Blastic Plasmacytoid Dendritic Cell Neoplasm: Single Center Experience on a Rare Hematological Malignancy

PurposeBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and poor prognostic hematological malignancy. There is still no standard treatment established for BPDCN patients. We aim to summarize the main clinical, biological features and treatment of 9 BPDCN patients.MethodsNine patients with BPDCN who had been diagnosed between July 2008 and December 2018 in Ankara University School of Medicine, were retrospectively evaluated.ResultsAll patients (n = 9) were male, median age was 64 (21–80). Five patients (55.6%) had bone marrow infiltration, 5 patients (55.6%) cutaneous lesions, 6 patients (66.7%) lymph node involvement, 2 patients (22.2%) central nervous system involvement and 2 patients (22.2%) spleen involvement at time of diagnosis. Complex karyotype was observed in 2 patients. CHOP was given to 5 patients (55.6%), hyper-CVAD to 2 patients (22.2%), fludarabine, cyclophosphamide and mitoxantrone to 1 patient (11.1%) and cyclophosphamide, etoposide, methylprednisolone to 1 patient (11.1%) as first line chemotherapy. Four patients (44.4%) underwent allogeneic hematopoietic stem cell transplantation (AHSCT) in complete remission (CR) 1. Venetoclax was given to a transplant ineligible patient who had skin and lymph node involvement, with the off-label use. The median follow-up time was 15.9 months (3–48.6 months). Estimated median overall survival was 15.9 + 1.6 (95% CI 12.7–19.1) months.ConclusionIntensive induction therapies followed by AHSCT in CR seems to be best approaches for patients with BPDCN. Thus, more effective treatment strategies particularly targeted therapies should be warranted to improve the survival of patients with this rare disease.     

p53 point mutations in primary human gastric carcinomas

Summary p53 point mutations in primary gastric carcinomas were analyzed by performing cDNA deoxynucleotide sequencing of the gene. Out of 16,9 (56.3%) primary gastric carcinoma cases, including early cancer, showed one or more p53 point mutations in their open-reading frame, and 4 out of 9 cases had a p53 point mutation within highly conserved domains. The characteristics of the p53 mutation spectrum observed in primary tumors were (a) frequent mutation at an A:T pair (50%, 7 out of 14 mutations), (b) high transversion incidence (29%, 4 out of 14 mutations), (c) no transition at CpG, and (d) no G:C to T:A transversion. Our results suggest that p53 mutation is a common event in gastric carcinoma occurring from the early stage of progression with its specific mutation spectrum.Abbreviation PCR-SSCP polymerase chain reaction single-strand conformation polymorphism     

Early stage epithelial ovarian cancers: A study of morphologic prognostic factors

We intended to reevaluate the morphologic prognostic factors for early-stage ovarian carcinomas. We reviewed 111 patients diagnosed with early-stage ovarian cancer who had undergone primary surgery at Hacettepe Hospital between 1984 and 2001, using diagnostic criteria from the WHO-2003 classification. We applied the Universal grading system suggested by Shimizu/Silverberg and noted FIGO-stage, histotype, tumor size, bilaterality, and endometriosis. These features were compared with each other and survival. The survival analysis was carried out by Kaplan–Meier curves. Of the cases, 52 were reclassified as ‘borderline tumor’ or ‘cystadenoma with borderline foci’ and 59 as ‘invasive carcinoma’. FIGO-stage and mitotic count were significant for survivals of 59 patients with cancer. Mitotic index was also significant for the probability of metastasis. The patients with stage-II cancer had 5.65 times more risk of recurrence than stage-I cancer. The 5-year overall and disease-free survivals rates were 90.6% and 87.5% for stage-I, 54.7% and 39.3% for stage-II, respectively. Universal grade did not reach statistical significance for survivals but it was related to FIGO-stage significantly. In conclusion, FIGO-stage is the most reliable prognosticator. Although prognostic value of universal grade is not significant, mitotic count may provide important prognostic information for early-stage ovarian carcinomas.     

Microbiological and SEM assessment of atraumatic restorative treatment in adult dentition

Objectives

To investigate the practicability of atraumatic restorative treatment (ART) in adults in terms of marginal adaptation of restorations and microbiological changes in residual carious dentin.

Materials and methods

The occlusal dentin caries of 25 permanent molar teeth were removed with hand instruments. The total counts of bacteria (TCB) and the facultative anaerobic bacteria (FAB), mutans streptococci (MS), and Lactobacillus spp. (LB) counts in the affected dentin were evaluated quantitatively. The weights of the samples were measured with an electronic balance (Shimadzu, Type AX200, Japan). The cavities were restored with glass ionomer cement (Ketac^TM Molar Easymix, ESPE Dental AG, Seefeld, Germany). Twenty replicas of randomly selected ART restorations were prepared and marginal adaptation was evaluated by scanning electron microscopy (SEM). After 6 months, the same protocols were repeated. Data were analyzed with paired sample t-tests, Wilcoxon t-tests, Pearson and Spearman correlations, and chi-square tests (p<0.05).

Results

In the sixth month, restoration loss and pulpitis were not observed. The mean weight of samples removed from the cavity floor was less than the baseline (0.014±0.009 and 0.023±0.013 g, respectively) (p<0.01), and the counts of total bacteria, FAB, MS, and LB significantly decreased compared to baseline (p<0.01). The frequency of marginal gaps was increased (p< 0.01).

Conclusions

ART showed that the counts of microorganisms decreased after 6 months although the marginal gap rates of restorations increased.

Clinical relevance

ART can be a reliable treatment approach in adults for 6 months due to the decrease in microorganism counts, although gaps exist.

     

The Role of Serum Procalcitonin Levels in Predicting Ascitic Fluid Infection in Hospitalized Cirrhotic and Non-cirrhotic Patients

Objective: To determine the role of serum procalcitonin levels in predicting ascites infection in hospitalized cirrhotic and non-cirrhotic patients.Methods: A total of 101 patients (mean age: 63.4±1.3, 66.3% were males) hospitalized due to cirrhosis (n=88) or malignancy related (n=13) ascites were included in this study. Spontaneous bacterial peritonitis (SBP, 19.8%), culture-negative SBP (38.6%), bacterascites (4.9%), sterile ascites (23.8%) and malign ascites (12.9%) groups were compared in terms of procalcitonin levels in predicting ascites infection. Receiver operating characteristic (ROC) curves were used to evaluate the diagnostic performance of procalcitonin levels and predicting outcome of procalcitonin levels was compared with C-reactive protein (CRP).Results: Culture positivity was determined in 26.7% of overall population. Serum procalcitonin levels were determined to be significantly higher in patients with positive bacterial culture in ascitic fluid compared to patients without culture positivity (median (min-max): 4.1 (0.2-36.4) vs. 0.4 (0.04-15.8), p<0.001). Using ROC analysis, a serum procalcitonin level of <0.61 ng/mL in SBP (area under curve (AUC): 0.981, CI 95%: 0.000-1.000, p<0.001), <0.225 ng/mL in culture-negative SBP (AUC: 0.743, CI 95%: 0.619-0.867, p<0.001), <0.42 ng/mL in SBP and culture-negative SBP patients (AUC: 0.824, CI 95%: 0.732-0.916, p<0.001), and <1.12 ng/mL in bacterascites (AUC: 0.837, CI 95%: 0.000-1.000, p=0.019) were determined to accurately rule out the diagnosis of bacterial peritonitis. Predictive power of serum procalcitonin levels in SBP + culture-negative SBP group (AUCs: 0.824 vs 0.622, p=0.004, Fig 4), culture-positive SBP (AUCs: 0.981 vs 0.777, p=0.006, Fig 5) and (although less powerfull) in culture-negative SBP (AUCs: 0.743 vs 0.543, p=0.02, Fig 6) were found significantly higher than CRP.Conclusion: According to our findings determination of serum procalcitonin levels seems to provide satisfactory diagnostic accuracy in differentiating bacterial infections in hospitalized patients with liver cirrhosis related ascites.     

The Impact of Method on Kidney Graft and Patient Survival in Kidney-Pancreas Transplantations for Type I Diabetes Mellitus

Patients who develop end-stage renal disease (ESRD) associated with Type I Diabetes Mellitus may receive kidney alone (KA) transplantation, simultaneous pancreas-kidney (SPK) transplantation, or a pancreas after kidney (PAK) transplantation. The goal of this study is to examine the long-term impact of pancreas transplantation on kidney graft and patient survival rates. A total of 85 transplantation cases, consisting of 30 that received living donor KA, 21 that received SPK, and 34 that received PAK, from 2003–2010 at Akdeniz University Organ Transplantation Institute were retrospectively screened. There was a graft loss in 4 cases from the KA group, and in 1 case from each of the SPK and PAK groups. The five-year kidney graft survival rates were 86.7% in KA, 95.2% in SPK, and 97.1% in PAK. There was a single patient loss in both KA and SPK. The kidney survival percentages were higher in SPK and PAK groups compared to the KA group. Therefore, SPK should be the primary preference in these patients; however, for the cases that have a living donor, pancreas transplantation should be considered after kidney transplantation, or the patients can be followed-up on with close blood sugar control.Key words: Kidney, Pancreas, Transplantation, Kidney survival, Patient survivalThe discovery of insulin in 1921 enabled the transition from diabetic ketoacidosis and diabetic coma to an increasing number of patients with prolonged life expectancies in the clinical course of diabetes mellitus (DM). However, with prolonged lifetime, increases in the neurological, ocular, and renal complications of DM have become evident. With a 40% rate, DM is the leading cause of end-stage renal disease (ESRD) in the United States.¹ In patients with type I DM-related kidney failure, kidney transplant is highly more preferable in terms of the negative effects of long-term dialysis on the patient survival and quality of life compared with the benefits of kidney transplants.² In patients who develop type I DM-related kidney failure, kidney-alone transplantation (KA) from a living donor or a cadaver, simultaneous pancreas-kidney transplantation (SPK), or pancreas-after-kidney transplantation (PAK) are among the transplantation alternatives. The 10-year life expectancy in patients receiving hemodialysis for ESRD, and in those undergoing living donor or a cadaveric renal transplantation, was reported to be 4.4, 32.9, and 59.3% in the United States, respectively.³ Similarly, while the average life expectancy for diabetes patients waiting for kidney transplantation was 8 years, the average life expectancy after kidney transplantation was determined to be 22 years.² When pancreas transplantation is added to kidney transplantation, prolonged kidney and patient survival rates can be attained along with other benefits, such as protection from the secondary effects of diabetes and an increase in patients'' quality of life. While the 4-year mortality rate in the selected dialysis patients on the waitlist for pancreas-kidney was 40%, it was 10% in patients who received SPK transplantation.⁴ The goal of this study is to compare the impact of the KA, SPK, and PAK transplantation methods on kidney graft and patient survival rates in patients with ESRD associated with type I diabetes.     

Melatonin ameliorates chronic renal failure-induced oxidative organ damage in rats

Chronic renal failure (CRF) is associated with oxidative stress that promotes production of reactive oxygen species (ROS). Melatonin, the chief secretory product of the pineal gland, was recently found to be a potent free radical scavenger and antioxidant. The aim of this study was to examine the role of melatonin in protecting the aorta, heart, corpus cavernosum, lung, diaphragm, and kidney tissues against oxidative damage in a rat model of CRF, which was induced by five of six nephrectomy. Male Wistar albino rats were randomly assigned to either the CRF group or the sham-operated control group, which had received saline or melatonin (10 mg/kg, i.p.) for 4 wk. CRF was evaluated by serum blood urea nitrogen (BUN) level and creatinine measurements. Aorta and corporeal tissues were used for contractility studies, or stored along with heart, lung, diaphragm, and kidney tissues for the measurement of malondialdehyde (MDA, an index of lipid peroxidation), protein carbonylation (PC, an index for protein oxidation), and glutathione (GSH) levels (a key antioxidant). Plasma MDA, PC, and GSH levels and erythrocytic superoxide dismutase (SOD), catalase (CAT), and glutathione peroxidase (GSH-Px) activities were studied to evaluate the changes of antioxidant status in CRF. In the CRF group, the contraction and the relaxation of aorta and corpus cavernosum samples decreased significantly compared with controls (P < 0.05-0.001). Melatonin treatment of the CRF group restored these responses. In the CRF group, there were significant increases in tissue MDA and PC levels in all tissues with marked reductions in GSH levels compared with controls (P < 0.05-0.001). In the plasma, while MDA and PC levels increased, GSH, SOD, CAT, and GSH-Px activities were reduced. Melatonin treatment reversed these effects as well. In this study, the increase in MDA and PC levels and the concomitant decrease in GSH levels of tissues and plasma and also SOD, CAT, GSH-Px activities of plasma demonstrate the role of oxidative mechanisms in CRF-induced tissue damage, and melatonin, via its free radical scavenging and antioxidant properties, ameliorates oxidative organ injury. CRF-induced dysfunction of the aorta and corpus cavernosum of rats was reversed by melatonin treatment. Thus, supplementing CRF patients with adjuvant therapy of melatonin may have some benefit.