1. Disorder of hemostasis. Haemophylia. Trombocytopenias
Disorder of hemostasis. Haemophylia. Trombocytopenias. Willebrand’s disease. Thrombohemorrhagic syndromes. Etiology. Pathogenesis. Diagnostics. Clinical pattern. Principles of treatment. Typical changes in oral cavity in agranulocytosis and leukaemias. The role of a dentist in early diagnostics and prophylaxis N.Z. Yarema, ass.proff.

2. Hemorrhagic diathesis – group of diseases, which characterized by bleeding sickness due to hemostasis disorders Hemostasis or haemostasis is a complex process which causes the bleeding process to stop.

3. The main coagulogram indexes1
Index
Hypo
coagulation
Normo
Hyper
Bleeding time (by Lee-White methods), min
>5
5-3
< 3
Platelets number
<180
>320
Platelet adhesiveness
<23
23-44
>45
Time of plasma recalcification, sec
>120
120-60
<60
Heparin tolerance test, min
>11
11-8
<8
Prothrombin index, %
<80
80-100
>100
U-factor
Procorventin (VII factor), %
Fibrinogen, g/l
<2
2-4
>4

4. Hemorrhagic diathesis
Bleeding diathesis is an unusual susceptibility to bleeding (hemorrhage) due to a defect in the system of coagulation. Several types are distinguished, ranging from mild to lethal.

5. Hemostatic mechanism The classic hemostatic mechanism include:
Platelets adhesion
Coagulation factor
Vascular response

6. The main bleeding sickness types
Angiomatosic (hemorrhage due to vascular dysplasia, teleangiectasia; the main clinical criteria is relapsing bleeding without hemorrhage in skin, subcutaneous and other tissue; nose bleeding are most often, dangerous and massive)

7. The main bleeding sickness types
Petechia-ecchymosis (is usually localized to superficial sites such as the skin and mucous membranes, wich often combined with menorrhagia, nose bleeding, gumms bleeding; rare – with gastrointestinal bleeding or brain hemorrhage; it is small, painless, provoked by simple action like skin cleaning, measure of blood pressure etc; pinch test is positive)

8. The main bleeding sickness types
Hematomic (massive, deep, painful; bleeding may occur anywhere. The most common sites of bleeding are into joints (knees, ankles, elbows), into muscles, from the gastrointestinal tract, cause of the bleeding can be intramuscular injection; characterized by early postoperative & posttraumatic bleeding)

9. The main bleeding sickness types
Mixed (Petechial & Hematomic) (combined features of both types, but there are some difference: in contrast to hematomic - bleeding are into joints very rare, mostly it is located in subcutaneous, retroperitoneal, mesenteric, subserous intestinal layer or into internal organ);in contrast to petechial-ecchymosic bleeding - hemorrhagic syndrome characterized by large bruise)

10. The main bleeding sickness types
Vasculatic (hemorrhage due to inflammatory changes of small vessels, the main cause are immune disorders or infectious agent)

11. Classification of Hemorrhagic diathesis
Thrombocytopenia (decrease in the number of platelets)
- Idiopathic thrombocytopenic purpura (ITP)
- Thrombotic thrombocytopenic purpura (TTP)
- Heparin-induced thrombocytopenia (HIT)
- Hemolytic-Uremic Syndrome
Coagulopathies (disorders of bleeding and coagulation)
Disseminated intravascular coagulation
Hemophilia
Hemophilia A
Hemophilia B (Christmas disease)
Hemophilia C
Von Willebrand disease
Vasculitis

12. THE CLOTTING MECHANISM
INTRINSIC
EXTRINSIC
Collagen
Tissue Thromboplastin
XII XI
VII IX
VIII X V
FIBRINOGEN
(I)
PROTHROMBIN THROMBIN
(II)
(III)
FIBRIN

15. Thrombocytopenia
Thrombocytopenia (or -paenia, or thrombopenia in short) is the presence of relatively few platelets in blood.
Generally speaking a normal platelet count ranges from 180,000 and 320,000 per mm3.
Signs and symptoms
Often, low platelet levels do not lead to clinical problems; rather, they are picked up on a routine full blood count. Occasionally, there may be bruising, nosebleeds and/or bleeding gums.
It is vital that a full medical history is elicited, to ensure the low platelet count is not due to a secondary process. It is also important to ensure that the other blood cell types red blood cells, and white blood cells, are not also suppressed.

16. Deacrease of platels Diagnostic criteria Thrombocytopenia
Petechial-ecchymosic
Deacrease of platels

18. Platelet disorders Decreased production of platelets
Thrombocytopenia =Reduced platelet number
Causes
Decreased production of platelets
vitamin B12 or folic acid deficiency
Decreased platelet survival
Immunologic or Nonimmunologic etiology
Sequestration- Hypersplenism
ameliorated by splenectomy
Dilutional
Massive transfusions

19. Thrombotic Microangiopathies
Thrombotic thrombocytopenic Purpura (TTP)
Hemolytic-Uremic syndrome (HUS)

20. Petechia
small red or purple spot on the body, caused by a minor hemorrhage (broken capillary blood vessels).

24. Clotting factor abnormalities
Congenital disorders
Von Willebrand disease –MC with minimal bleeding
Factor VIII Deficiency - Hemophilia A or Classic Type
Factor IX Deficiency – Hemophilia B
Acquired disorders
Vit. K deficiency =Due to deficient carboxylation of factors II, VII, IX &X
Oral anti-coagulants
Coumarin derivatives = warfarin – inhibit Vit. K factors
Liver diseases ↓ synthesis of factors

25. Hemophilia Diagnostic criteria Hematomic bleeding sickness types
Arhtropathy

26. von Willebrand's Disease
Essentials of Diagnosis
Family history with autosomal dominant pattern of inheritance.
Prolonged bleeding time, either at baseline or after challenge with aspirin.
Reduced levels of factor VIII antigen or ristocetin cofactor.
Reduced levels of factor VIII coagulant activity in some patients.
Symptoms and Signs
von Willebrand's disease is a common disorder affecting both men and women. Most cases are mild. Most bleeding is mucosal (epistaxis, gingival bleeding, menorrhagia), but gastrointestinal bleeding may occur. In most cases, incisional bleeding occurs after surgery or dental extractions. von Willebrand's disease is rarely as severe as hemophilia, and spontaneous hemarthroses do not occur (except in the rare type III). The bleeding tendency is exacerbated by aspirin. Characteristically, bleeding decreases during pregnancy or estrogen use.

28. Haemophilia А В С factor VIII deficiency factor IX deficiency
Haemophilia А В С
factor VIII deficiency
factor IX deficiency
factor XI deficiency
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29. Forms
Haemophilia A - factor VIII deficiency, "classic haemophilia" (X-linked)
Haemophilia B - factor IX deficiency, "Christmas disease" (X-linked)
Haemophilia C - factor XI deficiency (Ashkenazi Jews, autosomal recessive)
The unrelated type 1 and type 2 von Willebrand disease (vWD)

30. Classification (F VIII C level)
Mild form 1 2
F VIII C level 1-5 %
Moderate
form 3
F VIII C level less than 1 % .
Severe form
F VIII C level 5-15 %
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31. A Treatment (cryoprecipitate)
Hemophilia
A Treatment (cryoprecipitate)
15-20 UN/kg 1 2
Moderate
35-40 UN/kg 3
Severe .
70 UN/kg
Mild
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36. Hemophilia B Treatment (cryopLASMA)
15-20 ml/kg 1 2
Moderate
35-40 ml/kg 3
Severe .
70ml/kg
Mild
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39. The main bleeding types
Petechial-ecchymosis
Hematoma
Mixed (Petechia & Hematoma)
Vasculatic
Angiomatosic

40. Screening tests for bleeding disorders
Abnormality detected
Blood count and film
Anaemia, leukaemia, disseminated
intravascular coagulation
Platelet count
Thrombocytopenia
Activated partial thromboplastin time
Deficiency of all coagulation factors
except VII, especially follows VIII and IX;
heparin
Prothrombin time
Deficiency of factors I, II, V, VII, and X;
warfarin
Thrombin time or fibrinogen
Hypofibrinogenaemia or dysfibrinogenaemia; heparin; fibrin degradation products
Bleeding time
Test of platelet-vessel wall interaction

41. Thank you for attention!