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Hemostatic System - general information. Normal hemostatic system vessel wall circulating blood platelets blood coagulation and fibrynolysis Platelets disc-shape cells arise from megakariocytes in bone marrow 1/3 of platelets are sequestrated in the spleen

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hemostatic system general information
Hemostatic System - general information
  • Normal hemostatic system
    • vessel wall
    • circulating blood platelets
    • blood coagulation and fibrynolysis
  • Platelets
    • disc-shape cells
    • arise from megakariocytes in bone marrow
    • 1/3 of platelets are sequestrated in the spleen
    • other 2/3 circulate for 7-10 days
    • are responsible for primary hemostasis
    • normal platelet count : 150-450 G/l
bleeding diathesis
Bleeding Diathesis
  • Is caused by inherited or acquired defects of
    • vessel wall
    • platelets number and/or function
    • coagulation system
  • Is charcterised by
    • spontaneous bleeding
    • extensive bleeding after minimal trauma
platelets disorder
Platelets Disorder
  • Disorder of platelet number
    • thrombocytopenia
    • thrombocytosis
  • Disorder of platelet function
disorder of platelets function
Disorder of platelets function
  • defects of platelet adhesion
    • inherited: vonWillebrandt’s disease , Bernard-Soulier syndrome
    • acquired: uremia
  • defects of platelet aggregation
    • inherited: Glantzmann’s thrombasthenia
    • acquired: dysproteinemia, drug ingestion (ticlopidin)
  • defects of platelet release
    • inherited: grey-platelet, Hermansky-Pudlak,Chediac-Higashi syndr.
    • acquired: cardiopulmonary bypass, myeloproliferative disorders, drugs
thrombocytosis
Thrombocytosis
  • Thrombocytosis resulting from myeloproliferation
    • essential thrombocythemia
    • polycythemia vera
    • chronic myelogenous leukemia
    • myeloid metaplasia
  • Secondary (reactive) thrombocytosis
    • systemic inflammation
    • malignancy
    • iron deficiency
    • hemorrhage
    • postsplenectomy
thrombocytopenia 1
Thrombocytopenia (1)

Decreased marrow production of megakariocytes

  • congenital disorders
    • thrombocytopenia with absent radii (TAR)
    • Fanconi’s anemia
    • May-Hegglin anomaly
  • acquired disorders
    • marrow infiltration with malignant cells
    • marrow fibrosis
    • aplastic and hypoplastic anemias ( idiopathic, drugs, toxins )
    • deficiency states ( vitamin B12, folate, iron )
    • paroxysmal nocturnal hemoglobinuria
thrombocytopenia 2
Thrombocytopenia (2)

Splenic sequestration of circulating platelets

  • splenic enlargement due to tumor infiltration
  • splenic enlargement due to portal hypertension
thrombocytopenia 3
Thrombocytopenia (3)

Increased destruction of circulating platelets

  • congenital disorder
    • Wiscott-Aldrich syndrome, Bernard-Soulier syndrome
  • acquired disorders

nonimmune destruction

    • vascular prostheses, cardiac valves
    • DIC
    • hemolytic-uremic syndrome/thrombotic thrombocytopenic purpura
    • sepsis

immune destruction

    • idiopathic thrombocytopenic purpura ( ITP ) acute and chronic
    • drug-induced thrombocytopenia
    • chronic autoimmune disorders
    • infection (HIV)
    • malignancies
platelets disorder signs and symptoms
Platelets Disorder - signs and symptoms
  • asymptomatic if platelets count > 50 G/l
  • onset of bleeding after trauma - immediate
  • site of bleeding - superficial: skin, mucous membranes, nose, genitourinary tract
  • physical finding - petechiae, ecchymoses
idiopathic thrombocytopenic purpura 1
Idiopathic Thrombocytopenic Purpura (1)
  • the most common cause of isolated thrombocytopenia
  • autoimmune disease with antiplatelet antibodies and shortened platelets life span
  • forms of ITP

acute ITP

    • children (90% of pediatric case of immune thrombocytopenia)
    • preceded by viral infection
    • spontaneous recovery within 4-6 weeks in 60% of patients

chronic ITP

    • 20-40 years
    • women predominance F:M=3:1
idiopathic thrombocytopenic purpura 2
Idiopathic Thrombocytopenic Purpura (2)
  • Clinical features
    • petechiae
    • ecchymoses
    • mucose membranes bleeding
    • menorrhagia
    • rare internal, intracranial bleeding
  • Diagnosis
    • platelet count <100G/l
    • bleeding time - usually normal
    • peripheral blood smear - large platelets
    • bone marrow examination - normal or increased number of megakariocytes
    • antibodies against Gp IIb/IIIa
    • shortened platelet survival
treatment of itp
Treatment of ITP
  • not necessary unless platelets count > 20G/l or there is extensive bleeding
  • corticosteroids permanent responses - 30%
    • prednisone 1mg/kg for 4-6 weeks
  • splenectomy permanent responses - 60%
  • immunosuppresive drugs
  • intravenous immunoglobulins
  • other - danazol, antiRhD
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