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Prepublished online as a Blood First Edition Paper on January 2, 2003; DOI 10.1182/blood-2002-10-3243.
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Blood, 15 May 2003, Vol. 101, No. 10, pp. 3872-3874
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Brief report
Clonal chromosomal aberrations in bone marrow cells of Fanconi
anemia patients: gains of the chromosomal segment 3q26q29 as an adverse
risk factor
Holger Tönnies,
Stefanie Huber,
Jörn-Sven Kühl,
Antje Gerlach,
Wolfram Ebell, and
Heidemarie Neitzel
From the Institute of Human Genetics, Charité,
Campus-Virchow, Humboldt-University, Berlin, Germany; and
the Department of General Pediatrics, Bone Marrow Transplant Unit,
Charité, Campus-Virchow, Humboldt-University, Berlin,
Germany.
Fanconi anemia (FA) is a condition that induces susceptibility to
bone marrow failure, myelodysplastic syndrome (MDS), and leukemia. We
report on a high incidence of expanding clonal aberrations with partial
trisomies and tetrasomies of chromosome 3q in bone marrow cells of 18 of 53 FA patients analyzed, detected by conventional and molecular
cytogenetics. To determine the clinical relevance of these findings, we
compared the cytogenetic data, the morphologic features of the bone
marrow, and the clinical course of these patients with those of 35 FA
patients without clonal aberrations of 3q. The 2 groups did not differ
significantly with respect to age, sex, or complementation group. There
was a significant survival advantage of patients without abnormalities
of chromosome 3q. Even more pronounced was the risk assessment of
patients with gains of 3q material with respect to the development of
morphologic MDS and acute myeloid leukemia (AML). Thus, our data from
18 patients with 3q aberrations reveal that gains of 3q are strongly
associated with a poor prognosis and represent an adverse risk factor
in FA.
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