Long-lasting partial remission by Interferon-alpha treatment in a child with essential thrombocythemia.

Abstract

Essential thrombocythemia (ET) is a clonal myeloproliferative disorder characterized by sustained thrombocytosis, isolated hyperplasia of megakaryocytic lineage, and association with thrombotic or bleeding episodes. It is extremely rare in childhood and frequently presents without evident clinical signs. We describe a 3-year-old girl with severe headache and dizziness suffering from ET, who was treated with Interferon-alpha-2a (IFN) based on the potent effect of this agent to inhibit myeloid colonies induced by phytohemagglutinin A stimulated leukocyte conditioned medium (PHA-LCM). Bone-marrow-derived mononuclear cells of this patient did not exhibit spontaneous colony formation but responded to recombinant human (rh) erythropoietin (EPO), rh granulocyte-colony stimulating factor (G-CSF), granulocyte-macrophage (GM)-CSF, and stem-cell factor in addition to PHA-LCM. After 65 months of in vivo IFN treatment, the patient experienced a sustained partial remission with platelet counts varying between 400 and 600 x 10(3)/microl.

• Authors: István Szegedi, Ilona Benko, Gabriella Mero, Agota Prinzinger, János Kappelmayer, Csongor Kiss
• Journal: Pediatric blood & cancer (Pediatr Blood Cancer) Vol. 49 Issue 5 Pg. 744-7 (Oct 15 2007) ISSN: 1545-5009 [Print] United States
• PMID: 16421912 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: (c) 2007 Wiley-Liss, Inc.

Chemical References

• Interferon-alpha

Topics

• Bone Marrow Examination
• Child, Preschool
• Colony-Forming Units Assay
• Female
• Humans
• Interferon-alpha (therapeutic use)
• Platelet Count
• Remission Induction
• Thrombocytosis (drug therapy, pathology)
• Treatment Outcome