Bleeding diathesis is a common name for a number of hematological syndromes that develop with a violation of one or another link of hemostasis (platelet, vascular, plasma). Common to all hemorrhagic diatheses, regardless of their origin, are the syndrome of increased bleeding (recurrent, prolonged, intense bleeding, hemorrhages of various localizations) and posthemorrhagic anemia syndrome. Determination of the clinical form and causes of bleeding diathesis is possible after a comprehensive examination of the hemostasis system – laboratory tests and functional tests. Treatment includes hemostatic, hemotransfusion therapy, local bleeding arrest.
Meaning
Bleeding (hemorrhagic) diathesis is a blood disease characterized by the tendency of the body to the occurrence of spontaneous or inadequate traumatic factor hemorrhages and bleeding. In total, over 300 hemorrhagic diatheses have been described in the literature. The pathology is based on quantitative or qualitative defects of one or more blood clotting factors. At the same time, the degree of bleeding can vary from small petechial eruptions to extensive hematomas, massive external and internal bleeding.
According to approximate data, about 5 million people in the world suffer from primary bleeding diathesis. Taking into account secondary hemorrhagic conditions (for example, DIC syndrome), the prevalence of is truly great. The problem of complications associated with bleeding diathesis is in the field of view of various medical specialties – hematology, surgery, intensive care, traumatology, obstetrics and gynecology, and many others.
Classification
Bleeding diathesis is usually distinguished depending on the violation of a particular hemostasis factor (platelet, coagulation or vascular). This principle is the basis of a widely used pathogenetic classification and, in accordance with it, 3 groups of hemorrhagic diatheses are distinguished: thrombocytopathies, coagulopathies and vasopathies.
Thrombocytopenia and thrombocytopathies, or bleeding diathesis associated with a defect in platelet hemostasis (thrombocytopenic purpura, thrombocytopenia in radiation sickness, leukemia, hemorrhagic aleikia; essential thrombocytemia, thrombocytopathies).
Coagulopathy, or bleeding diathesis associated with a defect of coagulation hemostasis:
- with a violation of the first phase of blood clotting – thromboplastin formation (hemophilia)
- with a violation of the second phase of blood clotting – the conversion of prothrombin into thrombin (parahemophilia, hypoprothrombinemia, Stuart Prauer disease, etc.)
- with a violation of the third phase of blood clotting – fibrin formation (fibrinogenopathy, congenital afibrinogenemic purpura)
- with a violation of fibrinolysis (DIC syndrome)
- with a violation of coagulation in various phases (Willebrand’s disease, etc.)
Vasopathy, or bleeding diathesis associated with a vascular wall defect (Randu-Osler-Weber disease, hemorrhagic vasculitis, vitamin C).
Causes
There are hereditary (primary) bleeding diathesis, manifested in childhood, and acquired, most often secondary (symptomatic). The primary forms are familial and hereditary and are associated with a congenital defect or deficiency of usually one coagulation factor. Examples of hereditary hemorrhagic diathesis are hemophilia, Glanzman’s thrombasthenia, Randu-Osler disease, Stuart Prauer disease, etc. The exception is Willebrand’s disease, which is a multifactorial coagulopathy caused by a violation of factor VIII, vascular factor and platelet adhesion.
The development of symptomatic bleeding diathesis is usually caused by the insufficiency of several hemostasis factors at once. At the same time, there may be a decrease in their synthesis, an increase in expenditure, a change in properties, damage to the vascular endothelium, etc. The causes of increased bleeding can be various diseases (SLE, cirrhosis of the liver, infectious endocarditis), hemorrhagic fevers (dengue fever, Marburg, Ebola, etc.), vitamin deficiency (C, K, etc.). The group of iatrogenic causes includes prolonged or inadequate dose therapy with anticoagulants and thrombolytics.
Most often acquired bleeding diathesis occurs in the form of disseminated intravascular coagulation syndrome (thrombohemorrhagic syndrome), complicating a variety of pathologies. Possible secondary development of autoimmune, neonatal, post-transfusion thrombocytopenia, hemorrhagic vasculitis, thrombocytopenic purpura, hemorrhagic syndrome in radiation sickness, leukemia, etc.
Symptoms
Hemorrhagic and anemic syndromes dominate in the clinic of various forms of hemostasiopathies. The severity of their manifestations depends on the pathogenetic form of bleeding diathesis and concomitant disorders. With different types of bleeding diathesis, different types of bleeding can develop.
Microcirculatory (capillary) type of bleeding occurs in thrombocytopathies and thrombocytopenia. It is manifested by petechial-spotted rashes and bruises on the skin, hemorrhages into the mucous membranes, bleeding after tooth extraction, gingival, uterine, nasal bleeding. Hemorrhages can occur with minor injury to the capillaries (when pressing on the skin, measuring blood pressure, etc.).
Hematomic type of bleeding is characteristic of hemophilia, it is possible with an overdose of anticoagulants. It is characterized by the formation of deep and painful hematomas in soft tissues, hemarthrosis, hemorrhages in subcutaneous fat and retroperitoneal tissue. Massive hematomas lead to tissue dissection and the development of destructive complications: contractures, deforming arthrosis, pathological fractures. By origin, such bleeding can be spontaneous, post-traumatic, postoperative.
Capillary-hematomic (mixed) hemorrhages accompany the course of DIC syndrome, Willebrand’s disease, are observed when the dose of anticoagulants is exceeded. They combine petechial-spotted hemorrhages and soft tissue hematomas.
Microangiomatous type of bleeding occurs in hemorrhagic angiomatosis, symptomatic capillaropathy. With these hemorrhagic diatheses, persistent recurrent bleeding of one or two localization occurs (usually nasal, sometimes gastrointestinal, pulmonary, hematuria).
Vasculitis-purple type of bleeding is noted in hemorrhagic vasculitis. It is a small-point hemorrhage, usually having a symmetrical arrangement on the limbs and trunk. After the hemorrhages disappear, residual pigmentation remains on the skin for a long time.
Frequent bleeding causes the development of iron deficiency anemia. The anemic syndrome accompanying the course of hemorrhagic diathesis is characterized by weakness, pallor of the skin, arterial hypotension, dizziness, tachycardia. With some bleeding diathesis, joint syndrome (joint swelling, arthralgia), abdominal syndrome (nausea, cramping pains), renal syndrome (hematuria, lower back pain, dysuria) may develop.
Diagnostics
The purpose of the diagnosis of hemorrhagic diathesis is to determine its form, causes and severity of pathological changes. The examination plan for a patient with increased bleeding syndrome is drawn up by a hematologist together with the attending specialist (rheumatologist, surgeon, obstetrician-gynecologist, traumatologist, infectious disease specialist, etc.).
First of all, clinical blood and urine tests, platelet count, coagulogram, feces for latent blood are examined. Depending on the results obtained and the intended diagnosis, an extended laboratory and instrumental diagnosis is prescribed (biochemical blood examination, sternal puncture, trepanobiopsy). In bleeding diathesis with immune genesis, the determination of antierythrocyte antibodies (Coombs test), antiplatelet antibodies, lupus anticoagulant, etc. is shown. Additional methods may include functional tests for capillary fragility (tourniquet, pinch, cuff test, etc.), kidney ultrasound, liver ultrasound; joint radiography, etc. To confirm the hereditary nature of bleeding diathesis, a consultation with a geneticist is recommended.
Treatment
When selecting treatment, a differentiated approach is practiced, taking into account the pathogenetic form of hemorrhagic diathesis. So, with increased bleeding caused by an overdose of anticoagulants and thrombolytics, the cancellation of these drugs or correction of their dose is indicated; the appointment of vitamin K (vikasol), aminocaproic acid; plasma transfusion. Therapy of autoimmune hemorrhagic diathesis is based on the use of glucocorticoids, immunosuppressants, plasmapheresis; with an unstable effect of their use, splenectomy is required.
In case of hereditary deficiency of one or another coagulation factor, replacement therapy with their concentrates, transfusions of freshly frozen plasma, erythrocyte mass, hemostatic therapy is indicated. In order to locally stop small bleeding, the application of a tourniquet, a pressure bandage, a hemostatic sponge, ice is practiced; nasal tamponade, etc. With hemarthrosis, therapeutic punctures of the joints are performed; with soft tissue hematomas, their drainage and removal of accumulated blood.
The basic principles of treatment of DIC syndrome include active elimination of the cause of this condition; cessation of intravascular coagulation, suppression of hyperfibrinolysis, hemocomponent replacement therapy, etc.
Complications and prognosis
The most common complication of bleeding diathesis is iron deficiency anemia. With recurrent hemorrhages in the joints, their stiffness may develop. Compression by massive hematomas of nerve trunks is fraught with the appearance of paresis and paralysis. Profuse internal bleeding, hemorrhages in the brain, adrenal glands are of particular danger. Frequent repeated transfusion of blood products is a risk factor for the development of posttransfusion reactions, hepatitis B infection, HIV infection.
The course and outcomes of hemorrhagic diathesis are different. With adequate pathogenetic, replacement and hemostatic therapy, the prognosis is relatively favorable. In malignant forms with uncontrolled bleeding and complications, the outcome can be fatal.
