Immune thrombocytic Purpura + Thrombotic thrombocytic Purpura Flashcards
What is Immune thrombocytic Purpura?
T2 hypersensitivity, Autoimmune, produce Ab against platelets (platelet destruction) IgG
What are the 2 types of ITP?
Acute and chronic
What is acute ITP?
typical Px?
Caused by?
Time length?
Children 2-6y/o
post viral infection / vaccine
acute + severe
<2 months
What is chronic ITP?
Typical Px?
Adult women with Malignancy, HIV, Other autoimmune conditions
Chronic and mild
What is Purpura?
Small bleeding spots under the skin
What are the symptoms of ITP in kids?
1-2 weeks post viral infection
Purpuric Rash
(+ easy bleeds, epistaxis + menorrhagia, mucosal bleeding/bruising)
Otherwise systemically well
Diagnosis of ITP?
Results?
FBC = Isolated Thrombocytopenia
BM examination if atypical features: high BM megakaryoblasts (negative feedback)
LNadenopathy, High WCC
Treatment of ITP in kids + adults?
(80% self resolve <6m)
In kids - don’t treat unless very low platelets, offer FBC in 1 week, avoid activities risking trauma
Adults = Prednisolone + IV IgG (decrease splenic platelet destruction)
(then splenectomy)
What is thrombotic thrombocytic Purpura?
Deficiency in enzyme ADAMTS13 (breaks down VWF cleaving enzyme)
familial inheritence
Pathology of TTP?
VWF normally cleaved into fragments, here they remain as multitimes and aggregate at endothelial Injury sites
No ADAM enzyme = small blood clots form more frequently
Which patients does TTP usually occur in?
Adult females, Malignancy, HIV, autoimmune
Which is the most common type of Purpura?
Immune thrombocytes Purpura
Symptoms of TTP?
Purpuric rash
menorrhagia
AKI
Fever
Anemia
Neurological Sx (not well)
Diagnosis of TTP?
Results?
Thrombocytopenia
Schistocytes (fragmented RBCs, microangiopathic haemolytic anemia)
Low ADAMTS13 (<10%)
Treatment for TTP?
- Plasmapharesis - swap for healthy plasma in machine (removes anti ADAM Ab and adds back enzyme)
- Prednisolone + Rituximab (low Anti ADAM Ab)
