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S Polychronopoulou

Publications and source records attributed to S Polychronopoulou.

12 recordsLinked to original sources
PubMed2005

p16 inactivation associated with aggressive clinical course and fatal outcome in TEL/AML1-positive acute lymphoblastic leukemia.

The authors describe a 7-year-old boy with TEL/AML1-positive pre-B acute lymphoblastic leukemia, with hemizygous 9p21 deletion at presentation and no p16(INK4A) protein expression. Despite an initial response to a standard chemotherapy regimen, the patient suffered two hematologic relapses and died 34 months after diagnosis. The authors discuss the possibility that complete p16(INK4A) gene inactivation may adversely modify the prognostic significance of TEL/AML1 fusion in childhood acute lymphoblastic leukemia, and present evidence from clinical and in vitro observations in favor of this assumption.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2004

An integrated evaluation of socioeconomic and clinical factors in the survival from childhood acute lymphoblastic leukaemia: a study in Greece.

An evaluation of the role of socioeconomic factors in the survival of children with leukaemia, controlling for major clinical prognostic indicators, has been attempted in very few studies and the role of these factors may be different in various cultural settings. Our investigation aims to study the independent role of socioeconomic factors on the prognosis of childhood acute lymphoblastic leukaemia (ALL) in Greece. During a 7-year period (1996-2002) 293 cases of incident ALL were diagnosed and followed up in four Childhood Haematology-Oncology Units, which covered over half of all childhood ALL cases nationwide. At the time of diagnosis, information concerning age, gender, maternal schooling, maternal marital status, sibship size, distance of residence from the treating centre, attendance of day care centre and clinical information was recorded. The influence of these factors on survival was studied by modelling the data through Cox's proportional-hazards regression. After adjustment for clinical prognostic factors, children of mothers who were not currently married, were of low educational level or were living far from the treating centre tended to have lower survival (P-values 0.02, 0.14 and 0.08, respectively). There was also evidence that two factors that are predictive of disease occurrence, that is sibship size and attendance of day care centre, may also predict survival (P-values 0.04 and 0.26, respectively). In conclusion, socioeconomic factors are likely to influence survival from ALL at least in some sociocultural contexts. Moreover, there is evidence that factors that could affect incidence of ALL through modulation of herd immunity may also have prognostic implications for this disease.

Age Factors↗
PubMed2004

Clinical and morphological features of paediatric myelodysplastic syndromes: a review of 34 cases.

BACKGROUND: The clinical and morphological spectrum of myelodysplastic syndromes (MDS) during childhood has not yet been completely documented. We herein present the clinical features and morphological data from peripheral blood (PB), bone marrow aspirates (BMA) and bone marrow biopsies (BMB) of a series of paediatric MDS patients, with particular emphasis on their specific morphological characteristics and their diverse underlying genetic background. PATIENTS AND METHODS: Thirty-four patients with MDS (median age 8.45 y) were consecutively diagnosed and treated during a period of 15 y (1988-2002). Diagnosis was based on clinical manifestations, morphology of PB, BMA and BMB, and cytogenetic analysis of BM cells. Clonogenic methylcellulose cell cultures were performed in 23/34 patients. Patients were categorized into group A [26 primary/de novo MDS, i.e. refractory anaemia (RA) 18, RA with excess of blasts (RAEB) 2, RAEB in transformation (RAEB-t) 6] and group B (8 secondary MDS, i.e. RA 4, RAEB 1, RAEB-t 3). Treatment options varied according to protocols active during the period of the study and the availability of a suitable BM donor. Survival probabilities were estimated using the Kaplan-Meier method. RESULTS: Dysplastic features of the erythroid, myeloid and megakaryocytic lineage were detected at BMA in 85%, 50% and 90% of the patients, respectively, while decreased cellularity was found at BMB in 21/34 patients (60%). RA patients of group A presented at BMB significant hypocellularity (14/18) as a prominent finding due to decrease of the myeloid (13/18 patients) and/or the megakaryocytic (14/18 patients) lineage. Hypocellularity in RA was accompanied by dysplasia of the erythroid (17/18 patients) and megakaryocytic (16/18 patients) lineage, the presence of abnormal localization of immature precursors (ALIP, 8/18 patients), fibrosis (5/18) and stromal changes (11/18). Chromosomal aberrations were revealed in 17/34 patients, of which monosomy 7 was present in seven. Cell cultures demonstrated abnormal myeloid and/or erythroid in vitro clonal growth pattern in all the examined patients. An associated disorder or inherited disease, was identified in 14/26 patients (54%) with primary MDS. Cumulative survival of group A patients was 44.2% (RA 66.6%, RAEB/RAEBt 14.6%; p = 0.001), and of the whole group 42.4%, at 14 y. CONCLUSIONS: Hypocellularity of significant degree is a constant and prominent feature among paediatric MDS, especially those with RA. A large variety of associated disorders underlies the clinical appearance of paediatric MDS, reflecting their marked heterogeneity. RA represents the prominent subtype during childhood (69% in this study), and it appears to have the best prognosis, while prognosis of RAEB/RAEBt remains extremely poor.

Adolescent↗
PubMed1996

Secondary malignancies in a child with Hodgkin's disease: T-cell lymphoma and myelodysplastic syndrome evolving into acute nonlymphoblastic leukaemia.

Hodgkin's disease (HD) has been linked to an increase risk of second malignant neoplasms (SMN), especially non-Hodgkin's lymphoma (NHL) and acute nonlymphoblastic leukaemia (ANLL). The mutagenic property of cytotoxic therapy as well as defective immunity have been implicated as playing a major role in the development of SMN in patients previously treated for HD. We report a case of a 14-year-old girl with HD who developed two different second malignancies within a latent period of 28 months following HD diagnosis. The patient presented initially with bilateral cervical and supraclavicular as well as mediastinal and paraaortic lymphadenopathy. She was staged as IIIA, nodular sclerosing type HD, and was given eight alternative cycles of MOPP-ABVD followed by "mantle" field radiotherapy to a total dose of 3.3 Gy plus 0.4 Gy to the upper mediastinum. Within 8 months following the completion of therapy, a period of myelodysplasia and progressive severe immune deficiency, considered as a result of initial treatment, occurred. Eighteen months after HD diagnosis while the patient was continuously neutropenic and heavily immunocompromised, a peripheral T-cell lymphoma of the angiocentric immunoproliferative lesion type (AIL) Grade III, appeared in both lungs within and beyond the radiation field, with no evidence of HD in biopsy specimens. After institution of a new chemotherapy regimen (L17M), a satisfactory response regarding NHL lesions was noted. However, 10 months later the myelodysplastic syndrome (MDS) accompanied by complex chromosomal abnormalities evoluted to frank ANLL with a rapid fatal course. This case supports the hypothesis that combined modality treatment accompanied by severe immunodeficiency may result in the development of multiple second malignancies even within a very short latent period, especially in a subgroup of HD patients who may be particularly increased risk of second cancers.

Acquired Immunodeficiency Syndrome↗
PubMed1993

Auxologic data and hormonal profile in long-term survivors of childhood acute lymphoid leukemia.

PURPOSE: Eighty patients, 50 girls and 30 boys 13.5 +/- 4.09 years of age, on continuous first remission, were evaluated 7.9 +/- 3.2 years after the diagnosis of acute lymphoid leukemia (ALL). PATIENTS, METHODS AND RESULTS: All patients were treated according to current protocols. Cranial irradiation dose was 1,800 rad in 10 fractions for 50 patients, and 2,400 rad in 18 fractions for the remaining 30. The mean percentile and SDS for height were 42.5 +/- 26 and -0.3 +/- 1.1 for boys, and 37.8 +/- 15.3 and -0.54 +/- 0.9 for girls, respectively. The final height and the corresponding SDS was 171 +/- 5.75 cm and -0.35 +/- 0.8 for boys, and 158 +/- 7.1 cm and -0.89 +/- 1.1 for girls, respectively. Using skin fold thickness, obesity was observed in a high percentage of patients. The head circumference (HC) percentile was 36.8 +/- 24 for boys and 40.9 +/- 29 for girls, and was even lower (30.96 +/- 25.5) for those who received a radiation dose of 2,400 rad. Menarche was earlier in girls with ALL than in normal girls (11.6 +/- 1.5 vs. 12.4 +/- 1.02 years). Testicular size was within normal limits, except in three boys whose size was at or below the third percentile. In two of them, testes had been irradiated. The mean thyroxin, thyroid-stimulating hormone, and prolactin values were within normal limits (8.6 +/- 2 micrograms/dl, 2.6 +/- 1.1 microU/dl, and 5.9 +/- 4.8 ng/ml, respectively). The somatomedin-C values for patients in the prepubertal stage were 1.49 +/- 0.85 versus 0.96 +/- 0.6 in the controls (p < 0.05), whereas for patients in the pubertal stage they were 1.92 +/- 1 versus 1.88 +/- 1 in the controls. The sex steroid and dehydroepiandrosterone sulfate values were within normal limits. In a high percentage of children, follicle-stimulating hormone and luteinizing hormone values were above the normal range for their age, sex, and pubertal stage. The mean glycosylated hemoglobin values were normal. CONCLUSIONS: 1. Linear growth, although impaired in the group as a whole, is within the normal range for the majority of children with ALL. A small percentage of children have significantly impaired growth, and must be identified early and receive appropriate therapy. 2. Obesity is more frequently present in patients with ALL. 3. HC is lower than expected, indicating impaired brain growth, which is worse in children irradiated with 2,400 rad. 4. Menarche is earlier and the gonadotrophin level is higher than normal, suggestive of either hypothalamic dysfunction, subtle ovarian failure, or both.

Adolescent↗
PubMed1991

Paediatric urolithiasis in Greece.

A group of 44 patients, aged from 10 months to 14 years, underwent surgery for urinary calculi over a 7-year period (1982-1989). Eleven patients had bilateral or multiple calculi (total number of stones = 55, 20 of which were staghorn). Metabolic disorders (n = 25) and Proteus urinary infection (n = 15) were the 2 factors most often associated with lithiasis. Of the 55 stones, 51 were removed by open surgery. Complete stone clearance was achieved in 29 of 36 kidneys. Follow-up periods in the remaining 7 renal units (with small residual fragments) ranged from 3.5 to 7.5 years (mean 6.2) and revealed stone recurrence in only 2 patients. Evaluation of childhood urolithiasis should include thorough metabolic investigation and sound surgical judgment; effective management requires prolonged post-operative follow-up.

Adolescent↗
PubMed1990

Surgical treatment of ectopic ureteroceles and the role of heminephrectomy with subtotal ureterectomy.

We evaluated 16 patients, 2 weeks to 8 years of age (median: 18 months), operated on for ectopic ureterocele. Five patients, in whom the upper kidney, drained by the ureter with the ureterocele, was viable, were submitted to excision of the ureterocele with reimplantation of the double ureter (n = 4) or high ureteroureterostomy (n = 1). A 2-week-old baby with a non-functioning kidney was treated by nephroureterectomy. The remaining 10 children with a non-functioning upper part of a duplex kidney were submitted to heminephrectomy with subtotal ureterectomy. In this group of patients no problems arose from the retained ureteral stump or the decompressed ureterocele, and vesicoureteral reflux accompanying the ureterocele resolved postoperatively in 6 of 10 ureters.

Choristoma↗
PubMed1990

Evaluation of open surgery for staghorn calculi in children.

We evaluated the results of open surgery in 19 children with staghorn calculi. The median age was 3.5 years (range 10 months to 14 years). One patient had bilateral calculi, hence 20 renal units were operated upon. Stone clearance was achieved in 19 cases by means of a Gil-Vernet pyelocalycolithotomy, alone (n = 10) or in combination with limited radial nephrotomies (n = 9). Extended nephrolithotomy was performed in only 1 case. Complete stone clearance was achieved in 13 kidneys (65%). Long-term follow-up showed that there was no stone recurrence in any of the 13 renal units rendered completely stone free at surgery. There was symptomatic stone recurrence in 1 of the remaining 7 renal units with small residual fragments left behind, while in 2 others the residual fragments were voided spontaneously. Preoperative and postoperative assessment with 99mTc-DMSA renal scans confirmed that no functional loss resulted in any of the operated kidneys. Open surgery proved to be safe and effective in the treatment of staghorn calculi in children.

Child, Preschool↗
PubMedDate not supplied

Conventional cytogenetics and fluorescence in situ hybridization in persistent cytopenias and myelodysplastic syndromes in childhood.

Accurate detection of the abnormal clone in children with persistent cytopenia (PC) may confirm the diagnosis of myelodysplastic syndrome (MDS) and determine prognosis and evolution of the disease. Bone marrow (BM) samples were obtained from 65 children, 11 of which were finally diagnosed as primary or secondary MDS. Ten to 20 G-banded metaphases were analyzed and FISH was performed using a-satellite probes for chromosomes 7 and 8. Conventional cytogenetic analysis (CCA) was successful in 40/65 samples, revealing clonal aberrations in 3 patients with MDS. FISH was successful in all cases, detecting monosomy 7 and trisomy 8 abnormal clones in 5 patients. Abnormalities were identified in 3/6 children with primary MDS and 3/5 with secondary MDS. None of the patients with PC of etiology other than MDS had a clonal abnormality in the BM. The results confirm the high incidence of chromosome abnormalities in childhood MDS and the sensitivity of FISH in detecting minor abnormal clones.

Adolescent↗
PubMedDate not supplied

Chromosome fragility and predisposition to childhood malignancies.

In order to clarify the possible connection between autosomal folate sensitive Fragile Sites (FS) and genetic susceptibility to haemopoetic disease in children we investigated the frequency and distribution of FS in the Peripheral Blood Lymphocytes (PBL) of 56 children with newly diagnosed and untreated haematologic malignancies and their parents. The incidence was compared with that of 146 normal controls (children and adults). In all patients the Bone Marrow (BM) karyotype was also determined. Heritable FS were detected in 49 patients (87.5%). 20 children had more than one FS and in all cases it was inherited from one of their parents, although there was a significant excess of transmitting mothers. 19 different FS were identified: 14 common, 4 rare and one, 22q11, which has not been previously reported, but it is considered as important as it coincides with the cancer breakpoint resulting in the formation of the Philadelphia (Ph) chromosome. The frequency of FS in the PBL of the patients was significantly higher than in the controls and this increase was independent of any abnormality detected in the malignant cells of the BM. However, patients with an abnormal BM karyotype displayed increased frequency of FS induction as compared to patients with a normal karyotype. In three cases the heritable FS was found to be at or near the breakpoints of the chromosomal rearrangements detected in the malignant cells. The findings are discussed with regard to cancer specific breakpoints, oncogene loci and sites where viral DNA can be inserted to the genome. The results of this study suggest that autosomal folate sensitive FS may increase the risk for haematologic malignancies through a complex mechanism which remains to be clarified.

Adult↗