Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman
European Journal of Clinical and Biomedical Sciences
Volume 3, Issue 6, December 2017, Pages: 109-114
Received: Oct. 15, 2017; Accepted: Oct. 27, 2017; Published: Nov. 20, 2017
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Authors
Naglaa Fawaz, Department of Hematology, Sultan Qaboos University Hospital, Muscat, Oman; Department of Haematopathology, Suez Canal University, Ismailia, Egypt
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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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.
Keywords
Pediatric, MDS, Cytopenia, Myelodysplasia, Monosomy 7, Down’s Syndrome
To cite this article
Naglaa Fawaz, Abdulhakim Al Rawas, Muhammad El Shinawi, Mathew Zachariah, Yasser Wali, Salam Alkindi, Anil Pathare, Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman, European Journal of Clinical and Biomedical Sciences. Vol. 3, No. 6, 2017, pp. 109-114. doi: 10.11648/j.ejcbs.20170306.12
Copyright
Copyright © 2017 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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