Article

Primary myelodysplastic syndrome in children--clinical, hematological and histomorphological profile from a tertiary care centre in India.

All India Institute of Medical Sciences, Department of Hematology, New Delhi, 110029, India.
Hematology (Impact Factor: 1.19). 01/2006; 10(6):495-9. DOI: 10.1080/10245330500155556
Source: PubMed

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.

0 Followers
 · 
78 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: The myelodysplastic syndromes (MDS) are a group of clonal disorders characterized by ineffective haematopoiesis, cytopenias, morphologic dysplasia and leukemic transformation. Difficulties exist in classifying and prognosticating MDS. This study was done to evaluate FAB and WHO classifications and the role of infection especially tuberculosis contributing to secondary myelodysplasia. The clinico-hematological profile of all cases (n=78) of MDS diagnosed over the last one and a half years was analyzed. This included 73 cases of primary MDS and five cases of infection associated myelodysplasia. There were 50 male and 28 female patients. Mean age at presentation was 46.1 years (range: 9 to 82 years). Out of 73 cases, two progressed to AML during the study period. Seventy cases could be classified based on FAB and 62 based on WHO criteria. Five cases of FAB-RAEBt were AML by FAB. One case not classifiable as per FAB could be categorized by WHO and four cases not classifiable as WHO could be categorized by FAB classification. All fulfilled the minimal diagnostic criteria for MDS. The commonest subtype of MDS was RA by FAB (55.7%) and RCMD (21%) and MDS-U (21%) by WHO. Four patients with tuberculosis and one with HIV showed significant myelodysplasia along with reactive changes. The consensus proposal of minimal diagnostic criteria for MDS was most helpful in cases difficult to diagnose and classify. Coexisting infection especially tuberculosis causing secondary myelodysplasia needs to be kept in mind especially in the Indian subcontinent.
    Hematology (Amsterdam, Netherlands) 07/2009; 14(3):145-9. DOI:10.1179/102453309X402232 · 1.19 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Cytogenetic analysis was performed on 224 bone marrow (BM) of Tunisian patients with de novo myelodysplastic syndrome (MDS) at our institution from January 1993 to December 2006. According to French-American-British (FAB) criteria, there were 36% of patients with refractory anaemia (RA), 26% with refractory anaemia with excess of blasts (RAEB), 10% with refractory anaemia with ringed sideroblasts (RARS), 12% with chronic myelomonocytic leukaemia (CMML), 9% refractory anaemia with excess of blasts in transformation (RAEB-t) and 7% of unclassified MDS. A clonal chromosomal abnormality was observed in 51% of the patients. The most frequent karyotypic change was 5q- in 30 cases (13%), followed by -7/7q- in 17 cases (8%), del(12p) in 8 cases (4%), del(20q) and trisomy 8 in 7 cases each (3%), i(17q) in 2 cases (1%) and -y in only one case (0.4%). This is the first large comparative series of MDS from an Arab country, with cytogenetic analysis showing haematological and cytogenetic features similar to those of MDS population of European or mixed European-subsaharian African origin (like Brazil), but different from those seen in Eastern populations.
    Leukemia Research 07/2008; 32(12):1824-9. DOI:10.1016/j.leukres.2008.05.002 · 2.69 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Myelodysplastic syndrome (MDS) comprises of a heterogeneous group of bone marrow disorders resulting from a clonal stem cell defect characterised by cytopenias despite a relatively hypercellular marrow, ineffective hematopoiesis, morphological dysplasia in the marrow elements, no response to hematinics such as iron, B12 or folic acid and risk of progression to leukemia. Myelodysplastic syndrome in childhood is extremely rare and accounts for less than 5 % of all hematopoietic neoplasms in children below the age of 14 y. The primary MDS in children, also known as de novo MDS differs from secondary MDS which generally follows congenital or acquired bone marrow (BM) failure syndromes as well as from therapy related MDS, commonly resulting from cytotoxic therapy. MDS associated with Down syndrome which accounts for approximately one-fourth of cases of childhood MDS is now considered a unique biologic entity synonymous with Down syndrome-related myeloid leukemia and is biologically distinct from other cases of childhood MDS. Refractory cytopenia of childhood (RCC) is the commonest type of MDS. Genetic changes predisposing to MDS in childhood remain largely obscure. Monosomy 7 is by-far the commonest cytogenetic abnormality associated with childhood MDS; however most cases of RCC show a normal karyotype. Complex cytogenetic abnormalities and trisomy 8 and trisomy 21 are also occasionally observed. The most effective and curative treatment is Hematopoietic stem cell transplantation and this is particularly effective in children with the monosomy 7 genetic defect as well as those displaying complex karyotype abnormalities provided it is instituted early in the course of the disease.
    The Indian Journal of Pediatrics 08/2013; 80(9). DOI:10.1007/s12098-013-1130-8 · 0.92 Impact Factor