International Journal of Hematology and Oncology
2023, Vol 33, Num 4 Page(s): 045-047
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A Case of Polycythemia Vera Accompanied with Neurofibromatosis Type 1
Semir PASA1, Abdullah ALTINTAS1, Kadim BAYAN2, Yekta TUZUN2, Timucin CIL1, Orhan AYYILDIZ1
1Dicle University Faculty of Medicine, Department of Hematology, Diyarbakır, TURKEY
2Dicle University Faculty of Medicine, Department of Gastroenterology, Diyarbakır, TURKEY
Keywords: Neurofibromatosis, NF1 gene, Polycythemia vera
Classical myelofibrosis syndromes (MPS) most frequently occur in adults, but MPS unique to childhood also exist. Such syndromes include juvenile chronic myelomonocytic leukemia (JMML), the MPS of monosomy 7 in childhood, familial chronic myeloid leukemia (CML), the transient MPS of infants with trisomy 21, and childhood forms of myelofibrosis. Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder with pigmentary abnormalities and are predisposed to benign and malignant neoplasms, mostly in children. MPSs were disproportionately common among children with NF1. Herein we reported a case of polycythemia vera (PV) accompanied to NF1 in an adult patient. The co-existance of NF1 and childhood MPS is a well known condition. The same relation was not demonstrated in adulthood NF1 patients according to our screening of the literature. Most of the reported cases were define a relation with JMML and monosomy 7 syndrome. Probably this is the first case of PV, a type of classic adult MPS accompanied to NF1.
Semir PASA1, Abdullah ALTINTAS1, Kadim BAYAN2, Yekta TUZUN2, Timucin CIL1, Orhan AYYILDIZ1
1Dicle University Faculty of Medicine, Department of Hematology, Diyarbakır, TURKEY
2Dicle University Faculty of Medicine, Department of Gastroenterology, Diyarbakır, TURKEY
Keywords: Neurofibromatosis, NF1 gene, Polycythemia vera
Classical myelofibrosis syndromes (MPS) most frequently occur in adults, but MPS unique to childhood also exist. Such syndromes include juvenile chronic myelomonocytic leukemia (JMML), the MPS of monosomy 7 in childhood, familial chronic myeloid leukemia (CML), the transient MPS of infants with trisomy 21, and childhood forms of myelofibrosis. Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder with pigmentary abnormalities and are predisposed to benign and malignant neoplasms, mostly in children. MPSs were disproportionately common among children with NF1. Herein we reported a case of polycythemia vera (PV) accompanied to NF1 in an adult patient. The co-existance of NF1 and childhood MPS is a well known condition. The same relation was not demonstrated in adulthood NF1 patients according to our screening of the literature. Most of the reported cases were define a relation with JMML and monosomy 7 syndrome. Probably this is the first case of PV, a type of classic adult MPS accompanied to NF1.
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