Hemorrhagic diathesis and syndromes

Hemorrhagic diathesis and syndromes are forms of pathology characterized by a tendency to bleeding.

Etiology, pathogenesis. Distinguish between hereditary (family) forms with a multi-year, beginning with childhood bleeding and the acquired forms are mostly secondary (symptomatic). Most of the hereditary forms are associated with anomalies of megakaryocytes and platelets, dysfunction of the latter either with a deficit or defect in plasma clotting factors, as well as with von Willebrand factor, less often with inferiority of small blood vessels (telangiectasia, Osler-Randu disease). Most of the acquired forms of hemorrhage are associated with the syndrome of DVS, immune and immunocomplex lesions of the vascular wall (vasculitis of Shenlaine-Henoch, erythema, etc.) and platelets (most thrombocytopenia), with violations of normal hemopoiesis (haemorrhage in leukemia, hypo- and aplastic states of hematopoiesis, radiation Diseases), toxic-infectious damage of blood vessels (hemorrhagic fevers, typhus, etc.), liver diseases and obstructive jaundice (leading to a breakdown in the synthesis of hepatocytes in blood coagulation factors), exposure to drugs that disrupt hemostasis (deaggregants, anticoagulants, fibrinolytics ) Or provoking immune disorders-thrombocytopenia (haptenic form), vasculitis. With many of these diseases, hemostasis disorders are mixed and dramatically intensified due to the secondary development of the DIC syndrome, most often due to infectious-septic, immune, destructive or tumor (including leukemia) processes.

The following groups of hemorrhagic diathesis are distinguished according to the pathogenesis: 1) caused by disorders of blood coagulability, fibrin stabilization or increased fibrinolysis, including treatment with anticoagulants, streptokinase, urokinase, defibrinizing agents (arvin, reptilase, defibraz, etc.); 2) caused by a violation of platelet-vascular hemostasis (thrombocytopenia, thrombocytopathy); 3) caused by violations of both coagulation and platelet hemostasis: a) Willebrand disease, b) disseminated intravascular coagulation (thrombohemorrhagic syndrome in) with paraproteinemia, hemoblastoses, radiation sickness, etc.; 4) caused by the primary lesion of the vascular wall with possible secondary involvement of hemostasis in the coagulation and thrombocytic mechanisms (hereditary telescoping of Osler-Randu, hemangiomas, hemorrhagic vasculitis of Shenlaine-Henoch, erythema, hemorrhagic fevers, hypovitaminosis C and B, etc.).

The special group includes various forms of the so-called neurotic, or imitating, bleeding caused by the patients themselves due to mental disorder by mechanical traumatization of the tissue (pinching or sucking of bruises, trauma to the mucous membranes, etc.), the secret reception of drugs for hemorrhagic action Most often anticoagulants of indirect action - coumarins, phenilin, etc.), self-torture or sadism on erotic soil, etc.

Less common are DVS-related thrombohemorrhagic diseases that occur with severe fever-thrombotic thrombocytopenic purpura (Moshkovich's disease) and hemolytic-uremic syndrome.

General diagnosis of hemorrhagic diseases and syndromes is based on the following main criteria:

1) determination of the timing, origin, duration and characteristics of the course of the disease (appearance in early childhood, adolescence or in adults and elderly people, acute or gradual development of hemorrhagic syndrome, recent or long-term (chronic, recurrent) course, etc. , 2) the detection, if possible, of a family (hereditary) genesis of bleeding (with specification of the type of inheritance) or acquired character of the disease; Clarification of the possible connection of the development of hemorrhagic syndrome with the previous pathological processes, effects (including medications - drugs, vaccinations, etc.) and background diseases (liver disease, leukemia, infectious septic processes, trauma, shock, etc.). ; 3) determination of the primary localization, severity and type of bleeding. Thus, with Osler-Randu disease, persistent nosebleeds prevail and are often the only ones; With pathology of platelets-bruise, uterine and nasal bleeding, with hemophilia-deep bruises and joint hemorrhages.

Types of bleeding. The hematomic type is inherent in a number of hereditary blood clotting disorders (haemophilia A and B, severe deficiency of factor VII) and coastal coagulopathy-the appearance of inhibitors of factors VIII, IX, VIII + V in the blood, overdose of anticoagulants (osteoarthritic hematomas). Characterized by painful intense hemorrhages in the subcutaneous tissue, muscles, large joints, peritoneum and retroperitoneal space (with compression of nerves, destruction of cartilage, bone tissue, impaired functions of the musculoskeletal system), in some cases, kidney and gastrointestinal hemorrhages, long anemic Bleeding from places of cuts, wounds, after tooth extraction and surgical interventions.

Capillary, or microcirculatory (petechial-sinus), the type is characterized by thrombocytopenia and thrombocytopathy, von Willebrand's disease, as well as the deficiency of factors of the prothrombin complex (VII, X, V and II), some variants of hypo-and dysfibrinogenemia, moderate overdose of anticoagulants. It is often combined with bleeding of mucous membranes, menorrhagia. Severe hemorrhages in the brain are possible.

Mixed capillary-hematoma type of hemorrhage-petechial-spotted hemorrhage in combination with extensive dense hemorrhages and hematomas. In the hereditary genesis of hemorrhage, this type is inherent in severe deficiency of factors VII and XIII, severe forms of Willebrand disease, and from acquired-is characteristic of acute and subacute forms of DIC syndrome, a significant overdose of anticoagulants. In differential diagnosis, one should also take into account the large differences in the frequency and prevalence of hemorrhagic diathesis, some of which are extremely rare, while others account for the vast majority of cases of bleeding occurring in clinical practice.

Hemorrhagic diathesis caused by disorders in the blood coagulation system. Among the hereditary forms, the deficiency of the components of factor VI 11 (haemophilia A, vWD) and factor IX (hemophilia B), 0.3-1.5% for deficiency of factors VII, X, V and XI. Very rare (single observations) forms associated with a hereditary deficit of other factors-XII (Hageman defect), II (hypop-thrombinemia), I (hypodysfibrinogenemia), XIII (deficiency of fibrin-stabilizing factor). Among the acquired forms, in addition to DIC syndrome, coagulopathies associated with deficiency or depression of factors of the prothrombin complex (II, VII, X, V), liver disease, obstructive jaundice, intestinal dysbiosis, overdose of anticoagulants-vitamin K antagonists (coumarins, phenylin and Etc.), hemorrhagic disease of newborns; Forms associated with the appearance in the blood of immune inhibitors of coagulation factors (most often antibodies to factor VIII); Bleeding due to heparinization, administration of fibro-rhinolytic drugs (streptokinase, urokinase, etc.) and defibrinating action (arvin, ancrod, defibraza, reptilase, ancistron-H).

Hemophilia. Hemophilia A and B are recessively inherited, linked to the sex (X-chromosome) disease; Sick men, women are transmitters of the disease. Genetic defects are characterized by inadequate synthesis or anomaly of factors VIII (coagulation part) - hemophilia A or factor IX - hemophilia B. Temporal (from several weeks to several months) acquired deficiency of factors VIII, less often IX, accompanied by severe bleeding, is observed in men, And in women (especially in the postpartum period, in individuals with immune diseases) due to the appearance of antibodies to these factors in the blood in high titer. Pathogenesis. At the heart of the bleeding lies an isolated disruption of the initial stage of the internal mechanism of blood coagulation, as a result of which the total coagulation time of the whole blood (including the R thromboelastogram parameter) and the more sensitive tests - autocoagulant (ACT), activated partial thromboplastin time (APTT) Etc. The prothrombin time (index) and the final stage of coagulation, as well as all parameters of platelet hemostasis (the number of platelets and all kinds of their aggregation) are not violated. Samples for the fragility of microvessels (cuff, etc.) remain normal.