Haemorrhagic diathesis means an increased tendency to often profuse spontaneous or traumatic bleeding. A haemorrhagic diathesis can therefore mean bleeding from even the smallest wound, after tooth extraction, after surgery, after a blood sample or a simple injection. What are the causes and symptoms of hemorrhagic diathesis? What is the treatment?

Hemorrhagic diathesis(purple) is classified as a blood coagulation disorder. These have two faces - they can be characterized by frequent formation of extensive hematomas, bruises or ecchymosis as a result of even the smallest contusions (even without the cause noticed by the suffering person, somehow for no reason) or manifested by a tendency to bleed profusely, even from the smallest wound, after tooth extraction , after surgery, blood collection or a simple injection. In the latter case, we are talking about hemorrhagic blemishes.

Haemorrhagic blemishes may be congenital or acquired

Generally speaking, three factors influence the blood clotting process: platelets, plasma coagulation factors, blood vessel walls. Depending on which one is failing (and even all of them can fail at once), diagnosis and treatment will depend. Due to the location of the defect, doctors distinguish several types of hemorrhagic diathesis:

1. platelet bleeding disorder- caused by abnormalities or deficiency of platelets

a) Immunological thrombocytopenic purpura, also known as idiopathic thrombocytopenic purpura or Werlholf's disease, is the most common platelet disease in children. It is a condition caused by the production of anti-platelet autoantibodies for no apparent reason, which shortens the lifetime of platelets.

b) genetically determined

  • Fanconi anemia - is a type of congenital anemia. The disease affects the bone marrow, as well as the kidneys, heart and is associated with a tendency to develop cancer (leukemia)
  • TAR syndrome - is a rare, genetically determined syndrome of birth defects characterized by a bilateral absence of a radius bone (one of the forearm bones), a low platelet count (thrombocytopenia), and other defects.
  • Wiskott-Aldrich syndrome - belongs to the groupprimary immunodeficiencies. Its symptoms include frequent recurring infections, bloody diarrhea and skin blemishes
  • Gaucher disease - the disease leads to dysfunction of the liver and spleen, as well as skeletal and nervous systems

c) other thrombocytopenic bleeding disorders:

  • infectious diseases (rubella, mononucleosis), other infections
  • bone marrow aplasia (aplastic anemia)
  • leukemia or other cancerous growth in the bone marrow
  • after medication
  • after transfusions of blood products
  • DIC, hemolytic microangiopathies, congenital cyanotic heart defects
  • allo and neonatal autoimmune thrombocytopenia

2. plasma hemorrhagic diathesis(coagulopathies) - result from a deficiency of blood coagulation factors in the plasma

a) congenital:

  • von Willebrand disease - is the most common congenital plasma disease
  • hemophilia A and hemophilia B
  • deficiencies of other factors: fibrinogen, part XI, part X, part V, part VII, fibrinogen

b) acquired

  • bleeding due to vitamin K deficiency
  • liver disease
  • DIC (Diffuse Intravascular Coagulation)

3. vascular hemorrhagic diathesis(vasculopathies) - are the result of abnormal structure of blood vessels.

a) Schönlein-Henoch disease - is the most common vascular disease in children

b) congenital vascular defects

  • Rendu-Osler-Weber disease
  • Ehlers-Danlos syndrome

c) other hemorrhagic vascular defects:

  • drug-induced: sulfonamides, penicillins, salicylates, NPZ
  • allergies
  • childhood infectious diseases
  • giardiasis
  • malnutrition
  • scurvy
  • Cushing's disease
  • orthostatic, mechanical

Bleeding disorders - symptoms

Plasma hemorrhagic blemishes manifest:

Every day it also manifests itself in such minor inconveniences as bleeding gums while brushing the teeth or heavy bleeding during menstruation.

  • recurring intramuscular hemorrhages
  • recurrent intra-articular bleeding
  • late traumatic bleeding

Vascular and platelet hemorrhagic blemishes manifest as:

  • petechiae on the skin and mucous membranes
  • tendency to bruise and bruise
  • recurrent heavy nosebleeds with no apparent local cause
  • prolonged heavy periods
  • prolonged bleeding aftertooth extraction and other injuries

There are no intramuscular and intraarticular bleeds in the case of vascular and platelet bleeding.

This is a serious and he alth-threatening condition as it causes unnecessary blood loss. Even these small, everyday symptoms of greater bleeding tendency than in others, a doctor should be consulted to find the cause of the disease, treat it, and above all - to be able to deal with a situation when bleeding, easy to stop for others, is prolonged excessively. In extreme situations, e.g. in the case of extensive injuries, it can even be life-threatening because the haemorrhage cannot be stopped.

Bleeding disorder - diagnosis

It is important whether these abnormalities have occurred since childhood, did they appear later in life (when?), Or if someone in the family suffered from a similar ailment. You will also need to answer questions about illnesses and medications you have taken, and look for the relationship between these events and the onset or worsening of the disease. However, the final diagnosis must be based on very detailed, complicated and time-consuming laboratory analyzes.

If a hemorrhagic diathesis is suspected, coagulation tests are performed: platelet count, bleeding time, protombin time (PT), activated partial thromboplastin time (APTT), thrombin time (TT),

Haemorrhagic diathesis - treatment

Patients suffering from a bleeding diathesis in the asymptomatic period do not require treatment, but must avoid any injuries or overloading the joints. Periodic blood tests are necessary. When the concentration of factors promoting blood clotting decreases, they are given substitutes.

Where to go for help

In the event of an injury, immediately put on a cold pressure dressing, immobilize the damaged area and transport the patient to the hospital. Here, treatment consists of the administration of fresh blood or blood products containing the missing plasma factor (e.g. in hemophilia - globulin). Treatment of patients suffering from acquired blood clotting disorders consists - in compliance with the above principles - first and foremost in combating the disease that caused them.

Category:

Cardiology