Principles of clinical laboratory Diagnostics of Hemostatic disorders basic

Clinical guidelines, determine the direction and amount of research

A detailed study of all parts of the hemostatic system is very cumbersome, laboriously, It requires a large investment of time and the blood of the test and does not meet the objectives of rapid diagnosis.

Clinical experience shows, that in most cases there is no need in such a comprehensive study and that the correct assessment of characteristics of existing pathology diagnosis and monitoring of treatment may be made by the shortest route by using a small number of logic circuit arranged in assays. However, these problems can not be solved by carrying out any one standard coagulation, needs situational selection tests, related to group affiliation existing pathology, its appearance and clinical features, the use of various methods of treatment.

In clinical practice, the study of the hemostatic system often involves the following objectives:

• Diagnosis of hemorrhagic diseases and syndromes.
• Detection of acute and chronic DIC, the definition of its kind, severity, development phase, monitor the effectiveness of treatment.
• Identification of hemostasis disorders in newborns and the determination of their nature.
• Recognition of hemostasis disorders (hemorrhagic vasculitis, erythema nodosum and other, glomerulonephritis, hemolytic-uremic syndrome, Gasser's disease or, and t. d.) in systemic and organ mikrotrombovaskulitah, monitoring of treatment of patients.
• Diagnosis and identification of the pathogenesis of thrombophilia, control of their prevention and treatment.
• Investigation of the role of hemostasis disorders in the development of local (regional, organ) hemodynamic, ischemia, thrombosis, heart attack; correction of the identified changes.
• Identification of hemostasis disorders and their correction is controlled in violation of hemorheology and circulatory failure.
• Detection, prevention and correction of hemostasis Ying- -invasive study of the circulatory system, extracorporeal circulation, gemodialize, plasmapheresis and hemosorption, prosthetic heart valves and blood vessels, etc.. d.
• Monitoring the function of organs and tissues, synthesizing coagulation factors (endothelium, liver and others.).
• Control of the hemostatic system during treatment with anticoagulants, fibrinolytic, Antiplatelet agents, hemostatic drugs, as well as other drugs, have adverse effects on the system.
• Monitoring the action transfusion and infusion therapy on the hemostatic system, as well as the effectiveness of substitution-infusion therapy all the above violations of hemostasis, (including in the therapeutic use of clotting factor concentrates).

However, the variety of purposes is strictly determines the need for a differentiated selection of the necessary tests in a given situation.

Clinical guidelines and recognition of hemorrhagic processes thrombohemorrhagic.

If there is bleeding, you must first find out from patients, whether the hemorrhage is strictly local in nature (eg, Only nasal or stomach bleeding) or at the same time there are several different hemorrhagic manifestations (bruising, hypermenorrhea, hemorrhage at the injection site and so. d.).

In the first case, often due to bleeding local destructive, tumor or vascular (Aneurysm, arteriovenous shunts and others.) processes, as well as violations of hemodynamics (hypertonic disease, atherosclerosis, heart failure).

Violations in humoral hemostasis wherein either not detected, or are secondary. Only under certain hemorrhagic diathesis for possible long monosemeiotic. In such cases, dominated by nasal bleeding hereditary telangiectasia and in some hypermenorrhea thrombocytopathy (violations of platelet aggregation).

In explaining the history, it is important first of all to establish prescription hemorrhagic diathesis (he began in his early childhood, or recently emerged).

In the first case, the most probable genesis of hereditary diseases, Second - Purchase, secondary.

The presence of bleeding in the patient's next of kin (parents, grandparents, brothers, sisters, etc.. d.) It confirms the likelihood of hereditary origin of the disease and in many cases to determine the type of inheritance.

So, eg, bleeding in the family only in males on the maternal side (t. it is. sign, X-linked) characterized mainly for hemophilia A or B, hence the need to study the internal mechanism of coagulation.

When suddenly emerged bleeding without prior even small hemorrhagic history should think about secondary (symptomatic) its genesis (Among these forms predominate DIC, the appearance in the blood clotting factor inhibitors immune and secondary thrombocytopathy). From Delayed hemorrhagic diathesis itself at any age having thrombocytopenic purpura and hemorrhagic vasculitis, and bleeding on telangiectasia.

To determine the possible genesis of secondary bleeding it is important to carefully examine a history of previous exposure and disease pathogens, as well as the background pathological processes. In recent years, as more frequent cases of bleeding, associated with the intake of drugs (antykoahulyantov, antiplatelet and others.), which should be taken into account when collecting history.

An objective study of the patient clarifies the nature and severity of bleeding (value, soreness, tension, localization of the hemorrhage and so on.), and the presence of previous and background diseases, that could be the cause of them (blood disease, liver, sepsis, shock, trauma). Thoroughly researched skin vessels, mucosas, fundus and, if necessary, performed endoscopy of the stomach and intestines, bronchoscopy, and other types of research.

It is important study of the properties of the skin and musculoskeletal system, defeat is often seen with hemophilia (hemarthrosises, hematoma, psevdoopuholy). When there are a number thrombocytopathy albinism (Hrzhmanskogo disease-Pudlaka etc..), giperelastoz skin ("Rubber" skin), the weakness of the ligamentous apparatus (laxity of the joints, recurred dislocation), heart valve prolapse (often combined with telangiectasia and angiohemophilia), which leads to misdiagnosis failure of the left atrioventricular valve and unjustified appointment of acetylsalicylic acid, even more disturbing hemostasis; bone changes (lack radius syndrome, fingers doubling, arachnodactyly etc.. d.).

In many trombotsitonatiyah from early childhood abruptly disturbed immune system and there is persistent recurrence of various hard occurring infections (Viskotta syndrome - Aldrich and others.).

Identify all of these features greatly help diagnosis, orients in the right direction laboratory study of the hemostatic system, as well as other systems - blood, hepatolyenalnoy, immune and t. d.

Determining the severity and type of bleeding

The overall pattern is, that hemophilia A and B (deficiency of factor VIII and IX), as well as the deficit factors VII and XIII appear much more severe bleeding, than thrombocytopathia, which are characterized by often so-called small form krovopodtechnaya pathology.

Angiogemofilija (von Willebrand disease) It occupies an intermediate position between the moderate forms of hemophilia and thrombocytopathies. However, even when there may be the hardest thrombocytopathy (deep anemiziruyuschie) uterine bleeding, due to which doctors sometimes have to resort to hysterectomy; also possible hemorrhage in the brain or retina, which significantly aggravates the course of the disease and its prognosis.

Important is to define the type of hemophilia.

According to the adopted classification, distinguish five types of bleeding.

Gematomny type of bleeding

Gematomny type with extensive, intense and extremely painful deep bleeding into joints (with the development of multiple acute hemarthrosis and chronic arthropathy), muscle, subcutaneous tissue, bones (osteoporosis and fractures, psevdoopuholyamy) and t. d.

As a result, patients become disabled because of the defeat of the musculoskeletal system, manifested strains, restriction of motion in joints, contractures, muscle atrophy, etc.. d. These patients are also observed long delayed (resulting sometimes in a few hours) bleeding from cuts, operations, tooth extraction, etc.. d. It is also possible spontaneous renal, gastrointestinal haemorrhage and other.

This type of bleeding characteristic for hemophilia, and identifying it in males immediately allows direct laboratory testing to determine the status of the internal mechanism of coagulation.

Increasing the activated partial thromboplastin time at normal rates of thrombin and prothrombin time confirms the diagnosis of haemophilia, then only be via the adjustable tests to determine the form of this disease.

Thus, based on a number of clinical guidelines (inheritance, male patient, gematomny type of bleeding) It manages to reduce all of the hemostatic system to study some simple tests very quickly and accurately diagnose.

When sudden onset of painful and stressful hematomas in adult patients, mostly women (after childbirth or injury), without destruction of the joints and bones can be expected or appearance in the blood of immune inhibitors against factor VIII (more) or IX (much less), or the presence of a rare form of hemophilia - autosensibilization stromal own erythrocytes. In the latter case, the recurrent painful hematomas are located mainly in the hips and pelvis, intradermal wherein stroma own erythrocytes causes very painful inflammatory reaction haemorrhagic, which confirms the diagnosis.

Petechial-spotted type of bleeding

Petechial-spotted type of bleeding is characterized by the appearance of the skin (especially in the areas of clothing friction) petechiae and small painless superficial ecchymosis in combination with hypermenorrhea, epistaxis, often increased capillary fragility and bruising at the injection site, hemorrhages of the gums, etc.. d.

Painful bruising while absent, musculoskeletal Intact.

This type of bleeding is typical for all types of thrombocytopenia and thrombocytopathy, therefore the laboratory study should be primarily aimed at determining the bleeding time, platelet count and adhesively-aggregation function. Only in some rare coagulation disorders (Hypo- and disfibrinogenemiyah, small deficiency of factors V and X) It can be expressed in the same mild bleeding.

Mixed-krovopodtechno gematomny type of bleeding

Mixed krovopodtechno gematomny-type is not the sum of the first two characteristics of the above types, and qualitatively different from both the first, and the second. With this type of bleeding is dominated by easy bruising appearing as spontaneous (mostly in the area of ​​the articular folds), and caused minor traumatization - palpation, measuring blood pressure, inaektsiyami.

These hemorrhages are slightly larger and deeper, than with petechial-spotted type of bleeding, sometimes slightly painful. The remaining hemorrhagic manifestations such as, as in the second type of bleeding, their background may arise hematoma, most often in the lumbar region, in perinephric tissue (imitating paranephritis), at the root of the peritoneum (with a secondary paresis of intestines), as well as the peritoneum or subserous in the intestinal wall.

There are nasal, renal and gastro-intestinal bleeding. Bleeding into joints are missing or are very rare, chronic hemarthrosis and other disorders of the musculoskeletal system, dominant type of bleeding during gematomnom, no.

Of inherited diseases, this type of bleeding is most often characteristic of severe angiohemophilia, the diagnosis of which in the first place and should be directed to inspection, but it can also be observed in these rare diseases, expressed as a factor VII deficiency, V and XIII. In all these cases, bleeding is observed for life, It occurs in early childhood.

The most frequently observed in the clinic secondary, acquired form mixed bleeding, which in most cases are due to acute or subacute trombogemorragichesky (ICE) syndrome. At this pathology easy occurrence of bruising or petechiae are often combined to form a large (as spots and bands) areas of hemorrhagic skin soaking, gematomami, bleeding into the peritoneal and pleural cavities, profuse uterine, gastrointestinal and epistaxis.

Hemorrhagic syndrome combined with pulmonary insufficiency, kidney, liver or adrenal, often - with venous thrombosis (especially in places catheterization and injection). A similar type of bleeding, but without the functions of internal organs failure and thrombosis observed with an overdose of anticoagulants and fibrinolytic agents. Of these, the most difficult to diagnose a hemorrhagic syndrome, due to the reception of indirect anticoagulants, as history data is not always possible to establish the fact of their oral (in violation of the mind and the artificial playing hemorrhagic syndrome on the basis of hysteria, for poisoning rat poison, which contains coumarin, and t. d.).

Vasculitis magenta type of bleeding

Vasculitis magenta type of bleeding, observed in infectious and septic or immune (gemorragicheskiy vasculitis, or Henoch's disease, erythema and various others.) microvascular damage, characterized by the emergence of clearly delineated, often with a seal, and sometimes with a central nekrotizirovaniem, symmetrical rash with hemorrhagic impregnation of its elements, after which long remained pigmentation.

simultaneously may result in renal vascular (protein, erythrocytes, cylinders in the urine), intestines (abdominal pain crises, enterorrhagia), light (breathlessness, perïvaskwlyarnaya infiltration) and other organs, and joints - poliartralgichesky syndrome, resembling rheumatism.

These forms have similar manifestations to DIC, which are often combined, in connection with the study of the hemostatic system when they found the same laboratory signs of intravascular coagulation (paracoagulation positive tests, etc.. d.).

Mikroangiomatozny type of bleeding

Mikroangiomatozny type of bleeding is characterized by the formation of telangiectasia and (or) mikroangiom and arteriovenous shunts (lung, stomach and other organs) with recurrent, sometimes very persistent bleeding from them.

Sometimes this pathology is combined with a deficit of von Willebrand factor, in this case there is a combination with signs petechial-spotted krovopodtechno and mixed-species bleeding gematomnogo. In all other cases, telangiectasia primary platelet disorders, microcirculatory hemostasis and blood coagulation are not found - samples for capillary fragility, duration of bleeding, platelet function and coagulation tests of general parameters are not violated, but there are long-term and difficult to stop bleeding of any one location.

Among hereditary hemorrhagic diathesis prevail thrombocytopathia (In total), Hemophilia A and B, angiogemofiliya and teleangiэktazii. Other forms of hemorrhagic diathesis are rare or very rare.

Of the acquired types of bleeding dominated immune thrombocytopenia, DIC, deficiency of factors of the prothrombin complex (in lesions of the hepatobiliary system and receiving indirect anticoagulants). Recently, also imitating the form of psychogenic genesis.