Leukemia in Fanconi’s Anemia: Cytogenetic and Tumor Virus Susceptibility Studies

Author:

DOSIK HARVEY12,HSU LILLIAN Y.13,TODARO GEORGE J.14,LEE STANLEY L.15,HIRSCHHORN KURT16,SELIRIO EVA S.17,ALTER AARON A.18

Affiliation:

1. Division of Hematology, Department of Medicine, Maimonides Medical Center, Brooklyn, N.Y., and the Department of Medicine, State University of New York, Downstate Medical Center, Brooklyn, N.Y., the Division of Medical Genetics, Department of Pediatrics, Mount Sinai School of Medicine, New York, N.Y., and the National Cancer institute, National Institutes of Health, Bethesda, Md.

2. Department of Medicine, Mainlonides Medical Center, Brooklyn, N.Y.; Clinical Instructor, Department of Medicine, State University of New York, Dotenstate Medical Center, Brooklyn, N.Y.

3. Division of Medical Genetics, Department of Pediatrics, Mount Sinai School of Medicine, New York, N.Y.

4. Molecular Biology Section, Viral Carcinogenesis Branch, National Cancer Institute, National Institutes of Healtil, Bethesda, Md.

5. Division of Hematology, Maimonides Medical Center, Brooklyn, N.Y.; Professor of Medicine, State University of New York, Downstate Medical Center, Brooklyn, N.Y.

6. Arthur J. and Nellie Z. Cohen Professor, Department of Pediatrics; Chief, Division of Medical Genetics, Mount Sinai School of Medicine; New York City Health Research Council Career Scientist 1-513.

7. Department of Medicine, Maimonides Medical Center, Brooklyn, N.Y.

8. Division of Hematology, Department of Medicine, Maimonides Medical Center, Brooklyn, N.Y.; Assistant Professor, Department of Medicine, State University of New York, Downstate Medical Center, Brooklyn, N. Y.

Abstract

Abstract Fanconi’s anemia is an inherited disorder characterized by multiple congenital abnormalities, chromosome anomalies and irreversible aplastic anemia. Patients, and possibly family members, have been noted to have an increased incidence of leukemia and other tumors. A family with two affected male children, one of whom developed acute myelomonocytic leukemia, is described. Chromosome preparations in both parents showed abnormalities, a previously unreported finding. Fibroblast cultures from both parents and one brother inoculated with Simian Virus 40, a known oncogenic agent, developed increased numbers of transformed colonies, compared with normal control cultures similarly inoculated. The presence of chromosome abnormalities and cellular susceptibility to oncogenic agents appear to be related to an increased risk of malignancy.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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