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Long‐lasting partial remission by Interferon‐alpha treatment in a child with essential thrombocythemia
Author(s) -
Szegedi István,
Benkő Ilona,
Merő Gabriella,
Prinzinger Ágota,
Kappelmayer János,
Kiss Csongor
Publication year - 2006
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.20744
Subject(s) - thrombocytosis , medicine , essential thrombocythemia , anagrelide , bone marrow , alpha interferon , myeloid , platelet , immunology , erythropoietin , haematopoiesis , myeloproliferative disorders , granulocyte colony stimulating factor , gastroenterology , interferon , stem cell , chemotherapy , biology , genetics
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 × 10 3 /µl. Pediatr Blood Cancer 2007;49:744–747. © 2006 Wiley‐Liss, Inc.