European Journal of Clinical and Biomedical Sciences 2017; 3(6): 109-114 http://www.sciencepublishinggroup.com/j/ejcbs doi: 10.11648/j.ejcbs.20170306.12 ISSN: 2575-4998 (Print); ISSN: 2575-5005 (Online) Pediatric Myelodysplastic Syndrome: Cytomorphological Correlation with Outcome from a Single Tertiary Institution in Oman Naglaa Fawaz 1, 2 , Abdulhakim Al Rawas 3 , Muhammad El Shinawi 3 , Mathew Zachariah 3 , Yasser Wali 3 , Salam Alkindi 4 , Anil Pathare 1, * 1 Department of Hematology, Sultan Qaboos University Hospital, Muscat, Oman 2 Department of Haematopathology, Suez Canal University, Ismailia, Egypt 3 Department of Child Health, Sultan Qaboos University Hospital, Muscat, Oman 4 College of Medicine & Health Sciences, Sultan Qaboos University, Muscat, Oman Email address: avp16@hotmail.com (A. Pathare) * Corresponding author 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 Received: October 15, 2017; Accepted: October 27, 2017; Published: November 20, 2017 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 1. Introduction Although MDS is one of the most common hematopoietic disorders in the elderly with a median age of 70 years at diagnosis, it is less frequently seen in pediatrics and is known to occur both as de novo and secondary forms. [1, 2] Presence of bone marrow failure, which manifests itself as cytopenia is an important indication for bone marrow investigation is this setting. The causes of cytopenia are varied and differ considerably between childhood and adulthood, as inherited bone marrow failure syndromes [IBMFS] are important causes of bone marrow failure in the pediatric age group but play only a minor role in adulthood. [2, 3] Furthermore, inherited bone marrow failure syndromes have a varied propensity to develop secondary MDS. Consequently, the diagnosis of pediatric MDS is really challenging particularly in a country like Oman with high rate of consanguinity and inherited hematological and non- hematological diseases. Moreover, dysplasia is frequently observed in non-MDS conditions including metabolic