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Extensive cytogenetic studies of clonality following interferon-alpha therapy in chronic myeloid leukemia occurring in monosomic cells in a patient with Turner syndrome mosaic.
Extensive cytogenetic studies of clonality following interferon-alpha therapy in chronic myeloid leukemia occurring in monosomic cells in a patient with Turner syndrome mosaic.
- Source :
- Leukemia (08876924); Nov99, Vol. 13 Issue 11, p1749-1753, 5p
- Publication Year :
- 1999
-
Abstract
- In a 27-year-old female with Turner syndrome mosaic, Philadelphia (Ph) chromosome-positive chronic myeloid leukemia (CML) occurred only in the monosomic cells (45, Xc). Extensive cytogenetic studies, including triple-color fluorescence in situ hybridization (FISH), revealed that Ph-positive monosomic cells (45, Xc), Ph-negative monosomic cells and normal diploid cells (46, XX) were present in her bone marrow at diagnosis. After successful interferon therapy, the non-leukemia cells expanded and reconstituted normal hematopoiesis resulting in complete cytogenetic response, following the selective suppression of the monosomic Ph-positive leukemia clone. The ratio of Xc to XX cells in bone marrow cells was significantly increased to that in skin fibroblasts. Moreover, the ratio of Ph-positive cells to Ph-negative cells was found to be significantly different between karyotyping and FISH. Studies of this quite unique case not only confirmed the clonality of CML, effectiveness of interferon-alpha and X chromosome imbalance among different tissues, but also demonstrated a discrepant increase of the BCR/ABL-positive clone in CML. The latter supports the hypothesis that reduced programmed cell death may be the primary mechanism responsible for the expansion of the leukemia clone in CML. Our study verifies the importance of extensive analysis of a neoplastic disease in patients with a constitutional chromosomal abnormality. [ABSTRACT FROM AUTHOR]
- Subjects :
- TURNER'S syndrome
MYELOID leukemia
TREATMENT of chronic myeloid leukemia
THERAPEUTIC use of proteins
MOSAICISM
PROTEINS
CHROMOSOMES
FIBROBLASTS
CHRONIC myeloid leukemia
CYTOMETRY
SKIN
KARYOTYPES
CHROMOSOME abnormalities
CELLS
FLUORESCENCE in situ hybridization
CHI-squared test
BONE marrow
DISEASE remission
DISEASE complications
Subjects
Details
- Language :
- English
- ISSN :
- 08876924
- Volume :
- 13
- Issue :
- 11
- Database :
- Complementary Index
- Journal :
- Leukemia (08876924)
- Publication Type :
- Academic Journal
- Accession number :
- 8883877
- Full Text :
- https://doi.org/10.1038/sj.leu.2401528