation

Bleeding diathesis may also be caused by impaired wound healing (as in scurvy), or by thinning of the skin, such as in Cushing's syndrome.

Genetic

Some people lack genes that typically produce the protein coagulation factors that allow normal clotting. Various types of hemophilia and von Willebrand disease are the major genetic disorders associated with coagulopathy. Rare examples are Bernard–Soulier syndrome, Wiskott–Aldrich syndrome and Glanzmann's thrombasthenia. Gene therapy treatments may be a solution as they involve in the insertion of normal genes to replace defective genes causing for the genetic disorder. Gene therapy is a source of active research that hold promise for the future.[9]

Diagnosis

Comparing coagulation tests
Laboratory findings in various platelet and coagulation disorders
Condition Prothrombin time Partial thromboplastin time Bleeding time Platelet count
Vitamin K deficiency or warfarin Prolonged Normal or mildly prolonged Unaffected Unaffected
Disseminated intravascular coagulation Prolonged Prolonged Prolonged Decreased
Von Willebrand disease Unaffected Prolonged or unaffected Prolonged Unaffected
Hemophilia Unaffected Prolonged Unaffected Unaffected
Aspirin Unaffected Unaffected Prolonged Unaffected
Thrombocytopenia Unaffected Unaffected Prolonged Decreased
Liver failure, early Prolonged Unaffected Unaffected Unaffected
Liver failure, end-stage Prolonged Prolonged Prolonged Decreased
Uremia Unaffected Unaffected Prolonged Unaffected
Congenital afibrinogenemia Prolonged Prolonged Prolonged Unaffected
Factor V deficiency Prolonged Prolonged Unaffected Unaffected
Factor X deficiency as seen in amyloid purpura Prolonged Prolonged Unaffected Unaffected
Glanzmann's thrombasthenia Unaffected Unaffected Prolonged Unaffected
Bernard–Soulier syndrome Unaffected Unaffected Prolonged Decreased or unaffected
Factor XII deficiency Unaffected Prolonged Unaffected Unaffected
C1INH deficiency Unaffected Shortened Unaffected Unaffected

Treatments

Consult a hematologist and have a regular blood check ups. Have an early diagnostic test for any blood disorders or blood diseases including hemophilia, hemorrhage, and sickle-cell anemia. Prothrombin time and partial thromboplastin time blood tests are useful to investigate the reason behind the excessive bleeding. The PT evaluates coagulation factors I, II, V, VII and X, while the PTT evaluates coagulation factors I, II, V, VIII, IX, X, XI and XII. The analysis of both tests thus helps to diagnose certain disorders.[10]

Blood transfusion involves the transfer of plasma containing all the necessary coagulating factors (fibrinogen, prothrombin, thromboplastin) to help restore them and to improve the immune defense of the patient after excessive blood loss. Blood transfusion also caused the transfer of platelets that can work along with coagulating factors for blood clotting to commence.[11]

Different drugs can be prescribed depending on the type of disease. Vitamins (K, P and C) are essential in case of obstruction to walls of blood vessels. Also, vitamin K is required for the production of blood clotting factors, hence the injection of vitamin K (phytomenadione) is recommended to boost blood clotting.[12]


References
  1. "Bleeding Diathesis: Causes, Symptoms, and Treatments". Doctors Health Press - Daily Free Health Articles and Natural Health Advice. 2017-06-23. Retrieved 2018-09-17.
  2. Douglas H (April 1949). "Cushing' Syndrome and Thymic Carcinoma". QJM: An International Journal of Medicine. 18 (2): 133–147. doi:10.1093/oxfordjournals.qjmed.a066529.
  3. Amin C, Sharathkumar A, Griest A (2014-01-01). "Bleeding diathesis and hemophilias". Handbook of Clinical Neurology. 120: 1045–59. doi:10.1016/B978-0-7020-4087-0.00070-X. ISBN 9780702040870. PMID 24365370.
  4. Wiskott–Aldrich Syndrome Archived 2010-12-21 at the Wayback Machine The International Patient Organisation for Primary Immunodeficiencies (IPOPI).
  5. Disorders of thrombosis and hemostasis: clinical and laboratory practice. Page Rodger L. Bick. Edition 3, illustrated. ISBN 0-397-51690-8, ISBN 978-0-397-51690-2. 446 pages
  6. Vitamin K Deficiency eMedicine. Author: Pankaj Patel, MD. Coauthor(s): Mageda Mikhail, MD, Assistant Professor. Updated: Dec 18, 2008
  7. Hemophilia Complications By Mayo Clinic staff. May 16, 2009
  8. Von Willebrand disease --> Complications By Mayo Clinic staff. Feb. 7, 2009
  9. McCain J (June 2005). "The future of gene therapy". Biotechnology Healthcare. 2 (3): 52–60. PMC 3564347. PMID 23393464.
  10. "Prothrombin Time (PT) w/INR and Partial Thromboplastin Time (PTT) Blood Test". Walk-In Lab. Retrieved 2018-09-17.
  11. "Hemorrhagic diathesis: causes, symptoms, diagnosis, treatment | Competently about health on iLive". iliveok.com. Retrieved 2018-09-17.
  12. "Treatments for bleeding disorders". Netdoctor. 2015-11-03. Retrieved 2018-09-17.
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