title: Primary myelodysplastic syndrome in children--clinical, hematological and histomorphological profile from a tertiary care centre in India.
abstract: We describe the clinical, hematological and histomorphological features in children of primary myelodysplastic syndrome (MDS) seen at the All India Institute of Medical Sciences over three years (Jan 2001-Jan 2004). Twenty-one patients of primary MDS aged 17 year or less were classified using the latest proposed WHO classification for Pediatric MDS. The median age was 9 years with male predominance (80%). Pallor was present in all the cases while fever and bleeding diathesis was present in more than 50% of the cases. Morphological assessment of the peripheral blood showed macrocytosis in 50%, pancytopenia in 15% and blast cells in 45% of cases. A complete analysis of clinical features in conjunction with the bone marrow profile revealed 8 cases of refractory cytopenia (RC), 3 cases of refractory anemia with excess blasts (RAEB), 5 cases of refractory anemia with excess blasts in transformation (RAEB-T), 4 cases of Juvenile myelomonocytic leukemia (JMML) and a solitary cases of acute myeloid leukemia (AML) in Downs syndrome. These children were followed up from 1-36 months (mean 15 months). Three patients of RAEB-T progressed to AML within 3-4 months. RC had the best prognosis and all are alive and under regular follow up. The solitary case of AML of Downs syndrome died 1.5 months after initial diagnosis. All 3 cases of RAEB are under regular follow-up and doing well. Three cases of RAEB-T died (all had progressed to AML); the remaining 2 cases were lost to follow up. Of the 4 cases of JMML 1 died within 6 months of diagnosis; the other 3 cases are under regular follow up of whom 1 has a progressively increasing blast count. We conclude that the latest proposed WHO classification for Pediatric MDS can be successfully applied to all cases of primary MDS.
authors.affiliation: All India Institute of Medical Sciences, Department of Hematology, New Delhi, 110029, India.
authors.forename: Tathagata
authors.initials: T
authors.lastname: Chatterjee
authors.forename: M
authors.initials: M
authors.lastname: Mahapatra
authors.forename: Ashish
authors.initials: A
authors.lastname: Dixit
authors.forename: Rahul
authors.initials: R
authors.lastname: Naithani
authors.forename: S
authors.initials: S
authors.lastname: Tyagi
authors.forename: Pravas
authors.initials: P
authors.lastname: Mishra
authors.forename: J
authors.initials: J
authors.lastname: Bhattacharya
authors.forename: P
authors.initials: P
authors.lastname: Dutta
authors.forename: H P
authors.initials: HP
authors.lastname: Pati
authors.forename: D R
authors.initials: DR
authors.lastname: Choudhary
authors.forename: Rajat
authors.initials: R
authors.lastname: Kumar
authors.forename: V P
authors.initials: VP
authors.lastname: Choudhry
authors.forename: R
authors.initials: R
authors.lastname: Saxena
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006403:Hematologic Tests; D006801:Humans; D007150:Immunohistochemistry; D007194:India; D007223:Infant; D008297:Male; D009190:Myelodysplastic Syndromes / Q000097:blood* / Q000473:pathology*; D012189:Retrospective Studies