Von Recklinghausen neurofibromatosis and hematologic malignancies: 2 case reports in adulthood.
Von Recklinghausen's neurofibromatosis is a hereditary disease predisposing to distinctive malignant hemopathies. These often develop during early childhood and are characterized by particular cytologic subtypes: juvenile chronic myeloid leukemia, monosomy 7-associated myeloproliferative syndrome and myelomonocytic leukemia. The etiopathologic mechanism underlying this association begins to be elucidated: the neurofibromatosis gene behaves like a tumor suppressor gene; its inactivation by mutation results in activation of the corresponding oncogenes. We report here the cases of two late-aged adults with neurofibromatosis: the first developed acute myelogenous leukemia, the second polycythemia vera. Based on a review of the literature, we suggest that, in opposition to childhood, the association between neurofibromatosis and malignant blood diseases is not demonstrated in adulthood.
Van Baren
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Service de Médecine Interne, Hôpital de Jolimont, Haine-St-Paul, Belgique.
Issa
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Delannoy
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D000368:Aged; D005260:Female; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000150:complications* / Q000175:diagnosis; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis; D011087:Polycythemia Vera / Q000150:complications* / Q000175:diagnosis