Kikawa Y, Fukumoto Y, Obata K, Tamura S, Takeuchi M, Tanizawa A, Mayumi M
Department of Pediatrics, Fukui Medical University School of Medicine, Japan.
J Pediatr Hematol Oncol. 1998 Sep-Oct;20(5):463-6. doi: 10.1097/00043426-199809000-00010.
Essential thrombocythemia and agnogenic myeloid metaplasia as one form of idiopathic myelofibrosis are myeloproliferative disorders and are quite rare in children. A patient with essential thrombocythemia that transformed to "adult-type" agnogenic myeloid metaplasia is described.
A routine examination of an 8-year-old girl with bronchial asthma showed thrombocytosis. Essential thrombocythemia was diagnosed 2 years later. During a 3-year follow-up without treatment, transition of the essential thrombocythemia to agnogenic myeloid metaplasia was noticed. The patient had an excellent response to interferon (IFN)-alpha therapy.
This case is unique because of the availability of premorbid hematologic data and the natural progression of essential thrombocythemia to agnogenic myeloid metaplasia. Whether IFN-alpha therapy can prevent the progression of idiopathic myelofibrosis to leukemia has not yet been determined.
