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Trisomy 19 as the sole chromosomal abnormality in proliferative chronic myelomonocytic leukemia

Daskalakis, M. ; Mauritzson, Nils LU ; Johansson, Bertil LU ; Bouabdallah, K. ; Onida, F. ; Kunzmann, R. ; Mueller-Berndorff, H. ; Schmitt-Graeff, A. and Luebbert, M. (2006) In Leukemia Research: A Forum for Studies on Leukemia and Normal Hemopoiesis 30(8). p.1043-1047
Abstract
Distinct morphologic and clinical features associated with specific chromosomal abnormalities have been described in subgroups of myelodysplastic syndromes (MDS), which often are losses or gains and only rarely translocations. Among 103 consecutive MDS patients diagnosed and karyotyped at the Albert-Ludwigs University of Freiburg (ALU) between 1993 and 1999, two chronic myelomonocytic leukemias (CMMoL) displayed trisomy 19 (+19) as the sole chromosomal abnormality. Three further CMMoL cases with +19 as the single abnormality, two of which previously reported, were collected from other centers. Four of the five patients presented with leukocytosis and splenomegaly, and an increased number of ringed sideroblasts was observed in two cases.... (More)
Distinct morphologic and clinical features associated with specific chromosomal abnormalities have been described in subgroups of myelodysplastic syndromes (MDS), which often are losses or gains and only rarely translocations. Among 103 consecutive MDS patients diagnosed and karyotyped at the Albert-Ludwigs University of Freiburg (ALU) between 1993 and 1999, two chronic myelomonocytic leukemias (CMMoL) displayed trisomy 19 (+19) as the sole chromosomal abnormality. Three further CMMoL cases with +19 as the single abnormality, two of which previously reported, were collected from other centers. Four of the five patients presented with leukocytosis and splenomegaly, and an increased number of ringed sideroblasts was observed in two cases. Treatment was low-dose Decitabine (cases 1 and 2), oral steroids (case 3), hydroxyurea (case 4), and daunorubicin/Ara-C (case 5). Transformation to acute myeloid leukemias (AML) occurred in three/five patients (cases 1, 2, and 4) 26, 12, and 22 months after diagnosis of CMMoL, respectively. We conclude that +19 as the sole anomaly is a rare but recurrent change in CMMoL, in particular of the proliferative type. It is at present unclear which gene(s) located on chromosome 19 might have a functional role for the development of this phenotype. (c) 2006 Elsevier Ltd. All rights reserved. (Less)
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; ; ; ; ; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Decitabine, karyotype, sideroblasts, myelodysplastic/myeloproliferative overlap syndrome, ringed
in
Leukemia Research: A Forum for Studies on Leukemia and Normal Hemopoiesis
volume
30
issue
8
pages
1043 - 1047
publisher
Elsevier
external identifiers
  • wos:000238936600019
  • scopus:33744827412
ISSN
1873-5835
DOI
10.1016/j.leukres.2006.01.003
language
English
LU publication?
yes
id
72087b30-a1e7-403f-bfa7-ecd4e8f7edda (old id 403962)
date added to LUP
2016-04-01 11:46:49
date last changed
2024-10-08 09:39:40
@article{72087b30-a1e7-403f-bfa7-ecd4e8f7edda,
  abstract     = {{Distinct morphologic and clinical features associated with specific chromosomal abnormalities have been described in subgroups of myelodysplastic syndromes (MDS), which often are losses or gains and only rarely translocations. Among 103 consecutive MDS patients diagnosed and karyotyped at the Albert-Ludwigs University of Freiburg (ALU) between 1993 and 1999, two chronic myelomonocytic leukemias (CMMoL) displayed trisomy 19 (+19) as the sole chromosomal abnormality. Three further CMMoL cases with +19 as the single abnormality, two of which previously reported, were collected from other centers. Four of the five patients presented with leukocytosis and splenomegaly, and an increased number of ringed sideroblasts was observed in two cases. Treatment was low-dose Decitabine (cases 1 and 2), oral steroids (case 3), hydroxyurea (case 4), and daunorubicin/Ara-C (case 5). Transformation to acute myeloid leukemias (AML) occurred in three/five patients (cases 1, 2, and 4) 26, 12, and 22 months after diagnosis of CMMoL, respectively. We conclude that +19 as the sole anomaly is a rare but recurrent change in CMMoL, in particular of the proliferative type. It is at present unclear which gene(s) located on chromosome 19 might have a functional role for the development of this phenotype. (c) 2006 Elsevier Ltd. All rights reserved.}},
  author       = {{Daskalakis, M. and Mauritzson, Nils and Johansson, Bertil and Bouabdallah, K. and Onida, F. and Kunzmann, R. and Mueller-Berndorff, H. and Schmitt-Graeff, A. and Luebbert, M.}},
  issn         = {{1873-5835}},
  keywords     = {{Decitabine; karyotype; sideroblasts; myelodysplastic/myeloproliferative overlap syndrome; ringed}},
  language     = {{eng}},
  number       = {{8}},
  pages        = {{1043--1047}},
  publisher    = {{Elsevier}},
  series       = {{Leukemia Research: A Forum for Studies on Leukemia and Normal Hemopoiesis}},
  title        = {{Trisomy 19 as the sole chromosomal abnormality in proliferative chronic myelomonocytic leukemia}},
  url          = {{http://dx.doi.org/10.1016/j.leukres.2006.01.003}},
  doi          = {{10.1016/j.leukres.2006.01.003}},
  volume       = {{30}},
  year         = {{2006}},
}