Successful treatment of essential thrombocythemia evolving into agnogenic myeloid metaplasia with interferon-alpha.
OBJECTIVE
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.


METHODS
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.


CONCLUSIONS
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.
Kikawa
Y
Y
Department of Pediatrics, Fukui Medical University School of Medicine, Japan.
Fukumoto
Y
Y
Obata
K
K
Tamura
S
S
Takeuchi
M
M
Tanizawa
A
A
Mayumi
M
M
D002648:Child; D005260:Female; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use*; D055728:Primary Myelofibrosis / Q000188:drug therapy* / Q000209:etiology*; D013922:Thrombocytosis / Q000150:complications* / Q000503:physiopathology
D016898:Interferon-alpha