Clinical comparison of weight- and age-based strategy of dose administration in children receiving intravenous busulfan for hematopoietic stem cell transplantation.

Bu, combined with TDM-guided dosing, is associated with fewer graft failures/relapses and lower toxicity in pediatric HSCT. We aimed this retrospective study for comparison of weight- and age-based dosing in terms of clinical outcomes such as time to engraftment, early complications, EFS, OS, and toxicity profiles in children receiving iv Bu. Sixty-one children who underwent HSCT from April 2010 to February 2013 by means of a Bu-based conditioning regimen and completed 100 days after transplantation at Ankara Children?s Hematology and Oncology Hospital Bone Marrow Transplantation Unit were enrolled in this study. SOS and neutropenic fever occurred more frequently in the weight-based dosing group. We found a statistically significant correlation between Bu dose and the incidence of SOS (r = 0.26, p = 0.04). Multivariate analysis showed only weight-based dosing of Bu was a significant predictor of SOS (HR = 9.46; p = 0.009). However, no relationship was found between two groups in terms of hemorrhagic cystitis, engraftment syndrome, acute or chronic GvHD, time to engraftment, chimerism, TRM, OS, and EFS rates. Weight-based dosing of Bu may cause higher incidence of SOS and early infectious complications at the places where TDM of Bu cannot be performed.

Protective effect of lycopene against radiation-induced hepatic toxicity in rats.

The radioprotective effect of lycopene against liver damage was investigated in 80 female Sprague Dawley rats (10 per group). Early-group rats included: controls (group 1), lycopene (group 2), radiotherapy alone (group 3), and lycopene + radiotherapy (group 4). Lycopene (5 mg/kg per day) was administered orally for 7 days; single-fraction 8 Gy abdominopelvic radiotherapy was administered on day 8. Early-group rats were sacrificed on day 10. Late-group rats (groups 5-8) underwent treatment with the same regimens but, in groups 6 and 8, lycopene was administered until all rats were sacrificed, 60 days postradiotherapy. Liver malondialdehyde levels increased significantly and glutathione (GSH) levels, GSH-peroxidase (GSH-Px) and superoxide dismutase (SOD) activity decreased significantly in radiotherapy versus control groups. In lycopene + radiotherapy groups, malondialdehyde levels decreased significantly and GSH levels, GSH-Px and SOD activity increased significantly compared with radiotherapy groups. No significant between-group histo pathological differences were observed in early groups; in late groups, histopathological changes increased significantly in the radiotherapy group versus control group. A significant decrease in histopathological changes occurred in the lycopene + radiotherapy group compared with the radiotherapy group. Lycopene supplementation significantly reduced radiotherapy-induced oxidative liver injury.

Multiple primary malignant neoplasms from the black sea region of Turkey.

Long-term cancer survival is increasing and, as a consequence, so is the prevalence of secondary malignancies. This study evaluated the patient and tumour characteristics of 117 patients with multiple primary malignant neoplasms (MPMN). The incidence of MPMN in children and adults was 0.28% and 1.23%, respectively. The male : female ratio was 1.7 : 1. The mean ± SE age at tumour diagnosis was 60.56 ± 1.18 years. Overall, the top three tumour sites were the larynx, bladder and breast. Among secondary tumours, lung cancer was the most frequent, followed by breast and colon cancer. Among males, the leading primary and secondary tumour sites were the larynx (30.1%) and lung (50.7%), respectively. Among females, the breasts were both the leading primary (32.6%) and secondary (37.2%) cancer site. The mean ± SE overall survival was 97.2 ± 15.0 months. During follow-up, the brain was the most commonly observed site of metastasis. The occurrence and characteristics of MPMN reported in the literature are also reviewed. The present study contributes towards increasing understanding and treatment of MPMN in a different population group.

Awareness of glucose-6 phosphate-dehydrogenase deficiency in celiac disease.

UNLABELLED: Individuals with celiac disease (CD) are predisposed to a number of haematological abnormalities including anaemia secondary to malabsorption of iron, vitamin B12 or folate; anaemia of chronic disease and coagulopathy secondary to vitamin K deficiency. Correction of coagulopathy with vitamin K is necessary before endoscopic biopsy in patients with suspected CD. However, vitamin K causes haemolysis in glucose-6 phosphate-dehydrogenase deficiency. CONCLUSION: When vitamin K administration becomes necessary for correction of coagulopathy in patients with CD; glucose-6 phosphate-dehydrogenase deficiency should be considered.

Gaucher disease with communicating hydrocephalus and cardiac involvement.

A 14-year-old female with Gaucher disease presented with hydrocephalus, corneal opacities, cirrhosis, and cardiac valvular involvement. A homozygous D409H mutation was identified. She underwent surgery for aortic and mitral valve replacement. Because of severe calcification of the aortic root, no successful valve replacement was performed. She died on the third day after the explorative cardiac surgery. Cardiac abnormalities represent a life-threatening presentation of the homozygous D409H mutation. Identification of this type is essential prior to initiating appropriate therapy with enzyme replacement and cardiac corrective surgery.

Prognostic value of the PAI-1 4G/5G polymorphism in invasive ductal carcinoma of the breast.

The study group was derived from the archive materials of 55 invasive ductal breast cancer (IDC) patients who had undergone breast-preserving surgery (partial mastectomy/ axillary dissection). All patients included in the study had clinically T(1)-2, N0-M0 invasive ductal carcinoma. Genomic DNA species were extracted from paraffin-embedded blocks, and plasminogen activator inhibitor type-1 (PAI-1) gene 4G/5G genotyping was done by polymerase chain reaction (PCR)-restriction fragment length polymorphism (RFLP). Patient demographics, axillary metastasis status, metastatic lymph nodi/total dissected lymph nodes from axilla, histopathologic characteristics of tumors, local recurrences, and survival ratio were assessed. PAI-1 4G/5G genotype frequencies were 4G/4G (64%), 4G/5G (31%), and 5G/5G (5%) in the patient group. According to the results based on frequencies, the demographics were not different. Five-year local recurrence rate of 4G/5G patients was the lowest (2/17, 12%) (P = 0.02). Also five-year distant metastases ratio of 4G/5G patients was the highest (18%) (P = 0.01). Five- and 10-year disease-free survival rates for the 4G/4G, 4G/5G, and 5G/5G groups were 97% and 94%, 82% and 77%, and 100% and 94%, respectively (P = 0.004). The results of this study indicate that the 4G allele in the PAI 1 gene had a negative impact on local recurrence and disease-free survival of patients with clinical T(1)-2N0M0 IDC.

A very unusual presentation of Niemann-Pick disease type B in an infant: similar findings to congenital lobar emphysema.

The main features of Niemann-Pick disease type B (NPD-B) are enlargement of the liver and spleen, and mild pulmonary involvement. Recurrent respiratory tract infection and progressive decline in pulmonary function are major contributors to morbidity and mortality in this patient group. Massive pulmonary involvement in early life is extremely rare. The most common finding on chest X-rays of NPD-B patients is reticular or nodular infiltration of the lungs. This article describes a very rare presentation of NPD-B in an infant who had suffered recurrent respiratory tract infections. Massive emphysema and marked infiltrative parenchymal changes (infiltration of the parenchyma) were initially attributed to congenital lobar emphysema and its compressive effects. However, NPD was suspected when a lung biopsy showed foamy cells and sea-blue histiocytes were detected in a bone marrow biopsy. The definitive diagnosis was established with an enzyme study for sphingomyelinase.

Successful use of recombinant factor VIIa (NovoSeven) during cardiac surgery in a pediatric patient with Glanzmann thrombasthenia.

Glanzmann thrombasthenia is a rare, hereditary, congenital disorder of platelet function characterized by inappropriate bleeding that is difficult to control. Recombinant activated factor VII (rFVIIa) is a new treatment that is used to stop bleeding and provide surgical support for these patients. This report describes the use of rFVIIa to prevent serious bleeding during and after open-heart surgery in a child with Glanzmann thrombasthenia.

The effect of pre-operative conventional and hyperfractionated radiotherapy schedules on wound healing and tensile strength in rats: an experimental study.

We examined the effects of pre-operative conventional and hyperfractionated radiotherapy schedules on wound healing and tensile strength in 90 female Wistar rats weighing between 182 and 240 g. The animals were randomized into three groups (n = 30 each). Group I was sham-irradiated. Group II (conventional) received 20 daily fractions of 200 cGy, to a total dose of 4000 cGy. Group III (hyperfractionated) received 40 fractions of 120 cGy, twice daily, to a total dose of 4800 cGy. Four weeks after radiotherapy, incision and primary repair with simple suturing was performed on one side of the neck. Twenty-one days after wounding, all the rats were sacrificed. Non-parametric Kruskal-Wallis and Mann-Whitney U-tests were used for the statistical analysis of wound tensile strength. The chi-squared test was used for the statistical analysis of the histopathologic findings. The hyperfractionated group had a significantly lower tensile strength than that of the control group (P = 0.03, z = -2.18). According to the histopathologic findings, fibrosis was increased significantly in the hyperfractionated group as compared to the other groups (P = 0.038, chi2 = 6.52). Hyperfractionated radiotherapy significantly reduced the wound tensile strength in the early evaluation period as compared to the control group.

Prognostic significance of seizure in patients with glioblastoma multiforme.

BACKGROUND: Several prognostic factors have been described but there are few studies evaluating the prognostic importance of seizure in patients with glioblastoma multiforme (GBM). AIMS: To evaluate the prognostic importance of seizure at the time of the diagnosis of glioblastoma multiforme (GBM) and compare it with other known prognostic factors. SETTINGS AND DESIGN: Between January 1994 and December 2000, 81 patients underwent irradiation for intracranial GBM at our institution. The criteria for inclusion in this study were biopsy-proven GBM, being treated for primary disease. Seventy-six patients were retrospectively evaluated and the remaining five patients could not be enrolled due to lack of details. MATERIAL AND METHODS: The prognostic importance of age, sex, performance status, a history of seizure at diagnosis, extent of surgery, radiotherapy field and dose were studied. STATISTICAL ANALYSIS: The Kaplan-Meier method, the Log rank test, the Cox proportional hazard model and the Mann-Whitney U test were used for statistical analysis. RESULTS: Survival at first and second years was 19.74% and 4.81%, respectively. Univariate analysis revealed age, performance status, history of seizure, and radiotherapy dose as significant prognostic factors and with multivariate analysis age, history of seizure and radiotherapy dose were positive prognostic factors. CONCLUSION: This study concluded that in GBM, history of seizure prior to diagnosis of GBM was a positive prognostic factor.

Bone marrow failure with concurrent enteroviral infection in a newborn.

A newborn baby, with transient pancytopenia concurrent to Echovirus type 11 infection, was hospitalized for fever, diarrhea, rash, generalized petechiae and hepatosplenomegaly. Subsequent investigation showed bone marrow failure. To our knowledge this is the first reported case of bone marrow failure with concomitant enteroviral infection.

Internal carotid artery occlusion in a patient with malignant peritoneal mesothelioma: is it a sign of malignancy-related thrombosis?

To our knowledge, the occlusion of arteries and platelet hyperaggregation have not been reported in patients with malignant mesothelioma. However, venous thromboembolism, especially in the pulmonary vasculature in association with thrombocytosis and hyperfibrinogenemia, are commonly noticed in this disorder. Furthermore, we detected enhanced platelet aggregation in a case of malignant peritoneal mesothelioma with internal carotid artery occlusion in whom there were postsplenectomy thrombocytosis and hyperfibrinogenemia. The possible mechanisms of ICA occlusion in this patient, including the role of MPM and postsplenectomy state, thrombocytosis, platelet functional changes, and other factors were investigated and discussed.

Effects of G-CSF and high-dose methylprednisolone on peripheral stem cells, serum IL-3 levels and hematological parameters in acute lymphoblastic leukemia patients with neutropenia: a pilot study.

Several agents, used either alone or in combination, have been shown to boost absolute numbers of PMLs and CD34+ stem cells in PB. In this study, we compared the effects of three different treatments, G-CSF, HDMP, and G-CSF + HDMP, for neutropenic patients (absolute PML count < 0.5 x 10(9)/l) who were on maintenance therapy for ALL with a control group who received no treatment. Hematological parameters, PB-CD34+33- and -CD34+ HLA-DR- stem cell numbers, and serum IL-3 levels were measured prior to, and a week after, the first day of treatment. WBC and absolute PML counts were significantly increased compared to pretreatment values in all treatment groups. However, peripheral CD34+ 33- and CD34+ HLA-DR stem cell numbers and serum IL-3 levels were increased significantly only in the G-CSF group. There was also a significant increase in serun IL-3 levels in the G-CSF + HDMP group. This study suggests that G-CSF may induce an increase in peripheral stem cell numbers, and that it supports hemopoietic recovery by increasing IL-3 in patients with chemotherapy-induced neutropenia.

Dealing with a hemophilia-A patient undergoing cerebral aneurysm surgery.

In this article anesthesiologic and hematologic aspects of a patient with Hemophilia-A, who underwent craniotomy for a right middle cerebral artery aneurysm, are discussed.

Effects of methylprednisolone on human myeloid leukemic cells in vitro.

We have demonstrated previously that high-dose methylprednisolone treatment induces differentiation and apoptosis of leukemic cells in patients with different morphological subtypes of acute myeloblastic leukemia (AML) in vivo. In the present study, we investigated the in vitro effects of high (10(-3) M) and low (10(-6) M) concentrations of methylprednisolone (MP) on freshly isolated bone marrow leukemic cells from nine newly diagnosed patients with AML by light and electron microscopy (EM) and agarose gel electrophoresis. A marked increase in MP-induced apoptosis of leukemic cells, with a maximum effect at 24 hr of exposure to both low and high concentrations of MP (10(-6) M and 10(-3) M), was demonstrated by light microscopy in cultures of four (three with AML-M1 and one with AML-M7) of the nine patients. In three cases, the increase in the number of apoptotic cells induced by high-concentration MP was approximately twice that observed when the lower concentration was used. A few apoptotic cells were detected in the cultures from the other five patients. However, a typical DNA ladder pattern of apoptosis was observed on gel electrophoresis of MP-treated leukemic cells from one patient (AML-M1) after 2 hr of incubation with both high- and low-MP concentrations. In two patients, a nonspecific DNA smear was observed only when high-concentration MP was used. The increase in differentiated leukemic cells induced by MP was also dose dependent, and was observed in cultures from all but one patient. Morphological features of apoptosis and differentiation were also confirmed by EM studies. The results of the present study, together with our previous clinical experience, suggest that MP, especially at high doses, could have a significant role in the treatment of some AML patients by inducing apoptosis and differentiation of leukemic cells.