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Hemorrhagic diathesis

Table of contents
Hemorrhagic diathesis
Clinic of hemorrhagic diathesis
Diagnosis, treatment of hemorrhagic diathesis

Carry diseases which cornerstone the disturbances of a vascular wall and various links of system of a hemostasis causing the raised bleeding or tendency to its emergence are to hemorrhagic diathesis.

Epidemiology
On the globe about 5 million people suffer from primary hemorrhagic manifestations. Considering that secondary hemorrhages, such as the IDCS in a preagonal state, not always are also fixed, it is possible to imagine prevalence of hemorrhagic diathesis.

Etiology and pathogeny
The pathogeny of hereditary hemorrhagic states is defined by disturbance of normal haemo static processes: anomalies of megacaryocytes and thrombocytes, deficit or defect of plasma blood-coagulation factors, inferiority of small blood vessels. The acquired hemorrhagic diathesis is caused by the IDCS, immune defeats of a vascular wall and thrombocytes, toxi-infectious defeats of blood vessels, liver diseases, influences of medicines.

Classification
1. The hemorrhagic diathesis caused by defect of a platelet link
— insufficiency of quantity of thrombocytes
— functional inferiority of thrombocytes
— combination of quantitative and qualitative pathology of thrombocytes
2. The hemorrhagic diathesis caused by defect of pro-coagulants (hemophilia) — their insufficient quantity necessary for fibrin forming
— insufficient functional activity of separate pro-coagulants
— existence in blood of inhibitors of separate pro-coagulants
3. The hemorrhagic diathesis caused by defect of a vascular wall
— inborn
— acquired
4. The hemorrhagic diathesis caused by an excess fibrinolysis
— endogenous (primary and secondary)
— exogenous
5. The hemorrhagic diathesis caused by a combination of disturbances of various components of system of a hemostasis (an angiohemophilia, the IDCS and so forth)

This classification does not include all known hemorrhagic diathesis. They are more than 300. It is the scheme of the principles of classification of hemorrhagic states, observing which it is possible to rubrifitsirovat not only any of the known hemorrhagic states, but also each again found.

Classification of trombotsitopeniye assumes their division depending on the basic reason causing them. These reasons a little: the broken reproduction, the increased destruction, deposition and cultivation of thrombocytes. The reasons of trombotsitopeniye are stated below.

Thrombocytopenia owing to the broken reproduction

Thrombocytopenia owing to the increased destruction

1. Physical factors
— radiation
2. Chemical factors
— hlotiazid, cytostatics, uraemia
3. Biological factors
— tumors etc.
4. Reduction of a thrombocytopoiesis
— osteomilofibroz
5. Inborn hypoplasia of megacaryocytes
6. Avitaminosis (B12 vitamins, folic acid)

1. Immune
— medicinal allergic thrombocytopenia
— posttransfusion allergic thrombocytopenia
— at collagenoses
— at lymphoid leukoses
— Verlgof's syndrome
— isoimmune neonatal thrombocytopenia
— transimmune neonatal thrombocytopenia
— viral infections
2. Not immune
— Bernard's illness — Sulye
— Viskott's syndrome — Olridzha
— Mai's syndrome — Hegglina

Trombotsitopaty — the second group of the hemorrhagic states caused by inferiority of a platelet component of a hemostasis. It integrates the diseases which are shown qualitative inferiority of thrombocytes at safety of their quantity. She received the name of trombotsitopatiya. In recent years in classification of trombotsitopatiya there were serious changes. Their essence is that many nosological forms which characteristic was bleeding were heterogeneous. Attempts to coordinate this or that feature of functional disturbances of thrombocytes to defeat or features of development of other bodies or systems (Hermansky's syndrome — Prudlak, Chediyak — Higasha and so forth) in this plan also show a certain polymorphism. All this forced doctors to concentrate attention on specific pathology of function of thrombocytes which formed the basis.

Distinguish the following types of trombotsitopatiya:

1) a trombotsitopatiya with disturbance of adhesion of thrombocytes;
2) a trombotsitopatiya with disturbance of aggregation of thrombocytes: a) to ADF, b) to collagen, c) to Ristomycinum, d) to thrombin, e) to adrenaline;
3) a trombotsitopatiya with release reaction disturbance;
4) a trombotsitopatiya with defect of "an accumulation pool" of the released factors;
5) a trombotsitopatiya with defect of retraction;
6) a trombotsitopatiya with a combination of the above defects.

Except ascertaining of platelet defects, it is necessary to supplement diagnosis of a disease of the obligatory indication of the quantitative party of a platelet link (a hypothrombocytosis, a hyperthrombocytosis, normal quantity of thrombocytes), and also ascertaining of the accompanying pathology.

Diseases which cornerstone deficit of certain plasma blood-coagulation factors is are generalized (can be more and more correct to call them hemophilias).

Defective factor

Name of an illness

I (fibrinogen)

Afibrinogenemiya, hypofibrinogenemia, dysfibrinogenemia, deficit of the I factor

II (prothrombin)

Prothrombinopenia, deficit of the II factor

V (pro-accelerin)

Deficit of the V factor, parahemophilia, Ovren's illness

VII (proconvertin)

Deficit of the VII factor, hypoproconvertinemia

VIII (anti-hemophilic globulin)

Hemophilia And, classical hemophilia, deficit of the VIII factor

IX (Kristmas's factor)

Cristmas disease, illness. Kristmasa, deficit of the IX factor

X (Stewart — Prauer a factor)

Deficit of the X factor. An illness Stewart — Prauer

XI (predecessor of plasma thromboplastin)

Deficit of the XI factor, hemophilia With

XII (Hageman's factor)

Deficit of the XII factor, Hageman's defect

XIII (a fibrinstabiliziruyushchy factor, Lucky's factor — Loranda, a L-L factor)

Deficit of the XIII factor

(Fletcher's factor), prekallikrein

Deficit of a prekallikrein, deficit of a factor of Fletcher, deficit of the XIV factor

Kinniogen of the high molecular weight of KVMV (Fittszherald, Williams's factor, Flozhak)

Deficit of a kinniogen of VMV. Illness
Fittszheralda — Williams — Flozhak.

Classification of the vascular diseases proceeding with hemorrhagic manifestations assumes their division depending on localization of defeat of morphological structures of a vessel.

Distinguish diseases with defeat of the endothelium and a disease with defeat of a subendothelium.

Defeats of an endothelium are subdivided on inborn and acquired. The representative of inborn damages of an endothelium is the hereditary hemorrhagic teleangiectasia (an illness of Randyu — Oslera). Among the acquired defeats of an endothelium distinguish diseases of inflammatory and immune character, the damages caused by mechanical factors. The inflammatory and immune acquired hemorrhagic states are an illness Shenleyn — Genokh, nodular arteritis, an allergic granulomatosis, vasculites at infectious diseases and influences of drugs. In the same subgroup chronic inflammatory infiltrates, such as Wegener's granulomatosis, temporal arteritis, Takayasu's arteritis integrate. Among mechanical damages of an endothelium distinguish orthostatic purpura and Kaposha's sarcoma.

The hemorrhagic diseases caused by disturbances of subendothelial structures are also subdivided on inborn and acquired. Distinguish a syndrome from inborn Euler — Danlosa, an elastic pseudoxanthoma, Marfan's syndrome, and also an illness of imperfect bone formation. Integrate hemorrhagic states at an amyloidosis, senile purpura, corticosteroid purpura, simple purpura in the acquired defects of a subendothelium and hemorrhagic states at a diabetes mellitus.

Approximate formulation of the diagnosis:
1. The immune Werlhof's disease proceeding with hemorrhages on skin and on visible mucous membranes, gingival, nasal, intestinal bleedings.
2. Hemophilia And (classical hemophilia), caused by deficit of the VIII factor with hemorrhages in muscles and joints, nasal, gingival, intestinal, uterine bleedings.
3. A syndrome of diyeseminirovanny intravascular coagulation with skin petechias, bleeding of mucous membranes, a hamaturia, a pneumorrhagia.



 
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