Reassessment of treatment modalities for paediatric patients with chronic immune thrombocytopenia.
Approximately 70% of children have the acute form of immune thrombocytopenia (ITP), which is defined by recovery within six months of presentation with or without treatment. Chronic ITP is to be reserved for patients with platelets < 100000/microl for more than twelve months and exclusion of other diagnosis like systemic lupus erythematosus or bone marrow failures. In children, the chance of spontaneous recovery is 52% after diagnosis of chronic ITP. The Intercontinental Childhood ITP Study group recommends that children without bleeding may not require therapy regardless of their platelet count. Whereas in patients with bleeding symptoms first line therapy is defined and includes steroids or immunoglobuline, second line therapy in refractory patients with significant hemorrhagic problems is unclear. Guidelines recommend splenectomy, but for more than 50 years patients and physicians look for pharmacological alternatives. It may be that rituximab is a promising option which has been proven to be effective with few adverse effects. Till now the treatment has focused on immunomodulation. Research has now focused on stimulating platelet production. In this review we discuss old and new therapy modalities for children with cITP.
Laws
Hans-Jürgen
HJ
Department of Paediatric Oncology, Haematology and Clinical Immunology, University Hospital, 40225 Duesseldorf, Germany. laws@med.uni-duesseldorf.de
Janssen
G
G
Borkhardt
A
A
D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D003907:Dexamethasone / Q000627:therapeutic use; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D007136:Immunoglobulins / Q000627:therapeutic use; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D000069283:Rituximab; D013156:Splenectomy
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D005938:Glucocorticoids; D007074:Immunoglobulin G; D007136:Immunoglobulins; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003907:Dexamethasone