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. 1994 Nov;70(5):969-72.
doi: 10.1038/bjc.1994.431.

Neurofibromatosis and childhood leukaemia/lymphoma: a population-based UKCCSG study

C A Stiller  1 , J M Chessells, M Fitchett
Affiliations
Free PMC article

Neurofibromatosis and childhood leukaemia/lymphoma: a population-based UKCCSG study

C A Stiller et al. Br J Cancer. 1994 Nov.
Free PMC article

Abstract

There is a well-known raised risk of leukaemia in children with neurofibromatosis type 1 (NF-1). We carried out the first detailed population-based study of leukaemia and non-Hodgkin lymphoma (NHL) associated with NF-1 in order to estimate the risk and elucidate the relationship between these conditions. Over the 17 year study period there were five cases of chronic myelomonocytic leukaemia (CMML) in patients with NF-1 (relative risk 221; 95% CI 71-514), 12 cases of acute lymphoblastic leukaemia (ALL) (relative risk 5.4; 95% CI 2.8-9.4) and five cases of NHL (relative risk 10.0; 95% CI 3.3-23.4). Marrow cytogenetics could be reviewed for seven patients. Specific abnormalities found were monosomy 21 in a child with CMML and 7p+, 17p- in a child with ALL. No abnormalities were reported of 17q, which includes the NF1 gene. CMML occurred predominantly in boys, who also had a family history of NF-1. ALL and NHL were more often found in children with no previous family history.

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Comment in

  • Neurofibromatosis and childhood leukaemia.
    Zvulunov A, Metzker A. Zvulunov A, et al. Br J Cancer. 1996 Aug;74(3):494. doi: 10.1038/bjc.1996.390. Br J Cancer. 1996. PMID: 8695373 Free PMC article. No abstract available.

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