Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman

Naglaa Fawaz; Abdulhakim Al Rawas; Muhammad El Shinawi; Mathew Zachariah; Yasser Wali; Salam Alkindi; Anil Pathare

doi:doi:10.11648/j.ejcbs.20170306.12

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Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman

Naglaa Fawaz, Abdulhakim Al Rawas, Muhammad El Shinawi, Mathew Zachariah, Yasser Wali, Salam Alkindi, Anil Pathare

Published in European Journal of Clinical and Biomedical Sciences (Volume 3, Issue 6)

Received: 15 October 2017 Accepted: 27 October 2017 Published: 20 November 2017

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Abstract

Pediatric MDS is currently classified into three distinct groups namely MDS, Juvenile myelomonocytic leukemia (JMML) and Down Syndrome-associated leukemias (DSAL). The aim of this study was to evaluate pediatric MDS patients using the WHO 2008 morphological classification with the clinical outcome. In this retrospective single centre study, 19 pediatric MDS patients (median age 6 years; range 3 months – 16 years) were analyzed for their initial presentation, type of progression, leukemic transformation and overall survival as well as MDS related cytogenetic abnormalities. The median follow-up was 26.5 months. The most common single presentation of childhood MDS was RCC (26%). Leukemic transformation was seen in almost half of this cohort but was completely restricted to the DSAL and RAEB-T subgroups only. The median survival in the MDS group was 50 months, whereas for the entire cohort it was 35 months. RCC cohort showed the best survival with the median of 53.75 months, whereas the Down-related disease cohort showed the worst survival estimates with a median of only 11.25 months. Multivariate analysis showed that gender, platelet count, HbF, bone marrow cellularity, bone marrow blast percentage contributed significantly to the prognosis and survival. The study shows that cytogenetics, including monosomy 7 did not influence the outcome or the overall survival of childhood MDS. We identified that thrombocytopenia and BM blasts >5% were associated with a poor survival in this childhood MDS cohort.

Published in	European Journal of Clinical and Biomedical Sciences (Volume 3, Issue 6)
DOI	10.11648/j.ejcbs.20170306.12
Page(s)	109-114
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2017. Published by Science Publishing Group

Keywords

Pediatric, MDS, Cytopenia, Myelodysplasia, Monosomy 7, Down’s Syndrome

References

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APA Style

Naglaa Fawaz, Abdulhakim Al Rawas, Muhammad El Shinawi, Mathew Zachariah, Yasser Wali, et al. (2017). Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman. European Journal of Clinical and Biomedical Sciences, 3(6), 109-114. https://doi.org/10.11648/j.ejcbs.20170306.12

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ACS Style

Naglaa Fawaz; Abdulhakim Al Rawas; Muhammad El Shinawi; Mathew Zachariah; Yasser Wali, et al. Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman. Eur. J. Clin. Biomed. Sci. 2017, 3(6), 109-114. doi: 10.11648/j.ejcbs.20170306.12

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AMA Style

Naglaa Fawaz, Abdulhakim Al Rawas, Muhammad El Shinawi, Mathew Zachariah, Yasser Wali, et al. Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman. Eur J Clin Biomed Sci. 2017;3(6):109-114. doi: 10.11648/j.ejcbs.20170306.12

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@article{10.11648/j.ejcbs.20170306.12,
  author = {Naglaa Fawaz and Abdulhakim Al Rawas and Muhammad El Shinawi and Mathew Zachariah and Yasser Wali and Salam Alkindi and Anil Pathare},
  title = {Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman},
  journal = {European Journal of Clinical and Biomedical Sciences},
  volume = {3},
  number = {6},
  pages = {109-114},
  doi = {10.11648/j.ejcbs.20170306.12},
  url = {https://doi.org/10.11648/j.ejcbs.20170306.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ejcbs.20170306.12},
  abstract = {Pediatric MDS is currently classified into three distinct groups namely MDS, Juvenile myelomonocytic leukemia (JMML) and Down Syndrome-associated leukemias (DSAL). The aim of this study was to evaluate pediatric MDS patients using the WHO 2008 morphological classification with the clinical outcome. In this retrospective single centre study, 19 pediatric MDS patients (median age 6 years; range 3 months – 16 years) were analyzed for their initial presentation, type of progression, leukemic transformation and overall survival as well as MDS related cytogenetic abnormalities. The median follow-up was 26.5 months. The most common single presentation of childhood MDS was RCC (26%). Leukemic transformation was seen in almost half of this cohort but was completely restricted to the DSAL and RAEB-T subgroups only. The median survival in the MDS group was 50 months, whereas for the entire cohort it was 35 months. RCC cohort showed the best survival with the median of 53.75 months, whereas the Down-related disease cohort showed the worst survival estimates with a median of only 11.25 months. Multivariate analysis showed that gender, platelet count, HbF, bone marrow cellularity, bone marrow blast percentage contributed significantly to the prognosis and survival. The study shows that cytogenetics, including monosomy 7 did not influence the outcome or the overall survival of childhood MDS. We identified that thrombocytopenia and BM blasts >5% were associated with a poor survival in this childhood MDS cohort.},
 year = {2017}
}

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TY  - JOUR
T1  - Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman
AU  - Naglaa Fawaz
AU  - Abdulhakim Al Rawas
AU  - Muhammad El Shinawi
AU  - Mathew Zachariah
AU  - Yasser Wali
AU  - Salam Alkindi
AU  - Anil Pathare
Y1  - 2017/11/20
PY  - 2017
N1  - https://doi.org/10.11648/j.ejcbs.20170306.12
DO  - 10.11648/j.ejcbs.20170306.12
T2  - European Journal of Clinical and Biomedical Sciences
JF  - European Journal of Clinical and Biomedical Sciences
JO  - European Journal of Clinical and Biomedical Sciences
SP  - 109
EP  - 114
PB  - Science Publishing Group
SN  - 2575-5005
UR  - https://doi.org/10.11648/j.ejcbs.20170306.12
AB  - Pediatric MDS is currently classified into three distinct groups namely MDS, Juvenile myelomonocytic leukemia (JMML) and Down Syndrome-associated leukemias (DSAL). The aim of this study was to evaluate pediatric MDS patients using the WHO 2008 morphological classification with the clinical outcome. In this retrospective single centre study, 19 pediatric MDS patients (median age 6 years; range 3 months – 16 years) were analyzed for their initial presentation, type of progression, leukemic transformation and overall survival as well as MDS related cytogenetic abnormalities. The median follow-up was 26.5 months. The most common single presentation of childhood MDS was RCC (26%). Leukemic transformation was seen in almost half of this cohort but was completely restricted to the DSAL and RAEB-T subgroups only. The median survival in the MDS group was 50 months, whereas for the entire cohort it was 35 months. RCC cohort showed the best survival with the median of 53.75 months, whereas the Down-related disease cohort showed the worst survival estimates with a median of only 11.25 months. Multivariate analysis showed that gender, platelet count, HbF, bone marrow cellularity, bone marrow blast percentage contributed significantly to the prognosis and survival. The study shows that cytogenetics, including monosomy 7 did not influence the outcome or the overall survival of childhood MDS. We identified that thrombocytopenia and BM blasts >5% were associated with a poor survival in this childhood MDS cohort.
VL  - 3
IS  - 6
ER  -

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Author Information

Naglaa Fawaz

Department of Hematology, Sultan Qaboos University Hospital, Muscat, Oman
Abdulhakim Al Rawas

Department of Child Health, Sultan Qaboos University Hospital, Muscat, Oman
Muhammad El Shinawi

Department of Child Health, Sultan Qaboos University Hospital, Muscat, Oman
Mathew Zachariah

Department of Child Health, Sultan Qaboos University Hospital, Muscat, Oman
Yasser Wali

Department of Child Health, Sultan Qaboos University Hospital, Muscat, Oman
Salam Alkindi

College of Medicine & Health Sciences, Sultan Qaboos University, Muscat, Oman
Anil Pathare

Department of Hematology, Sultan Qaboos University Hospital, Muscat, Oman

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APA Style

Naglaa Fawaz, Abdulhakim Al Rawas, Muhammad El Shinawi, Mathew Zachariah, Yasser Wali, et al. (2017). Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman. European Journal of Clinical and Biomedical Sciences, 3(6), 109-114. https://doi.org/10.11648/j.ejcbs.20170306.12

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ACS Style

Naglaa Fawaz; Abdulhakim Al Rawas; Muhammad El Shinawi; Mathew Zachariah; Yasser Wali, et al. Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman. Eur. J. Clin. Biomed. Sci. 2017, 3(6), 109-114. doi: 10.11648/j.ejcbs.20170306.12

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AMA Style

Naglaa Fawaz, Abdulhakim Al Rawas, Muhammad El Shinawi, Mathew Zachariah, Yasser Wali, et al. Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman. Eur J Clin Biomed Sci. 2017;3(6):109-114. doi: 10.11648/j.ejcbs.20170306.12

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@article{10.11648/j.ejcbs.20170306.12,
  author = {Naglaa Fawaz and Abdulhakim Al Rawas and Muhammad El Shinawi and Mathew Zachariah and Yasser Wali and Salam Alkindi and Anil Pathare},
  title = {Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman},
  journal = {European Journal of Clinical and Biomedical Sciences},
  volume = {3},
  number = {6},
  pages = {109-114},
  doi = {10.11648/j.ejcbs.20170306.12},
  url = {https://doi.org/10.11648/j.ejcbs.20170306.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ejcbs.20170306.12},
  abstract = {Pediatric MDS is currently classified into three distinct groups namely MDS, Juvenile myelomonocytic leukemia (JMML) and Down Syndrome-associated leukemias (DSAL). The aim of this study was to evaluate pediatric MDS patients using the WHO 2008 morphological classification with the clinical outcome. In this retrospective single centre study, 19 pediatric MDS patients (median age 6 years; range 3 months – 16 years) were analyzed for their initial presentation, type of progression, leukemic transformation and overall survival as well as MDS related cytogenetic abnormalities. The median follow-up was 26.5 months. The most common single presentation of childhood MDS was RCC (26%). Leukemic transformation was seen in almost half of this cohort but was completely restricted to the DSAL and RAEB-T subgroups only. The median survival in the MDS group was 50 months, whereas for the entire cohort it was 35 months. RCC cohort showed the best survival with the median of 53.75 months, whereas the Down-related disease cohort showed the worst survival estimates with a median of only 11.25 months. Multivariate analysis showed that gender, platelet count, HbF, bone marrow cellularity, bone marrow blast percentage contributed significantly to the prognosis and survival. The study shows that cytogenetics, including monosomy 7 did not influence the outcome or the overall survival of childhood MDS. We identified that thrombocytopenia and BM blasts >5% were associated with a poor survival in this childhood MDS cohort.},
 year = {2017}
}

Copy | Download

TY  - JOUR
T1  - Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman
AU  - Naglaa Fawaz
AU  - Abdulhakim Al Rawas
AU  - Muhammad El Shinawi
AU  - Mathew Zachariah
AU  - Yasser Wali
AU  - Salam Alkindi
AU  - Anil Pathare
Y1  - 2017/11/20
PY  - 2017
N1  - https://doi.org/10.11648/j.ejcbs.20170306.12
DO  - 10.11648/j.ejcbs.20170306.12
T2  - European Journal of Clinical and Biomedical Sciences
JF  - European Journal of Clinical and Biomedical Sciences
JO  - European Journal of Clinical and Biomedical Sciences
SP  - 109
EP  - 114
PB  - Science Publishing Group
SN  - 2575-5005
UR  - https://doi.org/10.11648/j.ejcbs.20170306.12
AB  - Pediatric MDS is currently classified into three distinct groups namely MDS, Juvenile myelomonocytic leukemia (JMML) and Down Syndrome-associated leukemias (DSAL). The aim of this study was to evaluate pediatric MDS patients using the WHO 2008 morphological classification with the clinical outcome. In this retrospective single centre study, 19 pediatric MDS patients (median age 6 years; range 3 months – 16 years) were analyzed for their initial presentation, type of progression, leukemic transformation and overall survival as well as MDS related cytogenetic abnormalities. The median follow-up was 26.5 months. The most common single presentation of childhood MDS was RCC (26%). Leukemic transformation was seen in almost half of this cohort but was completely restricted to the DSAL and RAEB-T subgroups only. The median survival in the MDS group was 50 months, whereas for the entire cohort it was 35 months. RCC cohort showed the best survival with the median of 53.75 months, whereas the Down-related disease cohort showed the worst survival estimates with a median of only 11.25 months. Multivariate analysis showed that gender, platelet count, HbF, bone marrow cellularity, bone marrow blast percentage contributed significantly to the prognosis and survival. The study shows that cytogenetics, including monosomy 7 did not influence the outcome or the overall survival of childhood MDS. We identified that thrombocytopenia and BM blasts >5% were associated with a poor survival in this childhood MDS cohort.
VL  - 3
IS  - 6
ER  -

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