When a Child with Immune Thrombocytopenia Experiences Life-threatening Bleeding

Immune thrombocytopenia (ITP) is a rare disorder with an incidence rate of 4–6 newly diagnosed children/100,000 per year. Life-threatening bleeding, i. e. intracranial hemorrhage (ICH), is roughly estimated in 1:750 children with ITP. In registry 1 of the intercontinental cooperative ITP study (ICIS) group, 3 of 2071 evaluable children with ITP were reported as having ICH during the first 12 months after diagnosis. In addition, there is an obscure ICH figure.

The investigator of a child with severe ITP is confronted with a heterogenous disorder with multiple unanswered questions such as what is behind the pathophysiology of ITP and ICH, can ICH be prevented and how should severe bleeding in ITP be managed. In addition, the hematologists have to consider how to proceed with clinical and laboratory research in this bleeding disorder with peripheral platelet destruction.

This issue of Klinische Pädiatrie includes 2 patient reports [14] [24], a new approach regarding prognostic platelet count/bleeding severity [18] and a report of partial spleen embolization [1]. These articles and questions are the basis of the following comments.

References

• 1 Alzen G, Basedow J, Berthold L. et al . Partial splenic embolization as an alternative to splenectomy in hypersplenism – single center experience in 16 years.  Klin Padiatr. 2010;  222 368–373
• 2 Butros LJ, Bussel JB. Intracranial hemorrhage in immune thrombocytopenic purpura: a retrospective analysis.  J Pediatr Hematol Oncol. 2003;  25 660-664
• 3 Elalfy M, Elbarbary N, Khaddah N. et al . Intracranial hemorrhage in acute and chronic childhood immune thrombocytopenic purpura over a ten-year period: an Egyptian multicenter study.  Acta Haematol. 2010;  123 59-63
• 4 Foster CB, Zhu S, Erichsen HC. et al . Early Chronic ITP Study Group. Polymorphisms in inflammatory cytokines and Fcgamma receptors in childhood chronic immune thrombocytopenic purpura: a pilot study.  Br J Haematol. 2001;  113 596-599
• 5 Göbel U, Witt O. Pitfalls of clinical registries in pediatric oncology and haematology.  Klin Padiatr. 2008;  220 129-133
• 6 Imbach P, Barandun S, d’Apuzzo V. et al . High-dose intravenous gammaglobulin for idiopathic thrombocytopenic purpura in childhood.  Lancet. 1981;  1 (8232) 1228-1231
• 7 Imbach P. Refractory idiopathic immune thrombocytopenic purpura in children: current and future treatment options.  Paediatr Drugs. 2003;  5 795-801
• 8 Imbach P, Kühne T, Müller D. et al . Childhood ITP: 12 months follow-up data from the prospective registry I of the Intercontinental Childhood ITP Study Group (ICIS).  Pediatr Blood Cancer. 2006;  46 351-356
• 9 Imbach P, Lazarus AH, Kühne T. Intravenous immunoglobulins induce potentially synergistic immunomodulations in autoimmune disorders.  Vox Sang. 2010;  98 385-394
• 10 Iyori H, Bessho F, Ookawa H. et al . Japanese Study Group on childhood ITP. Intracranial hemorrhage in children with immune thrombocytopenic purpura. Japanese Study Group on childhood ITP.  Ann Hematol. 2000;  79 691-695
• 11 Kimura F, Itoh H, Ambiru S. et al . Long-term results of initial and repeated partial splenic embolization for the treatment of chronic idiopathic thrombocytopenic purpura.  Am J Roentgenol. 2002;  179 1323-1326
• 12 Kühne T, Blanchette V, Buchanan GR. et al . Intercontinental Childhood ITP Study Group: Splenectomy in children with idiopathic thrombocytopenic purpura. A prospective study of 134 children.  Pediatr Blood Cancer. 2007;  49 829-834
• 13 Kühne T, Imbach P. Editorial 3^rd Intercontinental Cooperative ITP Study Group (ICIS) Expert Meeting in Basel, Switzerland, September 2009.  Ann Hematol. 2010;  89 (S 01) S1-S2
• 14 Landgraf M, Siekmeyer M, Fischer L. et al . Coincidence of immune thrombocytopenia, intracranial hemorrhage and graft-versus-host disease in an 11-year-old girl.  Klin Padiatr. 2010;  222 378–382
• 15 Lilleyman JS. Intracranial haemorrhage in idiopathic thrombocytopenic purpura. Paediatric Haematology Forum of the British Society for Haematology.  Arch Dis Child. 1994;  71 251-253
• 16 Lilleyman J. Medical nemesis and childhood ITP.  Br J Haematol. 2003;  123 586-589
• 17 Neunert CE, Buchanan GR, Imbach P. et al . Intercontinental Childhood ITP Study Group Registry II Participants. Severe hemorrhage in children with newly diagnosed immune thrombocytopenic purpura.  Blood. 2008;  112 4003-4008
• 18 Pansy J, Minkov M, Dengg R. et al . Evaluating bleeding severity in children with newly diagnosed immune thrombocytopenia.  Klin Padiatr. 2010;  222 374–377
• 19 Provan D, Stasi R, Newland AC. et al . International consensus report on the investigation and management of primary immune thrombocytopenia.  Blood. 2010;  115 168-186
• 20 Psaila B, Petrovic A, Page LK. et al . Intracranial hemorrhage (ICH) in children with immune thrombocytopenia (ITP): study of 40 cases.  Blood. 2009;  114 4777-4783
• 21 Sutor AH, Harms A, Kaufmehl K. Acute immune thrombocytopenia (ITP) in childhood: retrospective and prospective survey in Germany.  Semin Thromb Hemost. 2001;  27 253-267
• 22 Tarantino MD, Bolton-Maggs PH. Update on the management of immune thrombocytopenic purpura in children.  Curr Opin Hematol. 2007;  14 526-534
• 23 Treutiger I, Rajantie J, Zeller B. et al . NOPHO ITP Study Group. Does treatment of newly diagnosed idiopathic thrombocytopenic purpura reduce morbidity?.  Arch Dis Child. 2007;  92 704-707
• 24 Uetz B, Wawer A, Nathrath M. et al . Intracranial hemorrhage in immune thrombocytopenic purpura (ITP): fatal course in spite of maximum therapy.  Klin Padiatr. 2010;  222 383–387
• 25 Vesely S, Buchanan GR, Cohen A. et al . Self-reported diagnostic and management strategies in childhood idiopathic thrombocytopenic purpura: results of a survey of practicing pediatric hematology/oncology specialists.  J Pediatr Hematol Oncol. 2000;  22 55-61
• 26 Wadenvik H, Olsson B. Moving towards a new era in the management of chronic immune thrombocytopenia26.  Ann Hematol. 2010;  89 (S 01) S87-S93

Correspondence