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Yigal Dror

Publications and source records attributed to Yigal Dror.

24 records · Page 2Linked to original sources
PubMed2002

Abnormal telomere shortening in leucocytes of children with Shwachman-Diamond syndrome.

Haemopoietic dysfunction, ranging from single-lineage cytopenia to severe aplasia and/or myelodysplasia (MDS), is prominent in Shwachman-Diamond syndrome (SDS). To assess haemopoietic stem cell proliferation in SDS, we compared leucocyte telomere length in 12 patients with SDS to that of 41 controls, using an in-gel hybridization technique. SDS patients had an age-adjusted mean telomere length 1.4 kilobase pairs (kbp) shorter than controls (P < 0.0001). Patients with'non-severe' SDS (one- or two-lineage cytopenias; no MDS) had shortened telomeres (-1.4 kbp; P = 0.0004), as did those with 'severe' SDS. We conclude that stem cell hyperproliferation is a feature of SDS from its outset.

Adolescent↗
PubMed2002

A practical, comprehensive classification for pediatric myelodysplastic syndromes: the CCC system.

PURPOSE: Pediatric myelodysplastic syndromes (MDS) are biologically diverse. The French-American-British (FAB) classification of adult forms of MDS is not always applicable because many pediatric patients do not fit into any of the categories. To circumvent the FAB schema and other flawed formats, the authors developed a practical classification system for childhood MDS. PATIENTS AND METHODS: The authors analyzed 40 children with MDS diagnosed in Toronto between 1988 and 1998 to test the utility of the classification. Children were classified according to three main features: category, cytology, and cytogenetics. RESULTS: Using this system the authors were able to classify all 40 patients; about half could not be classified by FAB. Patients could also be longitudinally classified by serial analysis to show progression of disease. Juvenile myelomonocytic leukemia was excluded because of its known myeloproliferative pathogenesis. Chronic myelomonocytic leukemia, which almost never occurs in children, was also omitted. Also excluded were other chronic myeloproliferative disorders and any cytopenias without malignant potential. CONCLUSIONS: Based on these data, the CCC system appears to have prognostic potential; children with advanced class and cytogenetic abnormalities had a poorer outcome. The authors urge international adoption of this system for uniformity in clinical practice and reporting purposes.

Adolescent↗
PubMedDate not supplied

Transient myelodysplastic syndrome associated with isochromosome 7q abnormality.

Myelodysplastic syndrome (MDS) in childhood is a rare hematological condition that is often associated with cytogenetic abnormalities, the most common being monosomy 7/del(7q). The clinical course of MDS can vary from stable disease to rapid progression into acute leukemia. Rarely, spontaneous remission of MDS has been observed. The authors report the first case of a transient MDS associated with a clonal marrow cytogenetic abnormality consisting of isochromosome 7q in a previously well child. Without intervention, the bone marrow cytogenetics reverted to normal and there was complete hematologic recovery. This case illustrates the importance of close follow-up in a child presenting with MDS, to detect spontaneous recovery or evolution of the disease.

Bone Marrow↗
PubMedDate not supplied

A practical, comprehensive classification for pediatric myelodysplastic syndromes: the CCC system.

PURPOSE: Pediatric myelodysplastic syndromes (MDS) are biologically diverse. The French-American-British (FAB) classification of adult forms of MDS is not always applicable because many pediatric patients do not fit into any of the categories. To circumvent the FAB schema and other flawed formats, the authors developed a practical classification system for childhood MDS. PATIENTS AND METHODS: The authors analyzed 40 children with MDS diagnosed in Toronto between 1988 and 1998 to test the utility of the classification. Children were classified according to three main features: category, cytology, and cytogenetics. RESULTS: Using this system the authors were able to classify all 40 patients; about half could not be unclassified by FAB. Patients could also be longitudinally classified by serial analysis to show progression of disease. Juvenile myelomonocytic leukemia was excluded because of its known myeloproliferative pathogenesis. Chronic myelomonocytic leukemia, which almost never occurs in children, was also omitted. Also excluded were other chronic myeloproliferative disorders and any cytopenias without malignant potential. CONCLUSIONS: Based on these data, the CCC system appears to have prognostic potential; children with advanced class and cytogenetic abnormalities had a poorer outcome. The authors urge international adoption of this system for uniformity in clinical practice and reporting purposes.

Acute Disease↗