ITP
often no cause (cause be due to URTI, SLE, HIV, HCV)
- Acute: children (2-6 y.o) => abrupt hemorrhagic complication following viral illness
- Chronic: insidious onset (no disease relation), most often in adults 20-40 (3:1 Female:Male)
IgG antibodies formed against the patient’s platelets
Clinical Presentation
- minor bleeding/ easy bruising
- petechiae, hematuria, hematemesis or melena
- General no splenomegaly
Bloodwork
- Look for increase megakaryocytes due to increase bone marrow production
- thrombcytopenia
*diagnosis of exclusion
Treatment
- Most children will have spontaneous resolution of ITP
- Treatment typically reserved for people with symptomatic bleeding
- If >20,000 platelets, typically asymptomatic
- 1) IVIG (wait 12-24hr and check platelets 2) corticosteroids 3) splenectomy
TTP
Due to lose of ADAMTS13 cleaving vWF => causes formation of platelet microthrombi that occlude small blood vessels, shear RBCS (schistocytes) and causes end organ damage.
CAUSES: unknown, can be due to infections, bacteria toxins, drug related, autoimmune, or idiopathic
INCREASED LDH
BLOOD SMEAR: schistocytes
- Also known as “helmet cells”
- fragmented RBC’s due to MAHA (formation of platelet-fibrin mesh due to increased activation of coagulation)
PENTAD
1) Neurological symptoms (delirium, seizure, stroke)
2) Renal dysfunction (increase Cr)
3) Fever
4) Thrombocytopenia
5) Microangiopathic hemolytic anemia
Treatment
- STEROIDS + PLASMAPHERESIS
- DO NOT give platelets (contraindicated); it may worsen the condition