Idiopathic Thrombocytopenic Purpura Flashcards
Define Idiopathic Thrombocytopenic Purpura
Haematological disorder characterised by isolated thrombocytopenia (platelet <100 x 109/L) in the absence of an identifiable cause
What are the causes and risk factors Idiopathic Thrombocytopenic Purpura
Thought to be the destruction of circulating platelets by IgG autoantibodies
Age <10yo
Women of childbearing age
What is the epidemiology of Idiopathic Thrombocytopenic Purpura
Childhood ITP has an incidence of 1.9-6.4 in 100,000 per year with equal distribution between sexes
Most common cause of thrombocytopenia in childhood
More common in females (3:1) for young adults
What are the symptoms of Idiopathic Thrombocytopenic Purpura
Mostly between 1-2yo and >10yo
Precipitated by viral infection of vaccination
Signs of bleeding
- Bruising (arms and legs, torso if severe)
- Petechiae (small red/purple spots, 1-5mm), mostly on the lower limbs
- Haemorrhagic bullae (2-5mm on mucosal surfaces of the oral cavity and tongue)
- Bleeding gums
NO systemic symptoms
NO causative medication Hx
What are the differentials for Idiopathic Thrombocytopenic Purpura
Pseudothrombocytopenia (Agglutination when exposed to EDTA
Henoch-schonlein purpura
NAI
Coagulation factor deficiency
Acute leukaemia
Congenital/acquired thrombocytopenia
thrombotic thrombocytopenic purpura
What are the signs of Idiopathic Thrombocytopenic Purpura on examination
General/skin
- Bruising (arms and legs, torso if severe)
- Petechiae (small red/purple spots, 1-5mm), mostly on the lower limbs
- Haemorrhagic bullae (2-5mm on mucosal surfaces of the oral cavity and tongue)
Head and neck: ?lymphadenopathy to exclude lymphoproliferative, autoimmune, or infectious aetiology
Abdominal: ?hepatosplenomegaly to rule out other differentials
What investigations should be done for Idiopathic Thrombocytopenic Purpura
Diagnosis of exclusion
Bedside: H. Pylori breath test/stool antigen test
Bloods:
- FBC: Isolated platelet count <100 × 10⁹/L (<100 × 10³/microlitre), otherwise NORMAL
- Blood smear: distinguish between pseudo and ITP
- HIV/hepC serology
Other: bone marrow aspiration (if suspecting neoplastic/aplastic processes)
What is the management for a first presentation of Idiopathic Thrombocytopenic Purpura
Minor bleeding: observe + avoid antiplatelets (aspirin, NSAIDs) + reduce trauma risk
Symptomatic/major bleeding: IVIg + corticosteroids ± platelet transfusion
What is the long-term management for Idiopathic Thrombocytopenic Purpura
First line: Mycophenolate (after acute treatment as failed)
Second line: rituximab, thrombopoietin receptor agonists e.g. eltrombopag
Third line: splenectomy
What are the complications of Idiopathic Thrombocytopenic Purpura
Intracranial haemorrhage
Persistent disease: ITP continues 3-6 months after diagnosis
Chronic disease: ITP continues >6 months after diagnosis
What is the prognosis for Idiopathic Thrombocytopenic Purpura
Benign condition in children
Majority of children who are asymptomatic or have minor bleeding symptoms will attain normalisation of platelet count
30-70% of children recover within 3 weeks
Major bleeding is rare
Chronic ITP affects 20% of children with ITP
What are the secondary causes of immune thrombocytopenic purpura
HIV
Hepatitis C
H. Pylori
Immunodeficiencies
Immunological/autoimmune disorders e.g. SLE, Evan’s syndrome, antiphospholipid syndrome, autoimmune thyroid disease
Lymphoproliferative disorders
Drug-induced
Vaccine exposure