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Semin Thromb Hemost 2011; 37(7): 745-755
DOI: 10.1055/s-0031-1297165
© Thieme Medical Publishers

Childhood Immune Thrombocytopenia: A Changing Therapeutic Landscape

Vicky R. Breakey1 , Victor S. Blanchette1 , 2
  • 1Divison of Hematology/Oncology, Department of Pediatrics, Hospital for Sick Children, Toronto, Canada
  • 2Department of Pediatrics, University of Toronto, Toronto, Canada
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Publication History

Publication Date:
20 December 2011 (online)

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ABSTRACT

Childhood immune thrombocytopenia (ITP) is generally a benign self-limiting disorder of young children with <10% of cases requiring regular platelet enhancing therapy at 1 year following diagnosis. Increasingly, children with newly diagnosed ITP, who have isolated thrombocytopenia and no atypical features in the history or physical examination, are managed with minimal investigation and observation alone. The role of up-front, short-course corticosteroid therapy without bone marrow aspiration in this subgroup of cases merits further investigation. For children with clinically significant chronic ITP, the timing of elective splenectomy and the role of splenectomy-sparing strategies such as rituximab continues to be debated. Management of children with combined autoimmune cytopenias secondary to systemic lupus erythematosus, common variable immunodeficiency, and the autoimmune lymphoproliferative syndrome is often a challenge. Splenectomy should be avoided in cases with documented immunodeficiencies because of the increased risk of overwhelming sepsis postsplenectomy. For these cases, as well as for children with resistant primary chronic ITP who have failed splenectomy, the role of therapies such as mycophenolate mofetil, sirolimus, and the thrombopoietins remains to be determined.

KEYWORDS

Immune thrombocytopenic purpura - immune thrombocytopenia - children

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Victor S. BlanchetteF.R.C.P. 

Medical Director, Pediatric Thrombosis and Hemostasis Program, Division of Hematology/Oncology, Hospital for Sick Children

555 University Avenue, Toronto, ON M5G 1X8, Canada

Email: victor.blanchette@sickkids.ca