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Purpose: This study aimed to investigate the status of DNA polymerase delta (POLD1 and POLD2) gene expression at the time of diagnosis in pediatric acute lymphoblastic leukemia (ALL) patients, compared with the normal population, and its relationship with prognosis and other clinical findings. Materials and Methods: Seventy-three patients diagnosed with ALL between January 2008 and November 2015 and 29 healthy control subjects were included in the study. Gene expression profiling of peripheral blood samples was performed using Real-time PCR. Results: The mean value of POLD1 gene expression was found to be significantly higher in ALL patients at the time of diagnosis than the control group (376.5± 685.8 and 17.9± 19.8, respectively), but there was no difference in POLD2 gene expression (511.5± 898.1 and 125.4± 132.7, respectively). POLD1 and POLD2 gene expressions were found to be low in patients with relapse and exitus, but the results were not statistically significant. Patients with low levels of POLD1 expression had lower survival rates in the 5th year than those with high levels of expression (54% and 68%, respectively), and similarly, patients with low levels of POLD2 expression had lower survival rates in the 5th year compared to those with high levels of expression (58% and 68%, respectively). Conclusion: Lower POLD1 and POLD2 expressions at the time of diagnosis in ALL patients may adversely affects the prognosis. [ABSTRACT FROM AUTHOR]

Acute lymphoblastic leukemia (ALL) is the most common type of cancer in children. The identification of prognostic factors has become essential in design and analysis of modern therapeutic trials. The aim of this study is to present the expression patterns of lamin A/C, lamin B1 and B2 and their prognostic value in childhood acute lymphoblastic leukemias, which are thought to be related with cell proliferation and apoptosis. The study was carried out between January 2008 and March 2014 at the Cukurova University Faculty of Medicine, Division of Pediatric Oncology and sixty-four patients diagnosed with ALL and 35 control cases were included in the study. Real Time-Polymerase Chain Reaction (RT-PCR) method was used to detect lamin A/C, lamin B1 and lamin B2 protein expression at the time of the diagnosis and at the end of the induction treatment. At the time of the diagnosis, lamin B1 protein gene expression was lower in ALL patients when compared to the control group (p= 0.001). When lamin gene expression levels at the time of diagnosis and after induction therapy were compared, the lamin A/C and B2 gene expressions were significantly lower after the administration of induction chemotherapy (both values p= 0.0001). The decrease in lamin B1 gene expression was not statistically significant (p= 0.403). When the lamin expression levels in the control group were compared with the levels after induction chemotheraphy, lamin expressions were found to be significantly lower (all p values 0.0001). In childhood ALL, lamin B1 protein gene expression could be used as a diagnostic marker, while decrease in the lamin A/C and B2 protein gene expressions after induction could be used as a marker for response to therapy. Our study is the first to show in lamin protein gene expression relationship with diagnosis and prognosis in ALL in literature. Comprehensive multicenter studies are needed with a larger cohort of patients to reach more reliable results. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study is to evaluate the prognostic value and expression pattern of lamin A/C, lamin B1 and B2 in childhood acute myeloid leukemias, which are thought to be related with cell proliferation and apoptosis. Materials and Methods: Twenty-five patients diagnosed with acute myeloid leukemia (AML) and 35 control cases were included in the study. Real Time-Polymerase Chain Reaction (RT-PCR) method was used to detect lamin A/C, lamin B1 and lamin B2 protein gene expression at the time of the diagnosis and at the end of the induction treatment. Results: At the time of the diagnosis, lamin B1 protein gene expression was lower in AML patients when compared to the control group. When lamin protein expression levels at the time of diagnosis and after induction therapy were compared, the lamin A/C and B2 protein gene expressions were lower after the administration of induction chemotherapy. AML patients with hepatomegaly when compared to patients without hepatomegaly, had only increased lamin A/C protein gene expression. Patients with splenomegaly had both increased lamin A/C and lamin B2 protein gene expressions when compared to the patients without splenomegaly. Conclusion: In childhood acute myeloid leukemias, lamin B1 protein gene expression could be used as a diagnostic marker, while decrease in the lamin A/C and B2 protein gene expressions after induction could be used as a marker for response to therapy. [ABSTRACT FROM AUTHOR]

In this study, we aimed to prospectively analyze the emergency diagnosis and clinical characteristics of the oncologic emergency patients admitted, as well as to evaluate the approach considerations to the cases, their treatment, patient outcomes and the prognostic factors. A total of 88 oncologic emergency patients (90 oncologic emergency hospitalizations), except for febrile neutropenia, were admitted to the pediatric oncology department between July 2014 and December 2015. A total of 30 cases of tumor lysis syndrome, 19 hyperleukocytosis, 11 convulsion, 7 spinal cord compression, 7 hemorrhagic cystitis, 9 pleural effusion, 6 vena cava superior syndrome, 4 typhlitis, 3 anuria, 4 gastrointestinal bleeding, 3 hypercalcemia, 4 acute respiratory distress syndrome, 2 acute massive hepatomegaly and 1 increased intracranial pressure were identified. Sixty-five (72.2%) of the study patients had a primary oncologic disease, whereas 25 (27.8%) had a recurring condition. The most common presenting complaint was fever (23.3%) followed by breathing difficulties (22.2%). Fifty-nine patients (65.6%) recovered and were discharged home, while 31 cases (34.4%) died. Tumor lysis syndrome was the most frequently encountered emergency condition in the pediatric oncology patient group. Oncologic emergencies need to be approached quickly and systematically in the light of current scientific knowledge. [ABSTRACT FROM AUTHOR]

Brain natriuretic peptide (BNP) is considered as a prognostic marker in patients with sepsis, but no data are available on BNP in pediatric cancer patients with febrile neutropenia (FN). Twenty-five pediatric cancer patients with FN were included in this study. Serum BNP level was measured. The mean BNP level was 330.8 ±± 765.3 pg/mL (5.9--3806 pg/mL). BNP levels of 12 patients were found over the normal level. High BNP levels were related to some conditions of the patients, and these were statistically significant ( P < .05). These conditions were required erythrocyte suspension, had pneumonia, time stayed in hospital, and neutropenia time. When regression test was done, required erythrocyte suspension for anemia and had pneumonia were found to be statistically significant. In conclusion, this is one of the first studies on BNP levels in pediatric cancer patients with FN. However, further studies with large sample sizes are needed to confirm the results and provide new data about this issue. [ABSTRACT FROM AUTHOR]

Aim: Disease free survival rate for acute myeloid leukemia (AML) is still below 50% for the last 30 years. Our objective was to compare the results of two different dosages of idarubicin (12 mg/m2 versus 8 mg/m2) therapy for newly diagnosed AML patients. Method: Sixty eight patients with AML were treated between February, 1998 and January, 2005. We designed two therapy groups comprising of 12 mg/m2/day idarubicin therapy (group I), 8 mg/m2/day idarubicin therapy and when oral nutrition is broken, we have given parenteral nutrition (group II). Overall survival (OS), event free survival (EFS), disease free survival (DFS) data were assessed and other tests were performed when needed. Result: There were 26 patients (38.2%) in group I and 42 patients (61.8%) in group II. After the first induction therapy, 20 patients (76.9%) in group I and 36 in group II (85.7%) had CR or partial remission. After two courses of induction, treatment-related mortality (TRM) was 34.6% in group I and 7.1% in group II (p: 0.006). OS of the patients in group I/II were 44/81% for 12 months, 34/54% for 24 months, 29/48% for 36 months. EFS were 43/65% for 12 months, 34/50% for 24 months, and 29/50% for 36 months. OS and DFS rates were statistically significant but EFS rates were not, in group I and II. Conclusion: The protocol with idarubicin dose of 8 mg/m2/day has less TRM in comparison to that of 12 mg/m2/day and has better OS and EFS. [ABSTRACT FROM AUTHOR]

Aim: To determine the efficacy and safety of cefoperazone-sulbactam combined with amikacin in the treatment of febrile neutropenia in children with cancer. Materials and Methods: The study included 20 cancer cases with 26 febrile neutropenia (FEN) episodes. Patient selection criteria were defined according to the guidelines issued by the Infectious Disease Society of America (IDSA). Results: Patients diagnosed with acute leukemia (58%) and solid tumors (42%) were recorded. Twelve (46.2%) of the primary disease cases were refractory. The number of infection episodes identified microbiologically and clinically was 10 (38.5%) and 12 (46.1%), respectively. Fever of unknown origin was observed in only 4 cases (15.4%). The success rate of the empirical treatment without additional modification was 42.3% (11 FEN episodes). Four episodes (15.4%) needed a replacement for sulbactam/cefoperazone because of persistent fever, adverse reactions, and/or clinical deterioration. Three patients died because of relapse or because they were refractory. As 15 (57.7%) of the patients deteriorated clinically and had fever, glycopeptide antibiotics were given after 48-72 h. The overall response rate at the end of the therapy was 80.8%, with/without modification. Conclusions: The combination of sulbactam/cefoperazone plus amikacin was effective and safe in the treatment of febrile episodes in neutropenic pediatric cancer patients. [ABSTRACT FROM AUTHOR]

Childhood histiocytosis constitutes a diverse group of disorders that have presented great difficulties for pediatricians in diagnosis and treatment. In the bone marrow, there is proliferation of histiocytes and cells of the mononuclear phagocytic systems. Class II Histiocytosis; the predominant cells are the normal cells of the monocytic-macrophage systems. There is no atypic cellularity. Commonly lymphohistiocytic infiltration is observed. Familial hemophagocytic lymphohistiocytosis, also known as erytrophagocytic lymphohistiocytosis (FEL) is a rare inherited disorder, whose genetic basis is unknown, characterised by multiorgan infiltration by lymphocytes and histiocytes. We would like to present three cases of FEL in which, two of them are siblings of the same family and the third case is the cousin of these two siblings. [ABSTRACT FROM AUTHOR]

Castleman's disease is a rare lymphoproliferative disorder of unknown aetiology. It has two clinical expressions. The localized form, which usually presents as a slow growing mass and the multicentric form, where tumoural involvement is multilocated. Mediastinal involvement is the most common location of presentation. Parapharyngeal involvement is very rare and, to our knowledge, five pediatric parapharyngeal Castleman's disease have been reported. We describe the case of a 14-year-old female child who was diagnosed as having parapharyngeal Castleman's disease. [ABSTRACT FROM AUTHOR]

To describe the clinical course of febrile neutropenic pediatric oncology patients undergoing granulocyte transfusions (GTF), we performed a retrospective chart review of all pediatric oncology patients with febrile neutropenia receiving granulocyte transfusions between September 2009 and September 2011 in a University Hospital setting. Thirty-seven patients received a total of 51 courses of GTFs. In 35 febrile episodes patients had leukemia and in 16 episodes patients had solid tumors. Three patients received allogeneic hematopoietic stem cell transplantation from their HLA identical sibling. Mean duration of fever and neutropenia was 18.5±17.5 days. Infections were classified as fever of unknown origin (21%, n= 11), microbiologically documented infection (44%, n= 23), clinically suspected infection (35%, n= 18). The mean granulocyte yield, WBC count and granulocyte count of the products were 3.1±1.2x1010/unit, 158.6±66.4x103/mm3 and 134.5±62x 103/mm3, respectively. The median donor WBC count at leukapheresis was 32.91x109/L. GTFs were well tolerated except one patient who had fever during transfusion. Thirty-nine (76.5%) of the episodes resolved from infection and discharged from the hospital. Of the 12 patients (23.5%) who died, seven of them were refractory to treatment, three patients had relapsed disease and two patients had newly diagnosed disease. This case series documents the course of 37 septic neutropenic pediatric oncology patients who underwent a total of 51 GTF courses. GTFs were generally well tolerated and improve short-term outcome in neutropenic pediatric oncology patients. [ABSTRACT FROM AUTHOR]