Christopher P. Coppola, Alfred P. Kennedy, Jr. and Ronald J. Scorpio (eds.)Pediatric Surgery2014Diagnosis and Treatment10.1007/978-3-319-04340-1_53
© Springer International Publishing Switzerland 2014
Idiopathic Thrombocytopenic Purpura
(1)
Pediatric Hematology/Oncology, Janet Weis Children’s Hospital, 100 N. Academy Av. MC 13-20, Danville, PA 17822, USA
Abstract
Idiopathic thrombocytopenic purpura (ITP) is marked by thrombocytopenia (often profound) occurring due to destruction of antibody sensitized platelets by the reticuloendothelial system (RES).
Idiopathic thrombocytopenic purpura (ITP) is marked by thrombocytopenia (often profound) occurring due to destruction of antibody sensitized platelets by the reticuloendothelial system (RES).
1.
Pathophysiology:
(a)
Incidence:
(i)
Most common autoimmune disorder affecting blood elements.
(ii)
Affects 1 in 10,000 children. This may be an underestimate: Asymptomatic patients may not come to medical attention.
(iii)
Acute ITP: duration of thrombocytopenia <6 months (some experts prefer using a 12 month cutoff for acute ITP).
(iv)
Chronic ITP: duration of thrombocytopenia >6 months (or 12 months, according to some).
(b)
Autoantibodies interact with membrane glycoproteins on platelets or megakaryocytes which are then cleared by the reticuloendothelial system.
(c)
Process is not well understood:
(i)
Viral immunocomplexes attach to platelet membrane.
(ii)
Antiviral antibodies cross react with platelet antigens.
(iii)
Absorption of viral particles on to platelet membranes.
(iv)
This process results in increased destruction and possibly decreased production of platelets.
(d)
Thrombopoietin levels are normal or only slightly elevated.
2.
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Clinical presentation:
(a)
Classical presentation is that of a healthy child who presents with bruising, petechiae, and isolated thrombocytopenia. It is important to rule out artifact or laboratory error and “spurious” thrombocytopenia secondary to EDTA anticoagulant.
(b)
Often preceded by immunization (measles mumps rubella) or viral syndrome.
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