Platelet Disorders

Question 1

What are the causes of thrombocytopenia?

Answer 1

Megakaryocyte suppression, Viral Infections

Bone Marrow Failure, Cytotoxic Drugs, Radiptherapy

Bone Marrow Infiltration(an increase in the number of macrophages in the bone marrow), Leukaemia, Granuloma {cancer}

Infective Megaloblastic Syndrome and,
Myelodysplastic Syndrome

Question 2

Describe the pathogenesis of ITP

Answer 2

Basically caused by antiplatelet antibodies that are directed against specific platelet glycoproteins. This basically leads to increased Platelet Clearance and ALSO decreased platelet production.

Question 3

Describe Clinical Presentation of ITP

Answer 3

Primary- would be in children of Ages 1 year to 5 years, typically after a viral infection. (caused by preceding viral infection)

Secondary-
HIV
lymphoproliferative Disorders
Autoimmune Disorders

Could cause Mucocutaneous Bleeding.

Question 4

What investigations would you make if you suspected ITP?

Answer 4

Full Blood Count and Peripheral Blood Smear review

check PLT concentration< 20 x10^9/litre

Question 5

Describe the management of ITP.
include First Line and Second Line of therapy

Answer 5

First Line Of Therapy
-Steroids
-IV Immunoglobulin

Second line Of Therapy
-Immunosuppressants
-Splenectomy
- RITUXIMAB
- Thrombopoietin Agonists (thrombopoeitin makes PLATELETS)

Question 6

Describe the laboratory and pentad of symptoms for Thrombotic Thrombocytopenic Purpura (TTP)

Answer 6

Pentad:
1. Thrombocytopenia, 2. Microangiopathic Haemolytic Anemia, 3. Neurological Abnormalities, 4, Renal Failure, and 5. Fever

Diagnosis:
profound thrombocytopenia (PLT< 20x10^9/litre)
impressively elevated serum LDH value (~3000)
Clotting studies are usually mostly NORMAL

Question 7

Describe the mangament principles of TTP(supportive and specific)

Answer 7

Supportive:
Avoid Platelet Transfusion, Give Folate, Seizure prophylaxis, and Prednisone 1mg/kg/day (this helps to decrease circulationg Auto-Antibodies)

Specific: Plasma Exhange