Haemorrhagic Disorders = Disorders characterized by abnormal bleeding tendency due to hemostatic defect

Haemostatic mechanisms 1. VASCULAR - Local vasoconstriction. - Initiation of intrinsic pathway (factor XII). - Activation of extrinsic pathway (tissue thromboplastin). - Promotion of platelet adhesion & aggregation (release of vWF & ADP). 2. PLATELET - Platelet adhesion. - First aggregation (release of ADP). - Second aggregation (release of thromboxane A2 & ADP). 3. COAGULATION

prothrombin (Factor II) active thrombin fibrinogen (Factor I) HAEMORRHAGIC DISORDERS BLEEDING PLATELET Thrombo cytopenia TAR Fanconi ITP Malignancy Drugs Infections Thrombo asthenia Glanzman vonwillebrand Drugs Uremia Hereditary Acquired VASCULAR COAGULATION DISORDER COAGULATION Hereditary Allergic Metabolic Infection (SBE) Collagenic Scurvey Acquired Hemophilia A,B,C -DIC -Hgic disease of newborn -Liver disease -Anticoagulants fibrin

Hereditary

Acquired

Tests for coagulation & bleeding 1. Tourniquet test 2. Platelet count 3. Platelet function 4. Bleeding time (4-8 min) 5. Clotting time (8-10 min) Bleeding time time elapses between the formation of a small cut and stopping of bleeding from the cute reflects hemostatic function of platelets normal value 2-5 mins - increase in thrombocytopenic purpura method * blotting paper *based on blood, lymph & immune system module definitiom 6. T.T. (15-20 sec) 7. PT (12-14 sec) 8. PTT (24-40 sec) 8. Thromboplastin generation test
Correction of thromboplastin activity of plasma of patients by addition of: Plasma VIII, XI hemophilia A Serum XI, IX hemophilia B Both hemophilia C

Clotting/coagulation time time elapses between the start of bleeding and the formation of clot blood clotting mechanism 5-10 mins deficiency of coagulation factor

characteristic

etiology

pathogenesis

C/P

BLEEDING DISORDER PLATELET DISORDER VASCULAR DISORDER ITP HSP * mostly children (2-8 y/o) - in children (2-8 y/o) - thrombocytopenia - Male : Female = 3:2 - shortened life span platelet - antiplatelet Ab in plasma - BM megakaryoctes - absence of 1ry disease - spleen: normal size - autoimmune platelet antibodies hypersensitivity vasculitis, of IgG or IgM type involving small BVs of skin, - 50% cases are preceded by viral joints, gut & kidney infection ( rubella, measle, EBV, viral *offending Ag may be a respiratory infection) preceding non specific URTI or a food Ag virus combined with platelet making i) Skin Rash them antigenic stimulate - recurrent attacks of non production of anti -platelet Ab itchy urticarial & MP lesion autoimmune reaction causing; which change into petechial i) platelet destruction & purpuric rash in all cases ii) megakaryocyte productive - characteristically involve capacity the buttocks & extensor surface of LL *roles of spleen = major site for i) antiplatelet Ab production ii) Abdominal manifestation ii) destruction of these sensitized (results from petechial platelets hemorrhage and/or edema in bowel wall) ACUTE ITP: (85-90% of cases) - severe crampy abd pain - mostly children (2-8 y/o) referred to umbilicus ass - both sexes are equally affected with vomiting - self limited within 6 months - most imp complication = - Hx of preceding viral infection intususception! - present with abrupt spontaneous/ induced bleeding in; iii) Arthralgia &/or arthritis i) skin : purpura, petichae, - affects big joints, swollen ecchymosis & tender (asymmetric, on anterior surface - transitory, mono/poly of legs & over body prominence) arthirits *DDX: RS ii) mucous mbrane : conjunctiva, buccal mucosa, gums, nose iv) Renal manifestation iii) hematuria & GIT bleeding - similar pict to APSGN iv) CNS bleeding (HTN, oliguria, hematuria + azotemia) but NORMAL CHRONIC ITP: (10% of cases) serum complement - thrombocytopenia > 6months

Hemophilia A - X-linked recessive trait ( females are carrier, males are diseased)

COAGULATION DISORDER Hemophilia B Von Willbrand Disease

DIC - dt widespread activation of coagulation in the circulation dt severe illness resulting in: i) consumption of some coagulation factors & platelets ii) fibrin deposition & thrombi iii) 2ry fibrinolysis - gastroenteritis + severe dehydration - shock, burn, heat stroke - septicaemia, severe infection - acute liver failure - disseminated malignancy

deficiency or functional abnormality of Factor VIII

severity

severe moderate

% of factor VIII activity <1 % 1-5 %

clinical manifestation

mild

5-25 %

spontaneous hemorrhage gross bleeding following mild to moderate trauma gross bleeding ONLY following moderate to severe trauma or surgery

- males are predominantly affected - +ve family history in 70% of cases - recurrent haemorrhages for hours/days after minor trauma or spontaneously - prolonged bleeding after circumcision - in NB : intracranial hemorrhage

Inv

Rx

BT : prolonged CT, PT, PTT : normal Peripheral blood picture: - thrombocytopenia - severe case : normocytic normochromic anemia (blood loss) BM exam : - megakaryocte :N or with /no budding ANA : rule out SLE Coombs test (autoimmune) mild cases: - mild bruising, no MM bleeding, platelet > 50K observation, avoid trauma severe cases: 1. Prednisone - antiplatelet Ab production - phagocytosis of platelet in spleen 2. High dose of IV Immunoglobulin (IVIG) - antiplatelet Ab production - blocks the receptor of phagocytes 3. IV AntiD, for Rh+ve 4. Platelet transfusion *temporary effect * in; - severe uncontrolled lifethreatening bleeding from any site - active intra-cranial bleeding 5. Plasma pheresis in refractory case *temporary effect 6. Splenectomy, in - as an emergency in acute severe ITP w intracranial hemorrhage - chronic ITP not responding to Rx 7. Immunosuppressive therapy *last resort in chronic ITP not respond to other measures - endoxan / 6-mercaptopurine

BT, CT : normal Torniquet test : +ve in 25% pt Platelet count & Fx: normal Serum complement : normal Serum Ig A : in 50% cases Abd U/S: suspect intussesception 1. Symptomatic treatment to control abdominal & arthritic manifestation 2. Prednisone, in severe cases 3. Specific treatment for complication