Sana with rash and easy Bruising

Sana with rash and easy Bruising

1. Hemostais

   • The three steps are
     • 1- vascular constriction
     • 2- platelet plug
     • 3- coagulation

2. Physiology of Coagulation

   • Extrinsic pathway → begins with trauma to the vascular wall & surrounding tissues.
   • Intrinsic pathway → begins in the blood itself.

3. Bleeding Disorders

   • Vascular bleeding disorders
     • 1-Congenital defects of the vessel wall Ehlers- Danlos syndrome
     • 2-Senile purpura: defect in the subcutaneous tissue supporting the bvs
     • 3-Scurvy vitamin C deficiency
     • 4- Septic Vasculitis Infections
     • 5-Henoch-Schonlein Purpura
   • Platelets disorders
     • Number :Thrombocytopenia (ITP)
       • Incidence
         • in adults is approximately 66 cases per 1,000,000 per year
         • in children is 50 cases per 1,000,000 per year
       • etiology
         • immune system mistakenly attacking and destroying platelets
       • Clinical manifestations
         • excessive bruising (purpura)
         • Bleeding from the gums or nose
         • Blood in urine or stools
         • petechiae
         • epistaxis
       • CHILDHOOD VS ADULT
         • Chronic ITP (adult)
         • persists longer than 6 months without a specific cause.
         • Acute ITP (childern)
         • young children (2 to 6 years old)
         • symptoms may follow a viral illness
         • symptoms usually disappear in less than six months
       • Management
         • Treatment in children
           • do not require treatment, and thrombocytopenia resolves spontaneously.
         • Treatment in adults
           • Corticosteroids such as prednisone
             • cataracts, high blood sugar, increased risk of infections and Osteoporosis
           • IVIG
             • headache, vomiting and low blood pressure
           • Thrombopoietin receptor agonists
             • headache, dizziness, nausea or vomiting, and an increased risk of blood clots
         • Surgical care:
           • splenectomy
             • serious post-surgical complications, and not having a spleen permanently increases your susceptibility to infection
             • the surgical removal of the spleen.
             • In ITP, the antibody-coated platelets are often removed from circulation by the spleen.
               • Theoretically, if the spleen is removed, the platelets will remain in the blood stream.
               • The spleen can also be the site of antibody production. Therefore removing the spleen may reduce the amount of anti-platelet antibodies in addition to removing the antibody-coated platelets.
         • SIDE EFFECTS
       • pathogenesis
         • an abnormal autoantibody, usually immunoglobulin G (IgG) with specificity for one or more platelet membrane glycoproteins, binds to circulating platelet membranes.
           • Autoantibody-coated platelets induce Fc receptor-mediated phagocytosis by mononuclear macrophages
             • mononuclear macrophages in the red pulp destroy immunoglobulin-coated platelets
     • Function: Disorders of platelet function
     • Acquired: Aspirin- uremia (RF)-MPD Inherited : von Willebrand Disease
   • Coagulation disorders
     • – Congenital: – hemophilia A – Factor VIII (8) _ Intrinsic pathway – Hemophilia B –Christmas disease -Factor IX(9) _ Intrinsic pathway – Von willebrand diseasen
     • – Acquired : – liver disease

4. investigation

5. DEMOGRAPHY

   • Sana , 28-Years Old, Female

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