Sana with Rach and Easy bruising

Sana with Rach and Easy bruising

1. Thrombopoiesis

   • Process by which mature platelets are generated within the bone marrow.
   • Function
     • -Hemostasis -Membrane receptors -Actin, myosin & thrombosthenin -Fibrin-stabilizing factor -Growth factors
     • Hemostasis means prevention of blood loss.
       • 1-vasoconstriction
       • 2-formation of blood clot
       • 3-Blood coagulation to form a blood clot
         • mechanism

2. bleeding disorder

   • investigations
   • causes
     • thrombocytopenia: below Below 20,000 cells/ul
       • caused by:
         • 2-Decreased production: Aplastic aneamia- megaloblastic anemia-drugs- infectionHIV- leukemia
         • 1-Decreased survival: immune thrombocytopenic purpura (ITP)-drug induced (heparin- quinidine)-Dissiminated intravascular coagulopathy (DIC)- thrombotic thrombocytopenic purpura (TTP)
     • DIC:Systemic activation of coagulation factors – formation of microthrombi throughout the circulation-consumptive coagulopathy.
       • secondary to many condition: -Obstetric: MAJOR OB complications; toxemia, sepsis, abruptio placenta, retained dead fetus -Infections: Gm-, meningococcemia, fungi, Malaria -Many neoplasms; AML, adenocarcinoma -Massive tissue injury; trauma, burns, surgery
     • Heparin induced thrombocytopenia
       • IgG bind to platelet factor 4 on platelet membrane in a heparin dependent fashion leading to platelet activation platelet aggregation resulting in thrombosis.
     • 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 :(meningococcaemia, infective endocarditis, measles, rickettsia)
     • plasma abnormalities
   • types

3. Idiopathic thrombocytopenic purpura (ITP)

   • disorder that can lead to easy or excessive bruising and bleeding. The bleeding results from unusually low levels of platelets
   • types
   • pathophysiology
     • An autoantibody usually an IgG will be formed in the spleen and that antibody will be directed against certain glycoproteins on the platelet surface (llb/lib, lb/IX) . After the binding of the antibody to the platelet surface, circulatng antibody-coated platelet are recognised by the Fc receptor on splenic macrophages, it will be ingested and destroyed.
   • common risk factors
     • -Genetics:Having close relatives who have also been diagnosed with ITP can sometimes increase the risk -Gender -Age -Receiving certain vaccines. Although very rare, some vaccinations can “trigger” ITP. -Viral infections. Sometimes, after a bout with the mumps, measles or other viruses, children can get ITP.
   • signs & symptoms
     • either internal or external bleedin
       • bleeding include: Petechiae (< 3mm) Purpura (3-10 mm) Ecchymoses (> 1 cm) Hematoma Nosebleeds or bleeding from the gums Blood in urine or stool Any kind of bleeding that's hard to stop could be a sign of ITP  Menstrual bleeding that's heavier than normal
   • Diagnosis
     • CBC: low platelet count
     • bone marrow biopsy & inspiration: normal to increased megakaryocytes to compensate for the platelets loss
     • PT& PTT for coagulation cascades: normal
     • peripheral smear
   • Complications
     • -Menorrhagia -Intracranial bleeding is rare -Petechial rash -Epistaxis -gum bleeding -Easy bruising
   • management
     • -Stop using any drug that might inhibit platelet function- Drugs that suppress your immune system -Injections to increase your blood count:injection of immune globulin (IVIG) -Drugs that boost platelet production
   • treatment
     • -Steroids + Steroid-sparing agents -IVIG or Anti Rh (D)- immunoglobulin -Rituximab -Platelets transfusion
       • Treatments for Resistant Disease
         • -Other drugs. Azathioprine (Imuran, Azasan) has been used to treat ITP.
         • Removal of the spleen. (splenectomy)
           • spleen
             • function:- filter for blood as part of the immune system.- Old red blood cells are recycled in the spleen, and platelets and white blood cells are stored there. -helps fight certain kinds of bacteria that cause pneumonia
               • recommended that people with asplenia are vaccinated against Neisseria meningitidis, Haemophilus influenzae, and Streptococcus pneumoniae. AKA SHiN.

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