Publications by authors named "Savas Kansoy"

28 Publications

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Metabolic syndrome and risk factors after hematopoietic stem cell transplantation in children and adolescents.

J Pediatr Endocrinol Metab 2021 Apr 25;34(4):485-493. Epub 2021 Feb 25.

Department of Pediatric Hematology & Oncology and Bone Marrow Transplantation, Medical School of Ege University, Izmir, Turkey.

Objectives: The early and late complications after hematopoietic stem cell transplantation (HSCT) determine the patients' prognosis and life quality. We aim to determine the metabolic syndrome development frequency after HSCT in children to find out the risk factors and compare them with healthy adolescents.

Methods: Thirty-six children who underwent HSCT at least two years ago were analyzed prospectively and cross-sectionally. Our study included 18 healthy children between the ages of 11 and 17 as a control group. All of the cases were assessed in terms of metabolic syndrome (MS) through the use of Modified WHO Criteria.

Results: The patients' median age was 10.6 (5.1-17) years, the median time of follow-up after HCST was 4.1 (2-13.5) years and 70% were male. Two cases were diagnosed with MS (5.6%). When considered in terms of the sub-components of MS, 2 cases (5.6%) were found to have obesity, 17 cases (47%) abnormal glucose tolerance, 11 cases (30.7%) dyslipidemia, and 3 cases (8.6%) hypertension. The MS rate was not different when compared with the 11-17 year-old healthy control group (0 vs. 11%, p=0.48). Myeloablative conditioning regimen (65 vs. 20%) and the increased age at which HSCT was performed were considered to be risk factors in terms of insulin resistance (p=0.025 and 0.002).

Conclusions: Age and conditioning regimens were found to be the risk factors for insulin resistance development. The long-term follow-up of the cases who had undergone HSCT in childhood in terms of MS and its sub-components is important in order to increase life quality.
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http://dx.doi.org/10.1515/jpem-2020-0584DOI Listing
April 2021

Haplo-identical or mismatched unrelated donor hematopoietic cell transplantation for Fanconi anemia: Results from the Severe Aplastic Anemia Working Party of the EBMT.

Am J Hematol 2021 May 4;96(5):571-579. Epub 2021 Mar 4.

French Reference Center for Aplastic Anemia and Paroxysmal Nocturnal Hemoglobinuria, Saint Louis Hospital and University Paris Diderot, Paris, France.

Allogeneic hematopoietic cell transplantation (HCT) is the only curative option for bone marrow failure or hematopoietic malignant diseases for Fanconi anemia (FA) patients. Although results have improved over the last decades, reaching more than 90% survival when a human leukocyte antigen (HLA)-identical donor is available, alternative HCT donors are still less reported. We compared HCT outcomes using HLA-mismatched unrelated donors (MMUD; n = 123) or haplo-identical donors (HDs), either using only in vivo T cell depletion (n = 33) or T cells depleted in vivo with some type of graft manipulation ex vivo (n = 59) performed for FA between 2000 and 2018. Overall survival (OS) by 24 months was 62% (53-71%) for MMUD, versus 80% (66-95%) for HDs with only in vivo T cell depletion and 60% (47-73%) for HDs with in vivo and ex vivo T cell depletion (p = .22). Event-free survival (EFS) was better for HD-transplanted FA patients with only in vivo T cell depletion 86% (73-99%) than for those transplanted from a MMUD 58% (48-68%) or those with graft manipulation 56% (42-69%) (p = .046). Grade II-IV acute graft-versus-host disease (GVHD) was 41% (MMUD) versus 40% (HDs with no graft manipulation) versus 17% (HDs with T cell depleted graft), (p = .005). No differences were found for the other transplant related outcomes. These data suggest that HDs might be considered as an alternative option for FA patients with better EFS using unmanipulated grafts.
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http://dx.doi.org/10.1002/ajh.26135DOI Listing
May 2021

Prognostic factors for survival in children who relapsed after allogeneic hematopoietic stem cell transplantation for acute leukemia.

Pediatr Transplant 2020 Dec 15:e13942. Epub 2020 Dec 15.

Pediatric BMT Unit, GOP Hospital, Yüzüncü Yıl University Faculty of Medicine, Istanbul, Turkey.

Background: Post-transplant relapse has a dismal prognosis in children with acute leukemia undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT). Data on risk factors, treatment options, and outcomes are limited.

Procedure: In this retrospective multicenter study in which a questionnaire was sent to all pediatric transplant centers reporting relapse after allo-HSCT for a cohort of 938 children with acute leukemia, we analyzed 255 children with relapse of acute leukemia after their first allo-HSCT.

Results: The median interval from transplantation to relapse was 180 days, and the median follow-up from relapse to the last follow-up was 1844 days. The 3-year overall survival (OS) rate was 12.0%. The main cause of death was disease progression or subsequent relapse (82.6%). The majority of children received salvage treatment with curative intent without a second HSCT (67.8%), 22.0% of children underwent a second allo-HSCT, and 10.2% received palliative therapy. Isolated extramedullary relapse (hazard ratio (HR): 0.607, P = .011) and relapse earlier than 365 days post-transplantation (HR: 2.101, P < .001 for 0-180 days; HR: 1.522, P = .041 for 181-365 days) were found in multivariate analysis to be significant prognostic factors for outcome. The type of salvage therapy in chemosensitive relapse was identified as a significant prognostic factor for OS.

Conclusion: A salvage approach with curative intent may be considered for patients with post-transplant relapse, even if they relapse in the first year post-transplantation. For sustainable remission, a second allo-HSCT may be recommended for patients who achieve complete remission after reinduction treatment.
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http://dx.doi.org/10.1111/petr.13942DOI Listing
December 2020

Role of a second transplantation for children with acute leukemia following posttransplantation relapse: a study by the Turkish Bone Marrow Transplantation Study Group.

Leuk Lymphoma 2020 06 8;61(6):1465-1474. Epub 2020 Feb 8.

Pediatric BMT Unit, Ege University Faculty of Medicine, Izmir, Turkey.

We examined outcomes of 51 pediatric patients with relapsed acute leukemia (AL) who underwent a second allogeneic hematopoietic stem cell transplantation (alloHSCT). After a median follow-up of 941 days (range, 69-2842 days), leukemia-free survival (LFS) and overall survival (OS) at 3 years were 26.6% and 25.6%, respectively. The nonrelapse mortality rate (NMR) and cumulative incidence of relapse (CIR) were 36.4% and 42.4%, respectively. The Cox regression analysis demonstrated that the risk factors at second transplantation for predicting limited LFS were active disease (hazard ratio (HR) = 5.1), reduced intensity conditioning (RIC) (HR = 5.0), matched unrelated donor (MUD) (HR = 3.4) and performance score <80 (HR = 3.2). Pediatric patients with AL who relapsed after their first alloHSCT may survive with a second alloHSCT. Disease status, conditioning intensity, donor type, and performance score at the second transplantation are the relevant risk factors. A score based on these factors may predict the results of the second transplantation.
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http://dx.doi.org/10.1080/10428194.2020.1716220DOI Listing
June 2020

Clinical Features and HSCT Outcome for SCID in Turkey.

J Clin Immunol 2019 04 28;39(3):316-323. Epub 2019 Mar 28.

Department of Pediatric Immunology, Hacettepe University Medical School, Ankara, Turkey.

Severe combined immunodeficiency (SCID) is the most serious PID, characterized by T cell lymphopenia and lack of antigen-specific T cell and B cell immune responses, inevitably leading to death within the first year of life if hematopoietic stem cell transplantation (HSCT) is not performed.

Purpose And Methods: Since SCID is a common type of PID with an estimated incidence of 1/10.000 in Turkey, a retrospective analysis of HSCT characteristics, survival, immune recovery, and the major clinical features of SCID prior to HSCT is the aim of this multi-transplant center-based analysis.

Results: A total of 234 SCID patients transplanted between the years 1994 and 2014 were included in the study. Median age at diagnosis was 5 months, at transplantation, 7 months, B- phenotype and RAGs were the most common defects among others. Immune phenotype did not seem to have an effect on survival rate (p > 0.05), Immunoglobulin (Ig) requirement following HSCT did not differ between B+ and B- phenotypes (p > 0.05). Overall survival rate was 65.7% over a period of 20 years. It increased from 54% (1994-2004) to 69% (p = 0.052) during the last 10 years (2005-2014). Ten-year survival after HSCT has improved over time although the difference was not significant. Infection at the time of transplantation (p = 0.006), mismatched related donor (MMRD) (haploidentical parents), and matched unrelated donor (MUD) donor transplants p < 0.001 were the most important factors, significantly affecting the outcome.

Conclusions: This is the first multicenter study with the largest data obtained from transplanted SCID patients in Turkey. Early diagnosis with newborn screening (NBS) together with emerging referrals, treatment by transplantation centers, and specialized teams are mandatory in countries with high parental consanguinity such as Turkey.
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http://dx.doi.org/10.1007/s10875-019-00610-xDOI Listing
April 2019

A Case of Allergic Broncopulmonary Aspergillosis Associated With Hematopoietic Stem Cell Transplantation Due to Chronic Granulomatous Disease.

J Pediatr Hematol Oncol 2019 Apr;41(3):e161-e163

Department of Pediatric Bone Marrow Transplantation, Ege University Faculty of Medicine, İzmir, Turkey.

Allergic bronchopulmonary aspergillosis is an immunologic pulmonary disorder caused by hypersensitivity to Aspergillus fumigatus. This disorder is most commonly seen in patients with poorly controlled asthma and cystic fibrosis. It is rarely reported in chronic granulomatous disease patients; however, there are no cases reported with hematopoietic stem cell transplantation in the English literature. Herein, we report a patient with chronic granulomatous disease who had hematopoietic stem cell transplantation and subsequently developed allergic bronchopulmonary aspergillosis.
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http://dx.doi.org/10.1097/MPH.0000000000001252DOI Listing
April 2019

Is there an increased risk of spinal relapse in standard-risk medulloblastoma/primitive neuroectodermal tumor patients who receive only a reduced dose of craniospinal radiotherapy?

Childs Nerv Syst 2018 09 4;34(9):1657-1662. Epub 2018 Jun 4.

Faculty of Medicine, Department of Radiation Oncology, Ege University, İzmir, Turkey.

Purpose: Medulloblastoma (MBL) is the most common pediatric brain malignancy. Postoperative radiotherapy to the entire craniospinal axis is the standard-of-care but has linked to long-term morbidity. In this study, we analyzed the implication of reduced dose craniospinal radiotherapy (RT) for survival and pattern of relapse in MBL patients.

Material And Methods: The clinical characteristics of 32 consecutively diagnosed medulloblastoma/primitive neuroectodermal tumor patients were analyzed. After surgical resection, a dose of 23.4 Gy of spinal RT with a posterior fossa boost of 30.6 Gy was prescribed to standard-risk patients, whereas high-risk patients received 36 Gy spinal RT with additional boosts to the posterior fossa up to 54 Gy. Then, both groups received the same chemotherapy protocol.

Results: Five-year OS for standard and high-risk patients was 94 and 50%, respectively. When analyzing prognostic factors, postoperative tumor size is the most important one which affects the OS. Ten patients relapsed during follow-up, and there was no isolated spinal relapse in either group.

Conclusion: The risk of isolated spinal relapse does not increase with reduced-dose craniospinal RT, since there is no isolated relapse in either the standard or high-risk groups of patients.
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http://dx.doi.org/10.1007/s00381-018-3842-6DOI Listing
September 2018

Risks and outcomes of invasive fungal infections in pediatric allogeneic hematopoietic stem cell transplant recipients receiving fluconazole prophylaxis: a multicenter cohort study by the Turkish Pediatric Bone Marrow Transplantation Study Group.

Med Mycol 2019 Feb;57(2):161-170

Department of Pediatric Hematology&Oncology and BMT Unit, Medical Park Antalya Hospital, Antalya.

Invasive fungal infections (IFIs) are a major cause of infection-related morbidity and mortality in patients undergoing allogeneic hematopoietic stem cell transplantation (HSCT). Data from pediatric settings are scarce. To determine the incidence, risk factors and outcomes of IFIs in a 180-day period post-transplantation, 408 pediatric patients who underwent allogeneic HSCT were retrospectively analyzed. The study included only proven and probable IFIs. The cumulative incidences of IFI were 2.7%, 5.0%, and 6.5% at 30, 100, and 180 days post-transplantation, respectively. According to the multivariate analysis, the factors associated with increased IFI risk in the 180-day period post-HSCT were previous HSCT history (hazard ratio [HR], 4.57; 95% confidence interval [CI] 1.42-14.71; P = .011), use of anti-thymocyte globulin (ATG) (HR, 2.94; 95% CI 1.27-6.80; P = .012), grade III-IV acute graft-versus-host-disease (GVHD) (HR, 2.91; 95% CI 1.24-6.80; P = .014) and late or no lymphocyte engraftment (HR, 2.71; 95% CI 1.30-5.62; P = .007). CMV reactivation was marginally associated with an increased risk of IFI development (HR, 1.91; 95% CI 0.97-3.74; P = .063). IFI-related mortality was 1.5%, and case fatality rate was 27.0%.The close monitoring of IFIs in pediatric patients with severe acute GVHD who receive ATG during conditioning is critical to reduce morbidity and mortality after allogeneic HSCT, particularly among those with prior HSCT and no or late lymphocyte engraftment.
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http://dx.doi.org/10.1093/mmy/myy015DOI Listing
February 2019

Hematopoietic Stem Cell Transplantation Using Preimplantation Genetic Diagnosis and Human Leukocyte Antigen Typing for Human Leukocyte Antigen-Matched Sibling Donor: A Turkish Multicenter Study.

Biol Blood Marrow Transplant 2017 May 10;23(5):790-794. Epub 2017 Feb 10.

Turkish Pediatric Bone Marrow Transplantation Study Group. Bahçeşehir University Medical Faculty, Medical Park Göztepe Hospital, Istanbul, Turkey.

Preimplantation genetic diagnosis involves the diagnosis of a genetic disorder in embryos obtained through in vitro fertilization, selection of healthy embryos, and transfer of the embryos to the mother's uterus. Preimplantation genetic diagnosis has been used not only to avoid the risk of having an affected child, but it also offers, using HLA matching, preselection of potential HLA-genoidentical healthy donor progeny for an affected sibling who requires bone marrow transplantation. Here, we share the hematopoietic stem cell transplantation results of 52 patients with different benign and malign hematological or metabolic diseases or immunodeficiencies whose donors were siblings born with this technique in Turkey since 2008. The median age of the patients' at the time of the transplantation was 8 years (range, 3 to 16 years) and the median age of the donors was 2 years (range, .5 to 6 years). The most common indication for HSCT was thalassemia major (42 of all patients, 80%). The stem cell source in all of the transplantations was bone marrow. In 37 of the transplantations, umbilical cord blood of the same donor was also used. In 50 of the 52 patients, full engraftment was achieved with a mean of 4.6 × 10 CD 34 cells per kg of recipient weight. Ninety-six percent of the patients have been cured through hematopoietic stem cell transplantation without any complication. Primary engraftment failure was seen in only 2 patients with thalassemia major. All of the donors and the patients are alive with good health status. Preimplantation genetic diagnosis with HLA matching offers a life-saving chance for patients who need transplantation but lack an HLA genoidentical donor.
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http://dx.doi.org/10.1016/j.bbmt.2017.02.002DOI Listing
May 2017

Early Diagnosis and Hematopoietic Stem Cell Transplantation for IL10R Deficiency Leading to Very Early-Onset Inflammatory Bowel Disease Are Essential in Familial Cases.

Case Reports Immunol 2016 6;2016:5459029. Epub 2016 Sep 6.

Faculty of Medicine, Department of Pediatric Immunology, Ege University, Izmir, Turkey.

Alterations of immune homeostasis in the gut may result in development of inflammatory bowel disease. A five-month-old girl was referred for recurrent respiratory and genitourinary tract infections, sepsis in neonatal period, chronic diarrhea, perianal abscess, rectovaginal fistula, and hyperemic skin lesions. She was born to second-degree consanguineous, healthy parents. Her elder siblings were lost at 4 months of age due to sepsis and 1 year of age due to inflammatory bowel disease, respectively. Absolute neutrophil and lymphocyte counts, immunoglobulin levels, and lymphocyte subsets were normal ruling out severe congenital neutropenia and classic severe combined immunodeficiencies. Quantitative determination of oxidative burst was normal, excluding chronic granulomatous disease. Colonoscopy revealed granulation, ulceration, and pseudopolyps, compatible with colitis. Very early-onset colitis and perianal disease leading to fistula formation suggested probability of inherited deficiencies of IL-10 or IL-10 receptor. A mutation at position c.G477A in exon of the gene, resulting in a stop codon at position p.W159X, was identified. The patient underwent myeloablative hematopoietic stem cell transplantation from full matched father at 11 months of age. Perianal lesions, chronic diarrhea, and recurrent infections resolved after transplantation. IL-10/IL-10R deficiencies must be considered in patients with early-onset enterocolitis.
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http://dx.doi.org/10.1155/2016/5459029DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5028855PMC
September 2016

Prognostic Factors and a New Prognostic Index Model for Children and Adolescents with Hodgkin's Lymphoma Who Underwent Autologous Hematopoietic Stem Cell Transplantation: A Multicenter Study of the Turkish Pediatric Bone Marrow Transplantation Study Group.

Turk J Haematol 2016 Dec 18;33(4):265-272. Epub 2016 Apr 18.

Gülhane Training and Research Hospital Clinic of Pediatric Oncology, Ankara, Turkey Phone: +90 312 304 43 94 E-mail:

Objective: The prognostic factors and a new childhood prognostic index after autologous hematopoietic stem cell transplantation (AHSCT) in patients with relapsed/refractory Hodgkin's lymphoma (HL) were evaluated.

Materials And Methods: The prognostic factors of 61 patients who underwent AHSCT between January 1990 and December 2014 were evaluated. In addition, the Age-Adjusted International Prognostic Index and the Childhood International Prognostic Index (CIPI) were evaluated for their impact on prognosis.

Results: The median age of the 61 patients was 14.8 years (minimum-maximum: 5-20 years) at the time of AHSCT. There were single relapses in 28 patients, ≥2 relapses in eight patients, and refractory disease in 25 patients. The chemosensitivity/chemorefractory ratio was 36/25. No pretransplant radiotherapy, no remission at the time of transplantation, posttransplant white blood cell count over 10x103/µL, posttransplant positron emission tomography positivity at day 100, and serum albumin of <2.5 g/dL at diagnosis were correlated with progression-free survival. No remission at the time of transplantation, bone marrow positivity at diagnosis, and relapse after AHSCT were significant parameters for overall survival.

Conclusion: The major factors affecting the progression-free and overall survival were clearly demonstrated. A CIPI that uses a lactate dehydrogenase level of 500 IU/L worked well for estimating the prognosis. We recommend AHSCT at first complete remission for relapsed cases, and it should also be taken into consideration for patients with high prognostic scores at diagnosis.
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http://dx.doi.org/10.4274/tjh.2015.0280DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5204179PMC
December 2016

Outcome of autologous hematopoietic stem cell transplantation in children and adolescents with relapsed or refractory Hodgkin's lymphoma.

Pediatr Transplant 2015 Nov 8;19(7):745-52. Epub 2015 Sep 8.

Pediatric BMT Units, Bahcesehir University Faculty of Medicine Medical Park Goztepe Hospital, Istanbul, Turkey.

This study evaluates the outcome of 66 pediatric patients with rrHL who underwent autoHSCT. Twenty-nine patients experienced early relapse, and 19 patients experienced late relapse. Of 18 newly diagnosed with HL, 13 were primary refractory disease and five had late responsive disease. At the time of transplantation, only 68% of the patients were chemosensitive. The majority of patients received BCNU + etoposide + ara-C + melphalan for conditioning (45/66), and peripheral blood (56/66) was used as a source of stem cells. After a median follow-up period of 39 months, 46 patients were alive. At five yr, the probabilities of OS, EFS, the relapse rate, and the non-relapse mortality rate were 63.1%, 54.3%, 36.4%, and 9.1%, respectively. The probability of EFS in chemosensitive and chemoresistant patients at five yr was 72.3% and 19%, respectively (p < 0.001). Multivariate analysis showed that chemoresistant disease at the time of transplantation was the only factor predicting limited both OS (hazard ratio = 4.073) and EFS (hazard ratio = 4.599). AutoHSCT plays an important role for the treatment of rrHL in children and adolescents, and survival rates are better for patients with chemosensitive disease at the time of transplantation.
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http://dx.doi.org/10.1111/petr.12573DOI Listing
November 2015

Successful haematopoietic stem cell transplantation in 44 children from healthy siblings conceived after preimplantation HLA matching.

Reprod Biomed Online 2014 Sep 12;29(3):340-51. Epub 2014 Jun 12.

ART and Reproductive Genetics Center, Istanbul Memorial Hospital, Istanbul, Turkey.

Haematopoietic stem cell transplantation (HSCT) remains the best therapeutic option for many acquired and inherited paediatric haematological disorders. Unfortunately, the probability of finding an HLA matched donor is limited. An alternative technique is PGD combined with HLA matching, which offers the possibility of selecting unaffected embryos that are HLA compatible with the sick child, with the aim of possible use of stem cells from the resulting baby in future. Since the first successful report for Fanconi anaemia a decade ago, the therapeutic success of this technique was reported in a few cases and for a limited number of disorders. Here, we report full recovery of 44 sick children who received HSCT from healthy infants conceived after pre-implantation HLA matching for the following 10 indications; beta-thalassaemia, Wiskott-Aldrich syndrome, Fanconi anaemia, sickle cell anaemia, acute myeloid leukaemia, acute lymphoblastic leukaemia, Glanzmann's thrombasthaenia, Diamond-Blackfan anaemia, X-linked adrenoleukodystrophy and mucopolysaccharidosis type I. No serious complications were observed among recipients and donors. Graft failure occurred in four children with beta-thalassaemia where a second HSCT was planned. Preimplantation HLA matching is a reliable technique and provides a realistic option for couples seeking treatment for an affected child when no HLA-matched donor is available.
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http://dx.doi.org/10.1016/j.rbmo.2014.05.010DOI Listing
September 2014

HLA-matched family hematopoetic stem cell transplantation in children with beta thalassemia major: the experience of the Turkish Pediatric Bone Marrow Transplantation Group.

Pediatr Transplant 2012 Dec 29;16(8):846-51. Epub 2012 Aug 29.

Akdeniz University School of Medicine, Antalya, Turkey.

From January 1991 to June 2009, 245 children with beta thalassemia major who underwent their first allogeneic HSCT in Turkey and who were followed for a minimum of one yr post-transplantation were enrolled this study. The median age of the patients was 6.6 yr old (range, 1-22 yr). The distribution of Pesaro risk class I, II, and III categories was 41, 130, and 63 children, respectively. The median serum ferritin level was 2203 ng/mL. Eighty-eight patients received bone marrow (BM) stem cells; 137, peripheral blood (PB) stem cells; and 20, cord blood (CB) stem cells. The donors were HLA-matched siblings or parents. Median engraftment times were shorter in PBSCT patients compared with the BMT group (p < 0.001). Grade II-IV acute GvHD was observed in 33 children (13.5%), while cGvHD was observed in 28 patients (12.5%), eight of whom had the extensive form. Thalassemic reconstitution was observed in 43 (17%) of the transplant patients. Post-transplant aplasia occurred in three patients, and the TRM rate was 7.75%. Seventeen patients were lost after 100 days. The thalassemia-free survival and OS rates were 68% (95% CI, 61.8-74.2) and 85.0% (95% CI, 80.2-89.8), respectively. We believe that this study is important because it is the first multicenter national data for children with beta thalassemia major receiving HSCT.
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http://dx.doi.org/10.1111/j.1399-3046.2012.01778.xDOI Listing
December 2012

Enteral nutrition is feasible in pediatric stem cell transplantation patients.

Pediatr Blood Cancer 2012 Dec 21;59(7):1327-9. Epub 2012 Aug 21.

Division of Nutrition, Pediatric Bone Marrow Transplantation Unit, Ege University School of Medicine, IZMIR, Turkey.

We aimed to demonstrate whether enteral nutrition (EN) is feasible in daily practice of hematopoietic stem cell transplantation (HSCT).Nutritional records of 100 patients were evaluated. Patients with poor oral intake were fed by EN with tube. A total of 79 patients required nutritional support. Of them, 71 were fed by EN only. Five were fed by EN plus parenteral nutrition (PN),three were fed by PN only. Median duration of EN was 21 days. In the EN only group, 68% gained or maintained their weight. EN should be considered as a feasible option for nutrition support in children undergoing HSCT.
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http://dx.doi.org/10.1002/pbc.24275DOI Listing
December 2012

Sustained seroconversion of chronic hepatitis B infection after stem cell transplantation.

Pediatr Transplant 2011 Aug 20;15(5):E92-5. Epub 2010 Jan 20.

Ege University, Pediatric Oncology and Bone Marrow Transplantation Unit Ege University, Pediatric Gastroenterology, Hepatology and Nutrition, Izmir, Turkey.

  We present an 18-yr-old adolescent with acute lymphocytic leukemia, who underwent peripheral blood SCT with serologically and histologically documented chronic hepatitis B infection. Prior and during the transplant process, lamivudine was administered orally and he underwent SCT with a twofold decrease in viral load at the time of transplant from his HLA full matched, HBV natural immune (anti-HBs and anti-HBc positive) donor. Successful engraftment was achieved and three months after SCT, HBV seroconversion was documented accompanied with an ALT flare. Chronic graft-versus-host disease coincided after the transplantation, and he has been on immunosuppressive treatment for 25 months with sustained HBV seroconversion. We assume that adoptive immunity transfer combined with antiviral treatment might also constitute sustained seroconversion in chronic HBV, besides the reported risk of reactivation.
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http://dx.doi.org/10.1111/j.1399-3046.2009.01272.xDOI Listing
August 2011

Cytoprotective effects of amifostine in the treatment of childhood malignancies.

Pediatr Blood Cancer 2009 Jul;52(7):829-33

Department of Pediatric Oncology, Ege University Faculty of Medicine, Izmir, Turkey.

Background: Multi-systemic acute side effects occur, in response to intensive therapies that have been applied in childhood malignancies in recent years. Amifostine has rarely been used in the childhood cancers as a multisystemic protective agent for minimizing these side effects.

Procedure: In this study, the effectiveness of amifostine in combination with chemotherapy for childhood cancer treatment has been researched. Of 11 subjects (2.5 months-17 years) 4 subjects had leukemia, 4 had solid tumor, and 3 had lymphoma. For these 11 subjects, 29 chemotherapy courses were given in combination with amifostine, and 20 without amifostine. Their hematological, gastrointestinal and hepatic toxicity were evaluated according to the WHO toxicity criteria. Amifostine was given intravenously in a dose of 740 mg/m(2), one to three consecutive days depending on the chemotherapy regimen.

Results: The hemoglobin, leukocyte, and platelet levels of the two groups were not statistically different. However, when comparing the courses of the patients receiving the same medications at the same doses, in the group with amifostine, mean erythrocyte transfusion requirement was significantly reduced (P = 0.025). In 31% of the courses with amifostine and 50% of the courses without amifostine, febrile neutropenia developed. Gastrointestinal system and hepatic toxicity was significantly reduced in the courses with amifostine with respect to those without it (P = 0.001). Vomiting, hypotension and nausea were the only side effects related to amifostine.

Conclusion: Use of amifostine during the treatment of childhood cancers with intensive chemotherapy and/or radiotherapy significantly reduced the erythrocyte transfusion requirements of the patients as well as gastrointestinal and hepatic toxicity.
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http://dx.doi.org/10.1002/pbc.21959DOI Listing
July 2009

Plasma natriuretic peptides levels and echocardiographic findings in late subclinical anthracycline toxicity.

Pediatr Hematol Oncol 2008 Dec;25(8):723-33

Pediatric Oncology, Ege University School of Medicine Department of Pediatrics, Izmir, Turkey.

The purpose of this study was to evaluate late cardiac toxicity by comprehensive echocardiographic study, and to determine whether plasma atrial natriuretic peptide and brain natriuretic peptide levels might be indicators of neurohumoral activation. The study included 49 long-term survivors and 21 controls. A wide variety of echocardiographic parameters were measured or calculated. Plasma peptide levels were determined. Patients had significant changes in different echocardiographic parameters that are suggestive of LV systolic and diastolic dysfunction. Plasma peptide levels were not increased. The authors have found significant subclinic cardiotoxicity by echocardiography. Survivors seem to have normal plasma natriuretic peptide levels in long-term period.
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http://dx.doi.org/10.1080/08880010802435393DOI Listing
December 2008

Successful bone marrow transplantation in an 8-month-old patient with chronic granulomatous disease.

Turk J Pediatr 2006 Jul-Sep;48(3):253-5

Department of Pediatric Oncology and Pediatric BMT Center, Ege University Faculty of Medicine, Izmir, Turkey.

An eight-month-old boy with chronic granulomatous disease (CGD) received HLA identical sibling bone marrow transplantation (BMT) following busulphan and cyclophosphamide conditioning. No graft-versus-host disease was demonstrated. Five years after transplantation, mixed chimerism was 60% in peripheral blood, and 85% of his neutrophils had normal oxidative burst activity. He is now six years old, in very good health and growing well. In this period, he experienced no severe infectious diseases. To our knowledge, this is the first case of CGD who had BMT in Turkey. His successful outcome illustrates that BMT in a patient with CGD in the first years of life should be considered early if an HLA-matched donor is already available, before development of any recurrent life-threatening infections or irreversible organ damage.
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January 2007

Meropenem plus amikacin versus piperacillin-tazobactam plus netilmicin as empiric therapy for high-risk febrile neutropenia in children.

Pediatr Hematol Oncol 2004 Mar;21(2):115-23

Department of Pediatric Oncology, Ege University Medical School, Izmir, Turkey.

The aim of this study was to evaluate the efficacy and safety of meropenem plus amikacin compared with piperacillin-tazobactam plus netilmicin for initial empirical antibiotic treatment of high-risk febrile neutropenia in children with cancer. Patients with hematologic malignancy (leukemia or stage III/IV non-Hodgkin lymphoma) who presented with fever and neutropenia (ANC < 500/mm3) and patients with solid tumors who presented with fever and severe neutropenia (ANC < 100/mm3) were considered to be at high risk and eligible for this study. In this prospective study, 33 patients with 50 febrile neutropenic episodes received i.v. neropenem (20 mg/kg every 8 h) plus amikacin (15 mg/kg/d in 2 divided doses) (in 31 episodes) or piperacillin/tazobactam (100 mg/4 mg/kg every 8 h) plus netilmicin (7 mg/kg every 24 h) (in 19 episodes). Clinical response was determined at 72 h and at completion of the therapy. The groups were comparable in terms of age, sex, initial ANC, use of growth factors, and classification of the infections. An infection was documented microbiologically in 12 episodes (39%) in the meropenem plus amikacin group and in 8 episodes (42%) in the piperacillin/tazobactam plus netilmicin group. Of the 22 microbiological isolates, 37% were gram-positives, 45% were gram-negatives, and 18% were fungi. Most of the clinically documented infections were of lower respiratory tract, gastrointestinal mucosa, or urinary tract origin. The mean duration of neutropenia was 9 days in both groups. Fever persisted for 1-30 days (mean 3 vs. 5 days). The success rate with initial empiric therapy was 52% in the meropenem plus amikacin and 42% in the piperacillin/tazobactam plus netilmicin group, respectively (p = .5). Total success rate (with or without modification) was 97% vs. 90% in the episodes. Three patients died due to infection (1 vs. 2 patients). No major adverse effects were observed in each group. Empirical therapy with meropenem plus amikacin or piperacillin/tazobactam plus netilmicin for high-risk febrile neutropenia is equally effective and safe in pediatric cancer patients.
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http://dx.doi.org/10.1080/08880010490277321DOI Listing
March 2004

Radiotherapy-induced secondary cranial neoplasms in children.

Childs Nerv Syst 2004 Jan 29;20(1):46-9. Epub 2003 Jul 29.

Department of Pediatrics, Division of Pediatric Oncology, Ege University School of Medicine, 35100, Izmir, Turkey.

Background: Secondary malignant neoplasms (SMN) in CNS tumor survivors has become problem of increasing concern over the last 20 years. These tumors usually occur in a different site from the primary brain tumor several years after treatment.

Case Report: We report secondary cranial malignant neoplasms in three patients who were treated with irradiation and chemotherapy for their primary brain tumors. The first case is a male survivor of an orbital rhabdomyosarcoma who developed a meningioma 8 years later. The other two cases are female survivors of ependymomas who were irradiated at the age of 3 and developed secondary gliomas 8 and 17 years after therapy respectively.

Conclusion: Patients carry a risk of developing SMNs many years later since irradiation is still an important part of the treatment. An SMN may have a benign course, as in meningioma, or be a dilemma for the patient, as in glioblastoma.
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http://dx.doi.org/10.1007/s00381-003-0798-xDOI Listing
January 2004

Immune deficiencies following cancer treatment in children.

J Trop Pediatr 2003 10;49(5):286-90

Department of Pediatric Oncology, Ege University School of Medicine, Izmir, Turkey.

The aim of this study was to determine serum immunoglobulins, IgG subclasses, lymphocyte subsets, and serum protective antitoxin levels of tetanus and diphtheria, and to investigate specific antibody response to tetanus and diphtheria vaccines in children with cancer who have been treated for leukemias and solid tumors. Forty patients with different types of childhood malignancies were enrolled in this study and their lymphocyte subsets, serum Ig A, M, G and IgG subclass concentrations were determined at completion of chemotherapy and 6 months later. We measured serum diphtheria (D) and tetanus (T) antitoxin levels and investigated specific antibody responses against DT vaccines at 6 months. Only the leukemic children had low CD19+ cells at completion of chemotherapy and 6 months later. The patients with solid tumors had reduced CD4+ cells, but increased natural killer cells at completion of chemotherapy. Serum IgA and IgM levels were decreased in leukemic patients after chemotherapy. There were no IgG subclass deficiency. Forty-two per cent of the patients did not have protective serum T antitoxins. All patients produced high levels of DT antibodies by vaccination. Immune system changes recover by 6 months after cancer therapy in children. Children with solid tumors, as well as leukemias, should be followed-up in terms of immune deficiencies. A repeat dose of tetanus toxoid should be recommended at 6 months.
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http://dx.doi.org/10.1093/tropej/49.5.286DOI Listing
October 2003

[Human herpesvirus 6 infections in kidney and bone marrow/stem cell transplant recipients].

Mikrobiyol Bul 2003 Apr-Jun;37(2-3):179-86

Ege Universitesi Tip Fakültesi, Mikrobiyoloji ve Klinik Mikrobiyoloji Anabilim Dali, Izmir.

In this study, active human herpesvirus (HHV)-6 infection were investigated in 39 renal and 9 bone marrow/stem cell transplant recipients. For this purpose, the presence of HHV-6 DNA in patients sera have been searched by nested polymerase chain reaction (nPCR). In addition, HHV-6 IgM and IgG antibodies were performed by micro-enzyme immunoassay (EIA) to detect seronegative patients before transplantation and IgM response in active or primary HHV-6 infection. Active infection with HHV-6 DNA positivity was detected in 5.3% of renal and 22.2% of bone marrow/stem cell transplant recipients. Active HHV-6 infection was found to be related with asymptomatic reactivation, graft disfunction and cytomegalovirus disease in renal transplant recipients, and, fever and graft versus host disease in bone marrow/stem cell transplant recipients. It has been concluded that, the investigation of HHV-6 DNA by nPCR in the transplant sera, was a practical and useful method for the laboratories, in order to diagnose active HHV-6 infection, while HHV-6 IgG antibody detection was also useful for the differential diagnosis of primary infection or reactivation/reinfection, but HHV-6 IgM antibodies has low value to detect active HHV-6 infection.
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June 2004

Experience of the Izmir Pediatric Oncology Group on Neuroblastoma: IPOG-NBL-92 Protocol.

Pediatr Hematol Oncol 2003 Apr-May;20(3):211-8

Department of Pediatric Oncology, Dokuz Eylül University, Institute of Oncology, Izmir, Turkey.

This multicentric study aimed to bring neuroblastoma patients together under IPOG-NBL-92 protocol and evaluate the results within the period between 1992 and 2001 in Izmir. Sixty-seven neuroblastoma patients from 4 pediatric oncology centers in Izmir were included in the study. IPOG-NBL-92 protocol modified from German Pediatric Oncology (GPO)-NB-90 protocol was applied: Patients in stage 1 received only surgery, while surgery plus 4 chemotherapy courses (cisplatin, vincristine, ifosfamide) were given in stage 2 and surgery plus 6 chemotherapy courses (cisplatin, vincristine, ifosfamide, epirubicin, cyclophosphamide) were given in stages 3 and 4 patients. In patients who were kept in complete remission (CR), a maintenance therapy of one year was applied. Radiotherapy was given to the primary site following induction chemotherapy plus surgery in stages 3 and 4 patients with partial remission (PR). The stages of the patients were as follows: 5% in stage 1, 39% in stage 3, 49% in stage 4, and 7% in stage 4S. Primary tumor site was abdomen in 88% of cases. CR rates were as 100% in stage 1, 76% in stage 3, 35% in stage 4, and 75% in stage 4S. Relapse was observed in 32% of patients in a median of 19 months. The median follow-up time for survivors was 33 (17-102) months. Five-year OS rate was 31% and the EFS rate was 30% in all patients. Five-year overall and event-free survival rates were 63 and 30% in stage 3, but 6 and 5%, respectively, in stage 4 patients. Univariate analysis established that the age, stage, primary tumor site, and high LDH and NSE levels conferred a significant difference. The IPOG-NBL-92 protocol has proved to be satisfactory with tolerable toxicity and reasonable CR and survival rates. However, more effective treatments suitable to Turkey's social and economic conditions are urgently needed for children over 1 year of age with advanced neuroblastoma.
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November 2003

Treatment results of osteosarcoma of the extremity in children and adolescents at Ege University Hospital.

Pediatr Hematol Oncol 2002 Oct-Nov;19(7):475-82

Department of Pediatrics, Division of Pediatric Oncology, Ege University School of Medicine, Izmir, Turkey.

Twenty-five patients were treated for osteosarcoma of the extremity at Ege University Hospital. Eight of them were metastatic. All patients received cisplatin, doxorubicin, ifosfamide, and methotrexate preoperatively. Twenty-three patients underwent surgery at around week 15 (11-18 weeks). All but one underwent limb-sparing surgery. While good responders continued to receive the same drugs, poor responders were given the same regimen before 1996, but high-dose ifosfamide alone after 1996. For all patients the projected event-free survival (EFS) rates were 63.5% at 2 years and 53% at 5 years. The projected overall survival (OS) rates were 72% at 2 years and 62% at 5 years. For nonmetastatic patients, 5-year EFS and OS rates were 67% as compared with metastatic patients (25 and 50%)(p =. 01 for EFS; p > .05 for OS). The results show that nonmetastatic patients with osteosarcoma of the extremity have favorable prognosis on this therapy regimen, allowing a high rate of limb-sparing surgery.
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http://dx.doi.org/10.1080/08880010290097297DOI Listing
January 2003

Rare tumors of the lung in children.

Pediatr Hematol Oncol 2002 Sep;19(6):421-8

Department of Pediatrics, Ege University, Izmir, Turkey.

The authors report rare and different types of lung tumors in 4 children. The first case is an 8-year-old boy with mucoepidermoid carcinoma, the second case is a 9-year-old girl with neuroendocrine carcinoma, the third is a 14-year-old girl with fetal lung adenocarcinoma (FLAC), and the last is a 16-year-old girl with bronchioloalveolar carcinoma. Among these tumors, FLAC has not been reported in children so far. Each tumor type displayed a different prognosis in the follow-up period. In the differential diagnosis of primary lung tumors, carcinoid tumor, bronchogenic carcinoma, and pulmonary blastoma are frequently encountered, but these rare tumor types should be borne in mind.
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http://dx.doi.org/10.1080/08880010290097189DOI Listing
September 2002