TY - JOUR
T1 - Severe Chronic Idiopathic Thrombocytopenic Purpura during Childhood
T2 - Definition, Management, and Prognosis
AU - Buchanan, George R.
AU - Journeycake, Janna M.
AU - Adix, Leah
PY - 2003/12
Y1 - 2003/12
N2 - 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.
AB - 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.
KW - Chronic
KW - ITP
KW - Severe
KW - Splenectomy
KW - Thrombocytopenia
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U2 - 10.1055/s-2004-815627
DO - 10.1055/s-2004-815627
M3 - Review article
C2 - 14719176
AN - SCOPUS:0346100478
SN - 0094-6176
VL - 29
SP - 595
EP - 603
JO - Seminars in Thrombosis and Hemostasis
JF - Seminars in Thrombosis and Hemostasis
IS - 6
ER -