Pediatrics InternationalVolume 52, Issue 3 p. e171-e174 Gastrointestinal presentation of late acute lymphoblastic leukemia relapse Vassilios Papadakis, Corresponding Author Vassilios Papadakis Departments of Pediatric Hematology-Oncology andVassilios Papadakis, MD, PhD, Department of Pediatric Hematology-Oncology, Goudi, Athens 11527, Greece. Email: [email protected]Search for more papers by this authorEleftheria Roma, Eleftheria Roma First Department of Pediatrics, University of Athens andSearch for more papers by this authorKalliopi Stefanaki, Kalliopi Stefanaki Pathology, Agia Sofia Children's Hospital,Search for more papers by this authorIoanna Panagiotou, Ioanna Panagiotou First Department of Pediatrics, University of Athens andSearch for more papers by this authorSofia Papargyri, Sofia Papargyri Departments of Pediatric Hematology-Oncology andSearch for more papers by this authorGeorge Paterakis, George Paterakis Department of Immunology, G. Gennimatas General Hospital, Athens, GreeceSearch for more papers by this authorSofia Polychronopoulou, Sofia Polychronopoulou Departments of Pediatric Hematology-Oncology andSearch for more papers by this author Vassilios Papadakis, Corresponding Author Vassilios Papadakis Departments of Pediatric Hematology-Oncology andVassilios Papadakis, MD, PhD, Department of Pediatric Hematology-Oncology, Goudi, Athens 11527, Greece. Email: [email protected]Search for more papers by this authorEleftheria Roma, Eleftheria Roma First Department of Pediatrics, University of Athens andSearch for more papers by this authorKalliopi Stefanaki, Kalliopi Stefanaki Pathology, Agia Sofia Children's Hospital,Search for more papers by this authorIoanna Panagiotou, Ioanna Panagiotou First Department of Pediatrics, University of Athens andSearch for more papers by this authorSofia Papargyri, Sofia Papargyri Departments of Pediatric Hematology-Oncology andSearch for more papers by this authorGeorge Paterakis, George Paterakis Department of Immunology, G. Gennimatas General Hospital, Athens, GreeceSearch for more papers by this authorSofia Polychronopoulou, Sofia Polychronopoulou Departments of Pediatric Hematology-Oncology andSearch for more papers by this author First published: 14 June 2010 https://doi.org/10.1111/j.1442-200X.2010.03113.xCitations: 5Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Citing Literature Volume52, Issue3June 2010Pages e171-e174 RelatedInformation

Clinical patient observations led to the hypothesis of increased ALL blast resistance in hyperglycemia (HG) states in pediatric ALL patients, either with preexisting diabetes mellitus (DM) or with steroid induced diabetes (SID). To evaluate the above, the chromometric MTT (methyl thiazole tetrazolium) assay was used Kaspers GJ et al, Leuk Res 1995; 19:175–81.Ficoll separated nucleated bone marrow cells from newly diagnosed pediatric ALL patients were cultured in usual conditions (NL) and in higher glucose levels (233–295 mg/dl), with the addition of titrated prednisolone (PDN) concentrations. The PDN concentration that lyses 50 % of the cultured blast cells (LD50) is the measure of PDN drug sensitivity. PDN concentrations of 0.02–2.0, 2.1–150, above 150 μg/uL indicate high, intermediate and low blast sensitivity to PDN, respectively. Hence, patient blasts with LD50 at >150 μg/uL are resistant to PDN. The MTT assay results have been related to patient outcome (remission induction and event free survival, Pieters R et al Pediatr Oncol 1994; 22:299–308). Twelve newly diagnosed pediatric patients, with median age 6.4 years (range, 1.7 to 16.5 years) with common/CD10+ (N=8), pre-B (N=2), early pro-B (N=1) and maturing B (N=1) ALL were studied. All patients exhibited >80% ALL blast BM infiltration. Sensitivity (LD50 < 150 μg/uL) in both NL and HG conditions was evident for 2 patients, while resistance to both conditions was seen in 3 patients. Patients resistant at NL conditions were resistant in HG, too. In contrast, 7 of 12 patients sensitive to PDN in NL conditions proved resistant in HG conditions (glucose 233–295 mg/dL). All 9 sensitive in NL conditions patients (in HG conditions 2 sensitive and 7 resistant) exhibited a median ×800 fold increase of the MTT LD50 measured (range 6–29.000 fold). For all patients (N=12) median MTT LD50 under NL conditions was 0.15 μg/uL and in HG conditions 167.5 μg/uL (× 116.6 fold increase). This change was statistically significant (Mc Nemar's test, p=0,016) and this change was not simply related to the change in glucose concentration. These findings of greatly increased ALL blast resistance in hyperglycemia environment, can support the notions given earlier (Weiser MA et al, Cancer 2004; 100:1179–85, Pieter's R EHA 2008) that hyperglycemia by itself can promote PDN resistance. Given that steroids is a mainstay in ALL treatment and that steroid induced hyperglycemia is a common occurrence, blood glucose monitoring and hyperglycemia correction appears to be of paramount significance in delivering an effective ALL treatment.

INTRODUCTION: TEL/AML1+ acute lymphoblastic leukemia (ALL) is considered to be a distinct nosological entity with excellent prognosis, but recent studies have indicated significant clinical heterogeneity. OBJECTIVE: In this study, we attempted to estimate the incidence and clinical features of TEL/AML1+ ALL for the first time in a representative cohort of Greek pediatric patients. METHODS: One hundred twenty children (<16 years old) diagnosed with ALL (107 of B-cell origin, 13 of T-cell origin) were screened for TEL/AML1 with interphase fluorescence in situ hybridization by using a commercial probe set. All patients were treated as either standard risk (SR) or high-risk (HR) cases according to a modified BFM-95 (Berlin-Frenkfurt-Munster) protocol. Follow-up ranged between 5 and 87 months (median: 45 months). RESULTS: Twenty-six patient (all of them will ALL of B-cell origin [24.3%]) were found to be positive for TEL/AML1. The presence of TEL/AML1 was significantly associated with younger age and lower white blood cell count at diagnosis but not with remission duration or overall survival rate. The number of children who relapsed (1 vs 7) or succumbed (1 vs 5) was comparable between the TEL/AML1+ and TEL/AML1− groups of children with ALL of B-cell origin. CONCLUSIONS: The incidence of TEL/AML1 in Greece seems comparable to that in other European and Mediterranean countries. As in most European studies, the independent prognostic value of TEL/AML1 is in doubt, because it is closely associated with other favorable factors. In this series, the modification of the therapeutic regimen (ie, omission of the SR arm) may be responsible for the similar outcome in TEL/AML1+ and TEL/AML1− cases, because it seems to lower the relapse risk for all children with ALL.

INTRODUCTION: Treatment results for pediatric non-Hodgkin's lymphoma (NHL) continue to improve internationally. OBJECTIVE: Our goal was to evaluate patient characteristics in our series of patients with NHL and outcomes for the last 16 years (1990–2006). METHODS: Our patients included 52 newly diagnosed children (11 girls) with a median age of 8.40 years (range: 0.33–14.5 years). Histology results included B-lymphocyte NHL, T-lymphocyte NHL, and Ki-1 in 35, 12, and 5 patients, respectively. In each 5-year period, 14 (3), 17 (3), and 21 (5) patients (girls) were diagnosed, respectively. Common presenting sites were the mediastinum (16), neck area (14), and abdomen (10). Disease was at stage I, II, III, and IV in 3, 14, 23, and 7 patients, respectively. Treatment varied over time. Berlin-Frankfurt-Munich (BFM) protocols had been applied since 1995 (BFM-NHL-90), and since 1997 the BFM-NHL-95 protocol had been applied. Irradiation was given to 5 patients (2 with B-NHL, 3 with T-NHL), and autologous stem cell transplantation was performed on 4 patients, all with B-NHL (1 with central nervous system disease, 1 with residual disease at the end of treatment, and 2 at relapse). RESULTS: At this writing, 41 patients are alive; 39, 2, and 1 are in first, second, and third remission, respectively. In total, 9 have succumbed (2 died soon after admission in other hospitals as a result of acute-phase complications), and 5 patients died during the first decade of our retrospective study (with T-histology and extensive disease). The event-free survival rate is 74.4% (39 of 52 patients), and the overall survival rate is 80.9% (41 of 52 patients), for a median follow-up time of 6.1 years (range: 0.01–14.7 years) for all patients. For the 39 patients treated with the BFM-95 protocol since 1997, event-free survival and overall survival rates are 79.4% and 88.2%, respectively, for a median follow-up time of 4.8 years. CONCLUSIONS: Overall and event-free survival rates and outcome of our patients with NHL treated during the last 16 years are standing high. There has been limited use of irradiation and stem cell transplantation.