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Sudan J Paed. 2012; 12(2): 60-4


Chronic immune thrombocytopenia in a child responding only to thrombopoietin receptor agonist.

Mohamed E Osman.




Abstract

Immune thrombocytopenia (ITP) is an acquired hematological disease in which the body produces antibodies against its own platelets leading to platelet destruction resulting in isolated thrombocytopenia. Childhood ITP may enter complete remission in the majority of cases within six months from diagnosis. However, 20-30% of affected children may develop chronic ITP (lasting for more than 12 months). First line treatment includes intravenous immunoglobulin (IVIG), corticosteroids or anti-D immunoglobulin. Second line treatment includes splenectomy, immunosuppressive therapy or Rituximab. Recently two thrombopoietin (TPO) receptor agonists (Romiplostim and Eltrombopag) are used to increase platelet count in refractory chronic ITP by increasing platelet production in bone marrow. Here is a case report on an 8 ½ -year-old boy with refractory chronic ITP who failed therapy with IVIG, corticosteroids, splenectomy and Rituximab. He showed excellent response to treatment with TPO receptor agonist (Romiplostim). His platelet count increased from less than 10 x10(3)/dl and maintained between 100x10(3)/dl to 200x10(3)/dl after few weeks of starting Romiplostim therapy.

Key words: Immune thrombocytopenia; Platelet; Romiplostim






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