Clinical disorders - Primary hemostasis - Thrombocytopenia
16 important questions on Clinical disorders - Primary hemostasis - Thrombocytopenia
What are the four different mechanisms for the development of thrombocytopenia? Give an example per mechanism.
- decreased production of platelets: bone marrow problems
- increased production of platelets: TTP, ITP, DIS and HUS
- sequestration: hypersplenism
- dilution: massive blood transfusion
What is the mechanism of ITP (immune thrombocytopenia)?
What are the most important symptoms of ITP?
- Primary hemostasis symptoms
- Petechiae
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What are the diagnostic methods that are used for ITP?
- Platelet count (10-50)
- Analysis for anti-platelet antibodies
- Increased number of megakaryocytes (bone marrow)
What is the first line treatment for ITP?
When the platelet count is < 30, then platelets transfusion needs to be in consideration.
What is the second line treatment for ITP?
- Splenectomy prevents platelet degradation
- Immunoglobulin: coat platelets that are coated with antibodies --> spleen doesn't recognize the platelets with antibodies --> no degradation
- Thrombopoeitin agonists: stimulates production of new platelets
- Tiruximab: inhibitis production of antibodies.
The effect is hort (1 year) and the medication options are expensive. Second line treatment is only done when the patient needs surgery for example.
What is the mechanisms of TTP (Thrombotic tromopathic purpura)?
What are the symptoms of TTP?
- Fever
- Anemia due to break down of red blood cells
- icterus
- Neurological symptoms
- renal insufficiency
What are the diagnostic methods that are used for TTP?
- Hb: anemia
- Platelet count: thrombocytopenia
- Signs of hemolytic anemia:
- fragmentocytes
- increased LDH
- increased bilirubin
- decreased haptoglobin
- absence of ADAMTS13 (takes long)
What is the standard treatment for TTP?
- remove large VWF multimeres
- remove antibodies against ADAMTS13
- infusion of ADAMTS13
Corticosteroids
What is the mechanism in DIC (disseminated intravasular coagulation)?
ONLY platelets are activated
What are the symptoms of DIC?
- Multi organ failure
- microthrombi
- sepsis
What are the diagnostic methods for DIC?
- Platelet count: thrombocytopenia
- Fibrinogen deficiency
- high D-dimer
- PT and APTT prolonged (all the factors are low)
- fragmentocytes
- DIC score
What is the treatment of DIC?
- Treat the underlying cause
- supporting treatment if necessary
What is the mechanism of HUS (Hemolytic uremic syndrome)?
What are the symptoms of HUS?
- Fever
- diarrea and vomit
- no neurological symptoms
- renal dysfunction
- TTP symptoms
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