Severe chronic idiopathic thrombocytopenic purpura during childhood: definition, management, and prognosis.
Chronic idiopathic thrombocytopenic purpura (ITP) can be categorized as mild, moderately severe, or severe. Severe chronic ITP during childhood is a rare disorder characterized by clinically significant mucocutaneous hemorrhage, usually in the setting of marked thrombocytopenia. It can cause substantial morbidity and rarely mortality. Many patients improve with time or even fully recover, but for those whose quality of life is negatively influenced by hemorrhage or side effects of conventional therapy (corticosteroids, intravenous immunoglobulin G, or anti-D), splenectomy is recommended. If splenectomy is unsuccessful or not feasible, other drug treatments are available, but few efficacy data exist, and the toxicity and cost of these treatments can be appreciable. Their use is best avoided outside of clinical trials conducted in specialty centers or in multi-institutional networks.
Buchanan
George R
GR
Division of Hematology-Oncology, Department of Pediatrics, The University of Texas Southwestern Medical Center at Dallas, Children's Medical Center of Dallas, Dallas, Texas, USA. george.buchanan@utsouthwestern.edu
Journeycake
Janna M
JM
Adix
Leah
L
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D002648:Child; D018450:Disease Progression; D006470:Hemorrhage / Q000209:etiology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D010976:Platelet Count; D011379:Prognosis; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000097:blood / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*; D013156:Splenectomy
D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous