Hemorrhagic diseases and syndromes - a pathological condition characterized by increased bleeding as a result of failure of one or more elements of hemostasis.In this article we consider the basic signs and symptoms of hemorrhagic disease in humans.
Hemorrhagic diseases and syndromes
Signs of hemorrhagic diseases
Bleeding with hereditary hemorrhagic telangiectasia is caused by the insufficient development of the carcass of small subendothelial vascular endothelium and disability in some areas of the vascular bed.In childhood or adolescence are formed thin-walled, small angioma easily sensitive to mechanical stress;in some cases also formed arteriovenous shunts in the lungs and other organs.Inferiority mesenchymal tissue may also exhibit enhanced extensibility of the skin ( "rubber skin"), the weakness of the ligamentous apparatus (habitual dislocation, prolapse of the leaflets of the heart valve).Rarely, the disease leads to death from acute post-hemorrhagic anemia where, for example, ligation of
Symptoms of hemorrhagic diseases
disease is manifested by recurrent bleeding from telangiectasias, located mostly in the nasal cavity.Less bleeding telangiectasias on the border of the lips, mucous membranes of the mouth, throat, stomach.Number of telangiectasia (and thus the bleeding of them) increases during puberty and the age of 20-30 years.In the formation of arteriovenous shunts may develop shortness of breath, cyanosis, hypoxic erythrocytosis.The combination with prolapse of the leaflets of the heart valves (noise, arrhythmia), hypermobility of joints, sprains and other disorders caused by deficiency of tissues of mesenchymal origin, as well as deficiency of von Willebrand factor.May develop cirrhosis.
forms of hemorrhagic diseases and syndromes
There are hereditary and acquired forms of hemorrhagic diseases and syndromes.
Hereditary forms associated with genetically determined pathological changes of the vascular wall, abnormalities of megakaryocytes, platelet adhesion proteins in blood plasma and plasma clotting factors.
Acquired forms in most cases are caused by:
- lesion of blood vessels immune, immunocomplex, and toksikoinfektsionnoy dismetabolic etiology;
- defeat megakaryocytes, platelets of different etiologies;
- pathology adhesive plasma proteins and clotting factors;
- multifactorial disorders blood coagulation (DIC acute syndromes).
Classification of hemorrhagic diseases
Pathogenesis distinguish the following groups of hemorrhagic diseases and syndromes:
Conditional primary lesion of the vascular wall with the possible development of a secondary coagulation and platelet disorders.This group includes hereditary hemorrhagic telangiectasia Rendu-Osler, Ehlers-Danlos syndrome, Marfan syndrome, the syndrome of the giant Hemangiomas Kazabaha-Merritt, Shёnlyayna hemorrhagic vasculitis, purpura, erythema, hemorrhagic fevers, hypovitaminosis C and B and others.
Conditional primary lesion megakaryocyte-platelet germ.
Thrombocytopenia : redistribution of platelets and their deposition in the spleen;increased destruction (e.g., SLE, this group also includes idiopathic thrombocytopenic purpura);increased platelet consumption and blood clots (DIC, thrombotic thrombocytopenic purpura-cal);the application of certain of LS
thrombocytopathy : conditions characterized by abnormal platelets and / or violation of their functions.The most common among them - trombastenii Glyantsmanna and von Willebrand's disease.
Conditional blood clotting disorders (coagulopathy):
- hereditary coagulopathies: hemophilia A, hemophilia B, von Willebrand disease, deficiency of blood coagulation factors;
- acquired coagulopathies: vitamin K-dependent coagulopathy (arise when insufficient liver function, malabsorption of vitamin K nutritional deficiency of vitamin K, reception drugs such as coumarin), DIC, liver disease (leading to a deficiency of many factorsclotting), abnormal clotting inhibitors ( "lupus" anticoagulant, specific inhibitors of coagulation - AT, specific to certain coagulation proteins);
- violation fibrin stabilization, increased fibrinolysis, including the treatment of direct and indirect anticoagulants, fibrinolytics (streptokinase, urokinase, alteplase, etc.);
- other acquired coagulation disorders: deficiency of blood clotting factors may occur in somatic diseases (for example, amyloidosis - deficiency of factor X).
Conditional complex disorders of various parts of blood coagulation (DIC acute syndromes).
As a special group identify different forms of so-called an artificial bleeding caused by the patients themselves (for example, mental illness) by mechanical tissue trauma (naschipyvanie nasasyvanie or bruises, injuries of the mucous membranes, etc.), intake of drugs hemorrhagic action (oftenindirect anticoagulants: coumarin, fenilina, etc.), self-torture or sadism..
Bleeding at hemorrhagic diseases
following types of bleeding:
Capillary or microcirculatory (petechial-sinyachkovy) - type of bleeding is characterized by petechial rash, bruises and ecchymosis on the skin and mucous membranes.Often combined with bleeding of mucous membranes (epistaxis, menorrhagia).Perhaps the development of severe bleeding in the brain.This type of bleeding is typical for thrombocytopenia and thrombocytopathy, von Willebrand disease, deficiency of prothrombin complex factors (VII, X, V and II), some variants of hypo- and disfibrinogenemy, moderate overdose of anticoagulants.When hereditary thrombocytopathy usually mark sinyachkovy type of bleeding, petechial rash is not typical.
Gematomny type of bleeding is characterized by painful, tense hemorrhages in the subcutaneous tissue, muscles, large joints, in the peritoneum and retroperitoneal space.Hematoma can cause compression of nerves, the destruction of cartilage and bone tissue, dysfunction of the musculoskeletal system.Sometimes developing renal and gastrointestinal bleeding.Characterized by prolonged bleeding for cuts, wounds after tooth extractions and surgical procedures, often resulting in anemia.This type of bleeding is observed in some inherited disorders of blood coagulation (haemophilia A and B, marked lack Factor VII), acquired coagulopathies, accompanied by the appearance of blood VIII factor inhibitors, IX, VIII + V, and an overdose of anticoagulants, as well as hereditary trombotsitopaty withthe absence of the third factor of the plate.
Mixed capillary gematomny type of bleeding is characterized by petechial rash, sinyachkovymi, combined with a broad dense hemorrhages and hematomas.Tracking with hereditary (pronounced insufficiency of factors VII and XIII, a severe form of von Willebrand disease) and acquired (acute syndromes of the internal combustion engine, a significant overdose of direct and indirect anticoagulants) violations.
vasculitis magenta type of bleeding is characterized by erythematous or hemorrhagic (on the basis of inflammatory) rash may develop nephritis and intestinal bleeding;observed in infectious and immune vasculitis.
angiomatous type of bleeding is characterized by persistent, strictly localized and tied to local vascular pathology bleeding.Tracking with telangiectasia, angioma, arteriovenous shunts.
diagnosis of hemorrhagic diseases
General diagnosis of hemorrhagic diseases and syndromes is based on the following principles:
Determining the timing of, old, disease duration and features of its course: Start at an early age or in adults, acute or gradual development of hemorrhagicsyndrome, recent or long-term (chronic, relapsing) for his.
Identify possible hereditary genesis bleeding (with specification of the type of inheritance) or acquired nature of the disease.Clarification of the possible connection of hemorrhagic syndrome with previous pathological processes, impacts (including medical - use of drugs, vaccination, etc.) and underlying diseases (liver disease, leukemia, infectious-septic processes, trauma, shock).
Definition preferential localization, severity and type of bleeding.For example, when Rendu-Osler disease is dominated by persistent nosebleeds (often the only clinical manifestation);when platelet disease - increased tendency to bruise, uterine and nasal bleeding, hemophilia - deep bruising and bleeding into the joints.
the differential diagnosis should take into account significant differences in the prevalence of certain diseases hemorrhagic: some of them are extremely rare, while others account for the vast majority occurring in the clinical cases of bleeding.
Hereditary hemorrhagic telangiectasia
Hereditary hemorrhagic telangiectasia (hemorrhagic angiomatosis, Rendu-Osler disease) is inherited in an autosomal dominant manner.
Laboratory and instrumental methods .Frequent and heavy bleeding can lead to the development of post-hemorrhagic anemia.In the formation of arteriovenous shunts may develop erythrocytosis, increased hemoglobin concentration in the blood;X-ray examination of lungs reveal individual rounded or irregular shadows, often mistaken for a tumor.
Diagnosis is based on the identification of the family nature of the disease (but there may be sporadic cases) and detecting the typical telangiectasias and recurrent bleeding from them.