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Prognosis of pediatric patients transplanted for Ph+ chronic myeloid leukemia in the period from 1989 to 2006 in the Czech Republic

Krol, L. LU orcid ; Formankova, R. ; Keslova, P. ; Vavra, V. ; Zemanova, Z. ; Michalova, K. ; Dobrovolna, M. ; Moravcova, J. ; Kobylka, P. and Ivaskova, E. , et al. (2008) In Neoplasma 55(2). p.101-106
Abstract

Chronic myeloid leukemia (CML) is a myeloproliferative disorder caused by clonal proliferation of primitive hematopoietic stem cell. The median age at diagnosis is 55 to 60 years with less than 10% of patients younger 20 years. Incidence of CML in children in the Czech Republic is 0.106 cases/100 thousands per year. Here we report outcome of 38 pediatric patients (median age 12.5 years; range 1.8 - 17.3) with Ph-positive CML diagnosed between years 1989 to 2006. Primarily chronic phase of the disease was diagnosed in 32 (84%) patients. 32 (84.2%) patients underwent hematopoietic stem cell transplantation (HSCT) with the median age at transplantation of 14.9 years (range 6.9 - 20.5 years). Out of transplanted patients 16 (50%) obtained... (More)

Chronic myeloid leukemia (CML) is a myeloproliferative disorder caused by clonal proliferation of primitive hematopoietic stem cell. The median age at diagnosis is 55 to 60 years with less than 10% of patients younger 20 years. Incidence of CML in children in the Czech Republic is 0.106 cases/100 thousands per year. Here we report outcome of 38 pediatric patients (median age 12.5 years; range 1.8 - 17.3) with Ph-positive CML diagnosed between years 1989 to 2006. Primarily chronic phase of the disease was diagnosed in 32 (84%) patients. 32 (84.2%) patients underwent hematopoietic stem cell transplantation (HSCT) with the median age at transplantation of 14.9 years (range 6.9 - 20.5 years). Out of transplanted patients 16 (50%) obtained graft from unrelated donor, 13 (41%) from matched sibling donor, 2 from haploidentical family donor and autologous transplantation has been performed in one case. 6 patients were not transplanted, 4 of them died (median 1.2 years from diagnosis), 2 are alive 0.6 and 17.8 years from the diagnosis. Overall survival (OS) in 25 patients after HSCT at our department during the whole period is 66.7% with 15/16 being in stable continuous molecular-genetic remission (94%). During the period of time results of transplantations have been significantly improved (p=0.0071). OS after HSCT until year 1997 is 25% while from year 1998 until now is 87.5%. All centers OS of patients after HSCT is 71%. Results of HSCT in children with CML obtained from the year 1998 at our center are fully comparable with results achieved in large and experienced centers. HSCT remains the only proven and effective method for the treatment of CML. Clinical studies assessing the role of tyrosine kinase inhibitors in children instead of early HSCT should be planned carefully in order to avoid sub-optimal outcomes.

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publishing date
type
Contribution to journal
publication status
published
subject
keywords
Allogeneic hematopoietic stem cell transplantation, Children, Incidence, Prognosis
in
Neoplasma
volume
55
issue
2
pages
101 - 106
publisher
Vydavatel'stvo Slovenkej Akademie Vied/Veda Publishing House of the Slovak Academy of Sciences
external identifiers
  • scopus:48549106575
  • pmid:18237247
ISSN
0028-2685
language
English
LU publication?
no
additional info
Copyright: Copyright 2020 Elsevier B.V., All rights reserved.
id
62363f21-1343-4eaf-b073-4a209132b588
alternative location
http://www.elis.sk/index.php?page=shop.product_details&flypage=flypage.tpl&product_id=1077&category_id=35&option=com_virtuemart&vmcchk=1&Itemid=1
date added to LUP
2021-04-29 08:12:43
date last changed
2024-01-05 10:33:29
@article{62363f21-1343-4eaf-b073-4a209132b588,
  abstract     = {{<p>Chronic myeloid leukemia (CML) is a myeloproliferative disorder caused by clonal proliferation of primitive hematopoietic stem cell. The median age at diagnosis is 55 to 60 years with less than 10% of patients younger 20 years. Incidence of CML in children in the Czech Republic is 0.106 cases/100 thousands per year. Here we report outcome of 38 pediatric patients (median age 12.5 years; range 1.8 - 17.3) with Ph-positive CML diagnosed between years 1989 to 2006. Primarily chronic phase of the disease was diagnosed in 32 (84%) patients. 32 (84.2%) patients underwent hematopoietic stem cell transplantation (HSCT) with the median age at transplantation of 14.9 years (range 6.9 - 20.5 years). Out of transplanted patients 16 (50%) obtained graft from unrelated donor, 13 (41%) from matched sibling donor, 2 from haploidentical family donor and autologous transplantation has been performed in one case. 6 patients were not transplanted, 4 of them died (median 1.2 years from diagnosis), 2 are alive 0.6 and 17.8 years from the diagnosis. Overall survival (OS) in 25 patients after HSCT at our department during the whole period is 66.7% with 15/16 being in stable continuous molecular-genetic remission (94%). During the period of time results of transplantations have been significantly improved (p=0.0071). OS after HSCT until year 1997 is 25% while from year 1998 until now is 87.5%. All centers OS of patients after HSCT is 71%. Results of HSCT in children with CML obtained from the year 1998 at our center are fully comparable with results achieved in large and experienced centers. HSCT remains the only proven and effective method for the treatment of CML. Clinical studies assessing the role of tyrosine kinase inhibitors in children instead of early HSCT should be planned carefully in order to avoid sub-optimal outcomes.</p>}},
  author       = {{Krol, L. and Formankova, R. and Keslova, P. and Vavra, V. and Zemanova, Z. and Michalova, K. and Dobrovolna, M. and Moravcova, J. and Kobylka, P. and Ivaskova, E. and Navratilova, J. and Stary, J. and Sedlacek, P.}},
  issn         = {{0028-2685}},
  keywords     = {{Allogeneic hematopoietic stem cell transplantation; Children; Incidence; Prognosis}},
  language     = {{eng}},
  number       = {{2}},
  pages        = {{101--106}},
  publisher    = {{Vydavatel'stvo Slovenkej Akademie Vied/Veda Publishing House of the Slovak Academy of Sciences}},
  series       = {{Neoplasma}},
  title        = {{Prognosis of pediatric patients transplanted for Ph+ chronic myeloid leukemia in the period from 1989 to 2006 in the Czech Republic}},
  url          = {{http://www.elis.sk/index.php?page=shop.product_details&flypage=flypage.tpl&product_id=1077&category_id=35&option=com_virtuemart&vmcchk=1&Itemid=1}},
  volume       = {{55}},
  year         = {{2008}},
}