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HEMORRHAGIC DISEASES

HEMORRHAGIC DISEASES. Prof. Dr. Shahenaz M. Hussien. Haemorrhagic Diasthesis Defects in blood coagulation usually give abnormal coagulation tests and usually have abnormal coagulation time.

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HEMORRHAGIC DISEASES

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  1. HEMORRHAGIC DISEASES Prof. Dr. Shahenaz M. Hussien

  2. Haemorrhagic Diasthesis • Defects in blood coagulation usually give abnormal coagulation tests and usually have abnormal coagulation time. • On the other hand, purpuras usually have normal coagulation time, abnormal bleeding time; and abnormal Hess tourniquet test. Diagnosis of Bleeding Diathesis -Present history: rash following infection, abdominal and joint pain. -Family History: For evidence of inheritance of any bleeding tendencies. -Past History: Excessive, prolonged bleeding of the surgical intervesions as tonsillectomy, circumcision or tooth extraction. -Physical Examination: General condition, type and distribution of the rash, fever, organ enlargement, lymph glands.

  3. Laboratory tests for screening : *Hemostasis at small vessel level : Tourniquet test (Hess test) *Full blood picture (including platelets count). *Blood coagulation : • Early stage: coagulation time or partial thromboplastin time • Late stage: - One stage prothrombin time -Thromboplastine generation test. -Specific factor assay -Bleeding time.

  4. Purpura • Purpura is a purplish hemorrhagic rash. The characteristic lesions are multiple spontaneous pinpoint hemorrhages into the skin or mucous membranes. • Purpura (Petechiae) occur in crops not raised, not blanched by pressure. • According to Platelet Count, purpuras can be classified into: *THROMBOCYTOPENlC PURPURAS (with decreased platelet count) Decreased platelet production (low megakaryocytes in bone marrow . Congenital -Megakaryocytic thrombocytopenia with absent radius (T.A.R). -Constitutional pancytopenia (Fanconi anemia). -Marrow infiltration (e.g. congenital leukemia). -Thrombopoietin deficiency.

  5. Acquired: -Megakaryocytic aplasia (idiopathic - drugs). -Aplastic anemia (idiopathic - drugs - toxin - irradiation). -Marrow infiltration (leukemia, lymphoma and metabolic disorders). -Nutritional deficiency (vitamin B12 - folic acid). -Renal failure. Immune: • Neonatal: Isoimmune thrombocytopenia, maternal immune thrombocytopenic purpura, • Systemic lupus eyrthematosis

  6. Non immune • Disseminated intravascular coagulopathy (DIC) • Infections. • Drug induced. • Hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. • Hypersplenism *Non thrombocytopenic purpura (with normal platelet count Qualitative platelet disorders (platelet function defect) • Acquired thrombocytopathies -Drug induced as in Aspirin or other drugs -Systemic disorders as in uremia and. -Defective small blood vessels (vascular bleeding) -Immune complex vasculitis (Henoch Schonlein purpura). -Infections: meningococcemia, DIC. -Scurvy.

  7. Idiopathic thrombocytopenic purpura Etiology:- • Virus particles attach to platelets provoking immunologic response. • Role of the spleen : • Sequestration of damaged platelets • Formation of antibodies against platelets Clinical picture:- • The onset is abrupt with history of preceding viral infection 1-2 weeks before the onset in 60-80% of cases. • Purpura: It is in the form of small pinpoint petechiae to large ecchymoses in skin of the trunk and limbs. • Bleeding: Oozing gums, epistaxis, melena or hematuria may occur, intracranial hemorrhage occurs in 1% of cases. • Anemia, if present, is only related to blood loss. • Liver and spleen are usually not enlarged

  8. It may be acute (85-90% of all cases) or chronic (10-15%). Those who have abnormal clinical or laboratory findings more than six months are considered chronic. Differential Diagnosis • In aplastic anemia, the 3 blood elements are reduced (pancytopenia) and the bone marrow shows reduced number of megakaryocytes. • In acute leukemia, other clinical manifestations are commonly present. The three blood elements are commonly reduced (pancytopenia) and the bone marrow shows bone marrow infiltration with blast cells.

  9. MANAGEMENT: - Whole fresh blood, platelet-rich plasma, packed platelets in severe bleeding. -Prednisone 2 mg/kg/ body weight until bleeding stops then taper gradually. -I.V lmmunoglobulins: It is indicated for serious bleeding specially preoperative, in corticosteroid resistant cases or in pregnancy. The usual dose of immunoglobulins is 900mg/kg over 4-8 hours in 3 consecutive days, the platelet count increase in 7-14 days after therapy. Booster doses every 2-4 weeks may be needed. -Anti D has been recently used. -Immunosuppressive drugs: Azathioprine or cyclosporin are used for resistant cases failed to respond to splenoectomy. -Plasmapharesis: In refractory cases to all other forms of therapy. -Splenoectomy: It improves 70-90% of cases, is indicated in chronic cases that are steroid resistant or dependent.

  10. Anaphylactoid Purpura (Henoch-schonlein purpura) It is a hypersensitivity vasculitis involving small blood vessles of skin, joints, gut and kidneys. Coagulation Pathways Phase I:Thromboplastin is formed by the interaction of certain coagulation factors, phospholipids, and tissue juice (which contains tissue factor). There are two pathways to the formation of thromboplastin. 1- Intrinsic Pathway: involves the activation of factors XII, XI and IX. The activated factor IX interact with factor VIII, calcium and phospholipid to activate factor X, The activated factor X interact with factor V, calcium and phospholipids and becomes the active complex that convert prothrombin to thrombin.

  11. 2- Extrinsic Pathway: involves the conversion of factor VII to activated factor VII by tissue factor then Vlla directly activates factor X. Phase II:- In this phase the prothrombin (Factor II) is converted to thrombin (activated factor II). Phase III:- Soluble fibrinogen is converted by thrombin to fibrin. HEMOPHILIAS • Hemophilia A: Deficiency of Factor VIII (antihemophilic globulin). • Hemophilia B: Deficiency of Factor IX (Christmas factor). • Hemophilia C: Deficiency of Factor XI (Plasma thormboplastin antecedent). All hemophilias produce similar clinical manifestation.

  12. Clinical features:- • Males are predominantly affected. Affection of females is rare. • Positive family history in 70% of cases (mother is asymptomatic carrier while patient's brother, maternal uncle are often affected). • Presentation: • Recurrent hemorrhages for hours or days after minor trauma or spontaneously. • Prolonged bleeding after circumcision. • Frequency and severity of bleeding are related to level of factor VIII Investigations and laboratory findings: • Prolonged clotting time. • Prolonged partial thromboplastin time (PTT): Usually > 120 seconds (normal =25-45"). • Normal prothrombin time (PT): this is a test for extrinsic coagulation. • Factor VIII assay: Reduced level. • Prenatal diagnosis: Using molecular biologic techniques on chorionic villus

  13. Treatment:- • Prevention of bleeding: Protect the hemophilic patient against any trauma. • Avoid exposure to trauma including IM injections and avoid aspirin. • Avoid surgery whenever possible and carry it out undercover of replacement therapy. • Careful dental hygiene and regular dental care. • Use pads for knees, encourage non-violent exercise. • Replacement therapy: Factor VIII (antihemophilic globulin). • Fresh frozen plasma: Every ml contains 1 Unit of factor VIII • Cryoprecipitate:Each bag contains 125 Units of factor VIII.

  14. Treatment continue • Factor VIII concentrates:available in vials containing 250 and 500 Units to be reconstituted 25ml. Doses:- • For usual hemarthrosis: 25 Units/kg/12 hours. • For hematuria and GIT bleeding: 50 Units/kg/12 hours. • For CNS bleeding: 50-75 Units/kg repeated every 8 hours. Hemophilia B • Inherited as X-Iinked recessive trait. • Due to deficiency of factor IX. Manifestations are similar to Hemophilia A. • Treated with fresh frozen plasma or factor IX concentrates. Hemophilia C -Inherited as autosomal recessive trait affecting males and females equally. -Homozygotes have clinical manifestation like moderate to severe Hemoph.A. -Heterozygotes bleed mildly.

  15. Von Willebrand Disease • Due to deficiency of von Willebrand factor (vWB). • Inheried as autosomal dominant trait. • Manifestation: BOTH bleeding + Coagulation defects. • Bleeding from skin, mucous membranes, hemarthrosis, etc. • Bleeding and clotting times are prolonged. • Defective platelet aggregation (with ristocetin assay). • Factor VIII and vWB factors are decreased. • Treatment (in severe bleeding): Cryoprecipitate and factor VIII concentrate.

  16. Consumption Coagulopathy (Disseminated Intravascular Coagulopathy ) (D.I.C.) • Certain diseases were accompanied with Severe shock, Blood stasis, Overwhelming infection especially gram-negative organisms, Acidosis and vasculitis are followed by DIC. • The diseases include purpura fulminans, meningococcemia; E. Coli infection, severe burns, gastro-enteritis with dehydration and acidosis, giant haemangioma (blood stasis.) and the haemolytic uraemic syndrome. • Widspread intravascular thrombosis and subsequent ischaemia and necrosis especially in lungs and kidneys (fibrin formation and deposition) will result in consumption of coagulation especially factors I, II, V, VIII and thrombocytopenia with secondary bleeding.

  17. Laboratory diagnosis: ↓ platelets ,↓ fibrinogen, ↓ factors II, V, VII and ↑ Fibrin split products (F.D.P.) Treatment: -Treatment of the cause. -Platelets and fresh frozen plasma transfusion. -Heparin is restricted to situations of widespread thrombosis e.g. purpura fulminans . -Exchange transfusion with fresh blood in neonates.

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