Bleeding Diathesis - Causes, Symptoms, Diagnosis, and Treatment

What Does Bleeding Diathesis Mean?

The term diathesis can be defined as a hereditary or acquired susceptibility of the body to diseases. Bleeding diathesis or hereditary diathesis is characterized by the body's increased tendency to bleed easily, without any injury. The bleeding is spontaneous and from the nose, mouth, or digestive tract which may sometimes be severe and life-threatening. Also, the clotting mechanism of the platelets and clotting factors fail to stop the bleeding.

What Causes Bleeding Diathesis?

The inability of the blood to clot can be attributed to any of the three main components that help the blood to clot, like platelets, clotting factors, and the blood vessels. The abnormality in them can be as follows

• Platelet Disorders - The disorders are caused due to decreased number of platelets or severe platelet deficiency. This may be contributed to aplastic anemia (the body stops producing enough new blood cells), cirrhosis (chronic liver damage), and disseminated intravascular coagulation (DIC) that occurs in certain conditions, like severe infections, injury, and cancers where the platelets and clotting factors are quickly used up, causing bleeding. Certain drugs (including Heparin, Quinidine, or Rifampin) can trigger the destruction of platelets. Some diseases, such as leukemia (cancer of the blood), HIV (human immunodeficiency virus), and thrombotic thrombocytopenic purpura (which is a rare disorder that causes blood clots in small blood vessels) also cause platelet disorder. Platelet disorders such as von Willebrand’s disease (low levels of clotting proteins), and diseases, such as leukemia (cancer of the blood) are inherited platelet disorders.

• Clotting Disorders - The decreased activity of the clotting factors produced by the liver can cause clotting disorders that may be classified as acquired that occur due to consuming anticoagulants (drugs that inhibit clottings, like Heparin, Warfarin, or Dabigatran), liver disease, like hepatitis (inflammation of the liver), vitamin K deficiency. Hereditary disorders, like hemophilia (blood doesn't clot normally) cause bleeding and decreased activity of clotting factors.

• Vascular Bleeding Disorders - Defects in blood vessels are either acquired due to vitamin C deficiency, infections and thinning of the skin or are due to hereditary disorders, such as Marfan syndrome (affects connective tissue) and hemangioma (noncancerous growth of blood vessels).

What Are the Signs and Symptoms of Bleeding Diathesis?

The symptoms of bleeding diathesis are generally related to the cause of the disorder and include

• Red spots or petechiae (tiny, purple, red, or brown spots on the skin).

• Spontaneous and easy bruising.

• Bleeding after injections or surgery.

• Heavy and prolonged menstrual bleeding.

• Excessive bleeding after dental extraction or surgery.

• Unexpected nosebleeds.

• Blood in urine, stool, or vomit.

• Bleeding gums.

• Purpura (rash or purple spots due to leak of small blood vessels into the skin).

How to Diagnose Bleeding Diathesis?

Mild cases of bleeding diathesis are difficult to diagnose.

• A detailed medical history including history of bleeding, history of bleeding in any of the family members, medications, herbal preparations, or supplements is required.

• Physical examination, especially for purpura or petechiae. The doctor may examine infants and small children for signs associated with congenital bleeding disorders.

• Laboratory screening tests, such as full blood count check platelets, coagulation proteins, and clotting factors. Tests, like bleeding time (time taken for a standardized skin puncture to stop bleeding), are effective in identifying patients with von Willebrand disease. Specific tests, like fibrinogen activity, von Willebrand factor antigen, and vitamin K deficiency tests may be performed. The prothrombin test is used to measure the time taken to generate fibrin (a protein involved in clotting). Partial or activated partial thromboplastin time helps to detect abnormalities in blood clotting.

• Genetic testing can also help to identify systemic diseases with bleeding disorders.

What Are the Complications of Bleeding Diathesis?

Failure to treat bleeding on time or prevent it can lead to complications including central nervous system hemorrhage, bleeding in the intestines, joint bleeding or pain, soft tissue bleeding, or infections. It can lead to anemia (a condition when enough blood cells are not produced by the body) leading to extreme weakness, shortness of breath, and dizziness.

How to Treat Bleeding Diathesis?

Treatment of bleeding diathesis depends on the cause and the severity of the disorder. Foremost, any underlying disease or deficiency should be treated appropriately. Other treatments may be specific to the disorder and include the following

• Supplementations - Replenishing the significant amounts of lost iron due to bleeding with iron supplements. Treating vitamin K deficiency with vitamin K supplements along with additional clotting factors, if necessary.

• Blood Transfusion - It is a valuable option to treat uncontrolled hemorrhagic conditions that involve replacing the loss of blood by receiving it from the donor, after matching the blood. In conditions, such as thrombocytopenia (reduced platelet production) platelet transfusion is a good choice of treatment. Coadministration of immune-modulating therapies, such as steroids or intravenous immunoglobulins (antibodies) helps to increase the platelet count rapidly.

• Therapeutic Plasma Exchange Therapy - It involves the replacement of the plasma in the body with a substitute. This helps to remove harmful antibodies from the plasma and also ensures the fluid balance necessary for maintaining normal plasma volume. It is usually indicated in conditions, like thrombotic thrombocytopenic purpura (a rare disorder in which blood clots in small blood vessels).

• Desmopressin - Also, known as 1-diamino-8-d-arginine vasopressin is a drug frequently used for hemodialysis patients with bleeding diathesis. The drug works by increasing Factor VIII (A essential blood-clotting protein) levels.

Conclusion:

Bleeding diathesis refers to the increased susceptibility of some people to bleeding or bruising. It may be a result of an acquired or congenital cause. Diagnosis begins with a thorough family and personal history followed by a range of laboratory tests. The management and treatment of bleeding diathesis involve collaborative efforts of an interprofessional health team who can timely diagnose the cause and provide effective treatment outcomes.