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Biomedical subjects

G Hicsonmez

Publications and source records attributed to G Hicsonmez.

13 recordsLinked to original sources

Tetrasomy 8 as a primary chromosomal abnormality in a child with acute megakaryoblastic leukemia. a case report and review of the literature.

Tetrasomy 8 is a relatively rare chromosomal abnormality in hematological disorders, and is mostly associated with myeloid malignancies and poor prognosis. In a number of cases, tetrasomy 8 has been reported as an accompanying anomaly with other chromosomal changes. In this report, we describe a 14-year-old girl with acute megakaryoblastic leukemia associated with tetrasomy 8 (primary) and trisomy 6, 19 and 20. She died 6 months after diagnosis, suggesting a relatively poor prognosis for AML with tetrasomy 8. To the best of our knowledge, this is the first report of a tetrasomy 8 abnormality associated with subtype FAB M7. Interestingly, this abnormality has not been previously reported in childhood AML patients.

Adolescent↗

Myelodysplastic syndrome associated with monosomy 7 in a child with Bloom syndrome.

Bloom syndrome is a genomic instability syndrome associated with predisposition to development of various types of malignancy. In this report, we described a 7-year-old boy with Bloom syndrome (BS) and myelodysplastic syndrome (MDS) associated with monosomy 7 and loss of the Y chromosome. To our knowledge, this was the first case with BS showing monosomy 7 and MDS during the early childhood period.

Anemia, Refractory, with Excess of Blasts↗

Loss of maternal allele in a child with myelodysplastic syndrome and monosomy 7.

Monosomy 7 or partial deletion of the long arm of chromosome 7 is frequently described in children with myelodysplastic syndrome and acute myeloblastic leukemia. Parental origin of chromosome 7 in children with sporadic monosomy 7 has been examined very rarely. To investigate if monosomy 7 shows parent-of-origin, we have studied a female child with monosomy 7 and de novo myelodysplastic syndrome by a series of polymorphic polymerase chain reaction markers. We found loss of maternal allele and discussed the results with the previous reports.

Alleles↗

Primary myelodysplastic syndrome in children: the clinical experience in 33 cases.

We describe the clinicomorphological features in 33 cases of primary myelodysplastic syndrome classified according to the FAB classification which presented to a single centre over a 12 year period. Presenting features were typically related to pancytopenia although hepatosplenomegaly and granulocytic sarcomas were far more prevalent than in the adult population. Morphological assessment of the peripheral blood and the bone marrow showed seven patients had refractory anaemia (RA), 13 patients had RA with excess of blasts (RAEB), nine patients had RAEB in transformation (RAEB-t) and four patients had chronic myelomonocytic leukaemia (CMML). The overall mean survival was short (9.9 months) in all the subgroups and the leukaemic transformation rate was high. None of the patients scored 0-1 according to the Bournemouth Scoring System; four patients scored 2 whereas 29 patients scored 3 to 4. We conclude that unlike adults, the myelodysplastic syndromes in children run an aggressive clinical course, irrespective of the FAB subtype, and the pathogenesis of these diseases in paediatric practice warrants scientific scrutiny. Intensive chemotherapy such as the one used in de novo-AML lead to complete remission in some children and these early results suggest that this should be the treatment of choice in paediatric MDS.

Adolescent↗

Acute leukemia in two patients with hemophilia.

A 10-year-old classic hemophiliac and 1.5-year-old child with hemophilia B who developed acute lymphocytic and acute myelomonocytic leukemia respectively are presented. No changes in coagulation status of the patients were observed. It is suggested that hemophiliacs should be regarded as "population at risk" for the development of leukemia.

Blood Coagulation Tests↗

The effect of high-dose methylprednisolone treatment on GM-CSF level in children with acute leukemia: a pilot study.

High-dose methylprednisolone therapy (HDMP) induces acceleration of leukocyte recovery in acute lymphoblastic leukemia (ALL) and the differentiation of myeloblasts to mature granulocytes in acute myeloblastic leukemia (AML). These effects of corticosteroids have been shown to be due to the enhanced colony-stimulating activity (CSA) and responses to corticosteroids in some patients with aplastic anemia and myelodysplastic syndromes (MDS) have been related to increased CSA activity. We measured the serum (granulocyte-macrophage colony-stimulating factor (GM-CSF) levels by a sandwich linked immunoabsorbent assay (ELISA) in patients with ALL and AML at presentation and following high-dose methylprednisolone (HDMP) therapy. Serum GM-CSF levels at presentation in the ten cases studied ranged between 160 and 700 pg/ml (mean 418.5 +/- 252.5). One week following HDMP therapy GM-CSF levels increased to between 260 and 950 pg/ml (733.5 +/- 203.2). Four weeks after therapy the GM-CSF levels increased to between 470 and 1350 pg/ml (911 +/- 278.7). GM-CSF levels were markedly elevated one week after HDMP in the patients with ALL, suggesting that in addition to the lymphotoxic effects on leukemic blasts, the acceleration in neutrophil recovery may be due to release of GM-CSF induced by HDMP and its effects on myeloid progenitors.

Acute Disease↗

Cerebrospinal fluid lactic dehydrogenase activity in children with acute lymphoblastic leukemia treated for cranial prophylaxis.

Total lactic dehydrogenase (TLDH) activity in cerebrospinal fluid (CSF) has been prospectively studied in order to determine whether it could be a biochemical marker for brain damage due to cranial prophylaxis in children with acute lymphoblastic leukemia (ALL). TLDH activity has been measured in 15 patients before prophylaxis, in 15 patients after the prophylaxis which consisted of cranial radiotherapy (2400 rads) and intrathecal methotrexate (0.5 mg/kg-dose, 5 doses), in 8 patients after radiotherapy alone (2400 rads) and in 9 patients after intrathecal methotrexate (0.5 mg/kg-dose, 5 doses) alone. TLDH activity in CSF of combined prophylaxis group has been found to be higher than the ones before study (p less than 0.05). There were insignificant elevations of TLDH activity in the other two groups (p greater than 0.05; p greater than 0.05). This result indicated combined cranial prophylaxis seemed to be more toxic than the other prophylaxis regimens when they were used alone.

Adolescent↗