Myelodysplastic syndrome/acute myeloid leukemia supervening previously untreated chronic B-lymphocytic leukemia: demonstration of the concomitant presence of two different malignant clones by immunologic and molecular analysis |
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Authors: | G Mitterbauer J Schwarzmeier M Mitterbauer U Jaeger G Fritsch I Schwarzinger |
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Institution: | Department of Laboratory Medicine, University of Vienna, W?hringer Gürtel 18–20, A-1090 Vienna, Austria, AT Department of Medicine I, Division of Hematology and Hemostaseology, University of Vienna, Austria, AT CCRI, St. Anna Children's Hospital, Vienna, Austria, AT
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Abstract: | The development of myelodysplastic syndrome (MDS) and/or acute myeloid leukemia (AML) has rarely been observed in patients
with chronic B-lymphocytic leukemia (B-CLL). So far, the discussion concerning the pathogenesis of the simultaneous occurrence
of these two malignancies has been speculative, opposing the theory of two separate malignant clones to the theory of a common
stem cell malignancy. We describe the case of a 77-year-old woman who developed MDS after 8 years of an indolent course of
B-CLL. The diagnosis of MDS was based on bone marrow (BM) morphology, showing the typical picture of a refractory anemia with
excess of blasts (RAEB). The clinical course of MDS was aggressive, terminating in AML within only 6 months. Immunophenotyping
of BM and peripheral blood (PB) cells revealed a CD34+/CD13+/CD33–/CD19– blast cell population and a CD19+/CD5+ B-cell population with kappa light chain restriction. Molecular analysis of PB and BM demonstrated the presence of an immunoglobulin
heavy chain (IgH) gene rearrangement by polymerase chain reaction (PCR) amplification of genomic DNA with three different
pairs of consensus primers. Cell-sorting experiments showed that the IgH gene rearrangement was present only in the CD19+/CD34– B-cell population, but not in the CD34+/CD19– blast cells. Furthermore, X-chromosome inactivation pattern analysis revealed two differently methylated cell populations.
These experiments demonstrate the concomitant existence of two different clones in a patient with CLL-MDS/AML.
Received: 30 October 1996 / Accepted: 8 January 1997 |
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Keywords: | Chronic B-lympohocytic leukemia Secondary MDS/AML Clonal analysis IgH gene rearrangement DXS 255/M27β |
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