Hemostasis

Anlagen:

• Immune Thrombocytopenic Purpura (ITP)

1. Pathophysiology

   Anmerkungen:

   • Anti-platelet antibodies bind to platelets. Platelets get destroyed by spleen. 
   1. Anti-platelet antibodies
2. Diagnosis

   Anmerkungen:

   • Diagnosis of exclusion
   1. Diagnosis of exclusion
3. Presentation

   Anmerkungen:

   • - Asymptomatic - Minimal bruising to severe bleeding
   1. Asymptomatic
   2. Mild to severe bleeding
4. Investigations

   Anmerkungen:

   • - CBC and retics: thrombocytopenia - PT and PTT normal- Test for HIV and HCV (increased risk factors)- Blood smear: decreased platelets, giant platelets- Bone marrow biopsy: increased number of megakaryocytes
   1. CBC and retics
      1. Thrombocytopenia
   2. PT and PTT
      1. Normal
   3. Bone marrow biopsy
      1. Increased number of megakaryocytes
   4. Blood smear
      1. decreased and giant platelets
   5. Test for HIV and HCV
5. Treatment

1. Pathophysiology
   1. Can't breakdown vWF multimers
      1. Congenital (ADAMST13)
      2. Acquired
2. Presentation
   1. Thrombocytopenia
      1. Purpura
      2. Hx of bleeding
      3. Bruising
   2. MAHA
      1. (L) haptoglobin
      2. (H) indirect bili
      3. Retics
      4. (H) LDH
   3. Renal failure
   4. Fever
   5. Neurologic symptoms
      1. Confusion
3. Investigations
   1. CBC and blood smear
      1. Schistocytes
      2. (L) platelets
      3. Indirect bili
      4. (H) LDH
      5. (L) haptoglobin
   2. PT amd PTT
      1. Normal
   3. Coomb's test
      1. Negative (non-immune)
   4. Renal fxn
      1. Creatinine
      2. Urea
4. Diagnosis
   1. Thrombocytopenia
   2. MAHA
5. Treatment
   1. Plasmapheresis
      1. + steroids
   2. Plasma transfusion