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

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
[1] Williamson PJ, Kruger AR, Reynolds PJ, Hamblin TJ, Oscier DG. Establishing the incidence of myelodysplastic syndrome. Br J Haematol,. 1994; 87:743-745. https://doi.org/10.1111/j.1365-2141.1994.tb06733.x
[2] Alter BP, Giri N, Savage SA, et al. Malignancies and survival patterns in the National Cancer Institute inherited bone marrow failure syndromes cohort study. Br J Haematol. 2010;150:179-188. https://doi.org/10.1111/j.1365-2141.2010.08212.x
[3] Glaubach T, Robinson LJ, Corey SJ. Pediatric myelodysplastic syndromes: they do exist! J Pediatr Hematol Oncol. 2014; 36:1-7. https://doi.org/10.1097/mph.0000000000000046
[4] Hasle H, Wadsworth LD, Massing BG, et al. A population based study of childhood myelodysplastic syndrome in British Columbia, Canada. Br J Haematol. 1999;106:1027–1032. https://doi.org/10.1046/j.1365-2141.1999.01645.x
[5] Passmore SJ, Chessells JM, Kempski H, et al. Paediatric myelodysplastic syndromes and juvenile myelomonocytic leukaemia in the UK: a population-based study of incidence and survival. Br J Haematol. 2003;121:758–767. https://doi.org/10.1046/j.1365-2141.2003.04361.x
[6] Bader-Meunier B, Mielot F, Tchernia G, et al. Myelodysplastic syndromes in childhood: report of 49 patients from a French multicenter study. Br J Haematol 1996; 92: 344–50. https://doi.org/10.1046/j.1365-2141.0000.d01-1480.x
[7] Sasaki H, Manabe A, Kojima S, et al. Myelodysplastic syndrome in childhood: a retrospective study of 189 patients in Japan. Leukemia. 2001;15:1713–1720. https://doi.org/10.1038/sj.leu.2402271
[8] Luna-Fineman S, Shannon KM, Atwater SK, et al. Myelodysplastic and myeloproliferative disorders of childhood: a study of 167 patients. Blood. 1999;93:459–466.
[9] Bennett JM, Catovsky D, Daniel MT, et al. Proposals for the classification of the myelodysplastic syndromes. Br J Haematol. 1982; 51:189-199 https://doi.org/10.1111/j.1365-2141.1982.tb02771.x
[10] Jaffe JS, Harris NL, Stein H, Vardiman JW, eds. World Health Organization Classification of Tumors. Pathology and Genetics of Tumors of Hematopoietic and Lymphoid Tissues. 3rd ed. Lyon, France: IARC Press; 2001.
[11] Hasle H, Niemeyer CM, Chessells JM, et al. A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases. Leukemia. 2003; 17:277-282. https://doi.org/10.1038/sj.leu.2402765
[12] Swerdlow SH, Campo E, Harris NL, et al, eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. Lyon, France: IARC Press; 2008.
[13] Vardiman JW, Thiele J, Arber DA, et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood. 2009;114:937-951. https://doi.org/10.1182/blood-2009-03-209262
[14] Mitelman F, editor. ISCN (1995): An international system for human cytogenetic nomenclature. Basel: S Karger; 1995.
[15] Niemeyer CM, Baumann I. Myelodysplastic syndrome in children and adolescents. Semin Hematol. 2008;45:60–70. https://doi.org/10.1053/j.seminhematol.2007.10.006
[16] Pitman SD, Victorio A, Rowsell E, et al. 5qsyndrome in a child with slowly progressive pancytopenia: a case report and review of the literature. J Pediatr Hematol Oncol. 2006; 28: 115–119. https://doi.org/10.1097/01.mph.0000210410.48877.15
[17] Kardous G, Baumann I, Passmore SJ, et al. Refractory anemia in childhood: a retrospective analysis of 67 patients with particular reference to monosomy 7. Blood. 2003;102:1997-2003. https://doi.org/10.1182/blood-2002-11-3444
[18] Gohring G, Michalova K, BeverlooHB, et al. Complex karyotype newly defined: the strongest prognostic factor in advanced childhood myelodysplastic syndrome. Blood. 2010;116:3766-3769. https://doi.org/10.1182/blood-2010-04-280313
[19] Boultwood J, Lewis S, Wainscoat JS. The 5q- syndrome. Blood. 1994; 84:3253-3260.
[20] Delhommeau F, Dupont S, Della Valle V, et al. Mutation in TET2 in myeloid cancers. N Engl J Med. 2009 May 28;360:2289-2301. https://doi.org/10.1056/nejmoa0810069
[21] Verburgh E, Achten R, Louw VJ, et al. A new disease categorization of low-grade myelodysplastic syndromes based on the expression of cytopenia and dysplasia in one versus more than one lineage improves on the WHO classification. Leukemia. 2007;21:668–677. https://doi.org/10.1038/sj.leu.2404564
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    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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    T1  - Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman
    AU  - Naglaa Fawaz
    AU  - Abdulhakim Al Rawas
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    JO  - European Journal of Clinical and Biomedical Sciences
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    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
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Author Information
  • Department of Hematology, Sultan Qaboos University Hospital, Muscat, Oman

  • Department of Child Health, Sultan Qaboos University Hospital, Muscat, Oman

  • Department of Child Health, Sultan Qaboos University Hospital, Muscat, Oman

  • Department of Child Health, Sultan Qaboos University Hospital, Muscat, Oman

  • Department of Child Health, Sultan Qaboos University Hospital, Muscat, Oman

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

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

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