A 5-year-old boy is brought in by his mother because of petechial bleeding and bruises on his torso and limbs. The child doesn't have any other signs or symptoms and doesn't appear to be ill. Mother reports gastrointestinal infection several weeks prior to petechiae and bruising. CBC shows thrombocytopenia (<20xl09/L). Other parameters of CBC are within expected range for age. Also, PT, PTT and metabolic panels are all within referent range. What is the expected outcome of this blood disorder?
Answer: a) Complete resolution is expectedDiagnosis appears to be UP. It usually follows viral infections in children aged 2 to 5 years and is of autoimmune origin. It is self-limiting in most cases and resolves completely in 6 weeks to 6 months in 80% of children.IMMUNE THROMBOCYTOPENIC PURPURA (ITP)CHRONIC ITP* More common form* Most common in adult women < 40 yrs (female-to-male ratio -3:1)* Can be associated with connective tissue diseases, lymphoproliferative disease, medications and infections (hepatitis C virus and HIV)* Auto-antibodies (mostly IgG) against platelet membrane glycoproteins (llb-llla or Ib-IX)* Spleen is the major site of destruction of platelets* Petechiae, ecchymosis, easy bruising, bleeding from nose and gums, menorrhagia* Splenomegaly & lymphadenopathy - rare* Subarachnoid and intracerebral hemorrhage are rare but fatal complications* Wet purpura (blood blisters in the mouth)* Peripheral smear shows abnormally large platelets (megathrombocytes) & low platelet count* Bone marrow - hypercellular & increased megakaryocytes* Bleeding time prolonged* PT and PTT are normalTreatment* Initial treatment for patients without significant bleeding: Glucocorticoids (Prednisone or Dexa) +- IV immunoglobulin or anti-D or anti-CD20 antibody (rituximab)* Two-thirds of patients respond to initial treatment, but most people relapse following reduction of the corticosteroid dose* Patients with severe ITP and/or symptoms of bleeding: hospital admission and combined therapy (high-dose glucocorticoids + IVIgG or anti-RhO (D) therapy)* Relapse: Splenectomy* Refractory cases: Thrombopoietin receptor agonists (Romiplostim and eltrombopag) or splenectomyACUTE ITP* Disease of childhood* Affects both sexes equally* Onset is abrupt* Many cases are preceded by viral illness* The usual interval between the infection and onset of purpura is 2 weeks* Self-limited, spontaneous resolution in 6 months* 20% children (those without viral prodrome) have persistent low platelet counts after 6 months
Complete resolution is expected
Survival rate is up to 70% depending on risk stratification
Lifelong disease dependent on factor VIII substitution
Lifelong disease dependent on factor IX substitution
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