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.
Service de Médecine Interne, Hôpital de Jolimont, Haine-St-Paul, Belgique.
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Van Baren
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Issa
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Delannoy
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