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.

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.

The main coagulogram indexes 1 Index Hypo coagulation Normo Hyper Bleeding time (by Lee-White methods), min >5 5-3 < 3 Platelets number <180 180-320 >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

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.

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

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)

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)

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)

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)

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

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

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

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.

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

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

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

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

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

Hemophilia Diagnostic criteria Hematomic bleeding sickness types Arhtropathy

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.

Haemophilia А В С factor VIII deficiency factor IX deficiency www.themegallery.com Haemophilia А В С factor VIII deficiency factor IX deficiency factor XI deficiency Company Logo

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)

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 % Company Logo

A Treatment (cryoprecipitate) Hemophilia A Treatment (cryoprecipitate) 15-20 UN/kg 1 2 Moderate 35-40 UN/kg 3 Severe . 70 UN/kg Mild Company Logo

Hemophilia B Treatment (cryopLASMA) 15-20 ml/kg 1 2 Moderate 35-40 ml/kg 3 Severe . 70ml/kg Mild Company Logo

The main bleeding types Petechial-ecchymosis Hematoma Mixed (Petechia & Hematoma) Vasculatic Angiomatosic

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

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