Prophylactic corticosteroid use prevents engraftment syndrome in patients after autologous stem cell transplantation

Engraftment syndrome (ES) following autologous stem cell transplantation (ASCT) at the time of neutrophil recovery may comprise fever, rash, pulmonary edema, or diarrhea. Usually, ES is easily manageable using corticosteroids but may prolong hospitalization. In two consecutive cohorts of subsequent patients with myeloma, lymphomas, and testicular/germ cell cancer, we assessed the benefit of corticosteroid use to prevent incidence and severity of ES following ASCT. Whereas Cohort A (82 patients) received no prophylactic corticosteroids, corticosteroids (4 mg dexamethasone oral daily) were started in Cohort B (60 patients) at day +9 until day +13 following ASCT. Steroid prophylaxis significantly reduced the incidence of ES (6/60; 10% vs. 33/82; 40%; p < 0.001). Hospitalization duration was longer in patients with ES than in patients without ES within both cohorts (in Cohort A: p = 0.007; and B: p = 0.011), but did not differ significantly between cohorts A and B. Finally, in Cohort A, there was a trend to an inferior 2‐year overall survival rate in patients without ES compared to patients with ES (p = 0.067), but definite conclusions are not yet allowed. Our results suggest that corticosteroid prophylaxis from days +9 to +13 following ASCT significantly reduces the risk of ES and shortens hospitalization duration.     

Congenital thrombotic thrombocytopenic purpura caused by new compound heterozygous mutations of the ADAMTS13 gene

Upshaw–Schulman syndrome (USS) is due to severe congenital deficiency of von Willebrand factor (VWF)‐cleaving protease ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 domains, nr 13) activity resulting in the presence of unusually large forms of VWF in the circulation, causing intravascular platelet clumping and thrombotic microangiopathy. Our patient, a 26‐year‐old man, had attacks of thrombotic thrombocytopenic purpura (TTP) with thrombocytopenia and a urine dipstick positive for hemoglobin (4+), often as the only sign of hemolytic activity. He had ADAMTS13 activity of <1% of normal plasma without the presence of inhibitors of ADAMTS13. ADAMTS13 deficiency was caused by two new mutations of the ADAMTS13 gene: a deletion of a single nucleotide in exon17 (c. 2042 delA) leading to a frameshift (K681C fs X16), and a missense mutation in exon 25 (c.3368G>A) leading to p.R1123H. This case report confirms the importance of the analysis of the ADAMTS13 activity and its inhibitor in patients who have episodes of TTP, with a very low platelet count and sometimes without the classic biochemical signs of hemolysis.     

Adjustment process in Iranian women with breast cancer

Breast cancer is a devastating event for a woman. Physical changes and psychological problems, treatment to improve the patient's condition, and increased survival rates compared with other cancers manifest the importance of quality of life in these patients. This quality of life is affected by how the patients adjust to their situation. Hence, to understand adjustment to breast cancer, this research aimed to investigate the experience from the patients' perspective and how they interact with others and interpret their experiences in adjusting to the disease. A qualitative research approach based on grounded theory was used. The data were the result of 45 interviews with patients in different phases of their illness trajectory during 1 year, 6 interviews with families, and 10 observation sessions. The main categories that emerged were perceived threat to live, religious aspects, supportive dimensions, will to recover, increase in endurance, barriers to efforts leading to health, living with the disease with tolerance, and inhibitors and facilitators of tolerance. These main categories were understood as passages to reach evolutionary peaceful coexistence. Adjustment to breast cancer has positive evolutional process, and its direction is toward better adjustment. By positive mental reconstruction, the patients feel that they can live with their disease.     

Simplified MSI marker panel for diagnosis of colorectal cancer

Background: Colorectal cancers (CRCs) tumors are diagnosed by microsatellite instability (MSI) due to accumulation of insertion/deletion mutations in tandem repeats of short DNA motifs (1–6 bp) called microsatellites. Microsatellite instability (MSI) is not only a hallmark marker for screening of hereditary nonpolyposis colorectal cancer (HNPCC), but also a prognostic and predictive marker for sporadic colorectal cancer. Our objective was to determine and study of five mononucleotide microsatellite markers status among Iranian patients with HNPCC and sporadic colorectal cancer. Material and Methods: In the current investigation 80 sporadic CRC and 80 HNPCC patients were evaluated for MSI. The pentaplex panel including 5 quasimonomorphic mononucleotide repeats (NR-21, BAT-26, BAT-25, NR-27 and NR-24) was used. Results: Our findings showed that the NR-21 was the most frequent instable marker among the other markers. 53% and 25.6% specimens had instability in sporadic CRC and HNPCC, respectively. Furthermore, the frequencies of instability BAT-25 was determined in 20% sporadic CRC and 23% HNPCC samples. Interestingly our results demonstrated that the frequency of instability NR-24 was similar 20% sporadic CRC and 20.5% HNPCC. Moreover, percentage of NR-27 in HNPCC was 19.2 and 0% in sporadic CRC. Finally, BAT-26 was instable in 21.8% HNPCC patients while we could find 6.6% instability for BAT-26 in sporadic cases. Conclusion: It seems that among 5 mononucleotides markers NR-21 was the most useful marker for diagnosis HNPCC and sporadic cancer. Following NR-21, BAT-25 and NR-24 are the most reliable markers. Therefore using a triplex panel including 3 aforementioned MSI markers should be more promising markers for identifying MSI status in both patients with HNPCC and/or sporadic colorectal cancer.     

BeEAM conditioning with bendamustine-replacing BCNU before autologous transplantation is safe and effective in lymphoma patients

BEAM with BCNU is commonly used for conditioning treatment followed by autologous stem cell transplantation (ASCT). However, pulmonary toxicity and availability issues associated with BCNU prompted us to evaluate bendamustine-replacing BCNU (BeEAM). We analyzed 39 lymphoma patients receiving BeEAM conditioning with 200 mg/m² bendamustine at days ?7 and ?6. The median duration until neutrophil recovery was 11 days, and 15 days for platelet recovery (>20 g/L). The most common grade 3/4 non-hematologic toxicities comprised mucosal side effects (27 pts.). Pulmonary toxicity was observed in one patient (2.5%), and one patient died of septic complications. The CR rate increased from 33% to 74% 100 days after ASCT. After a median follow-up of 18.5 months, progression and death each occurred in 11 patients (28%). Median progression-free and overall survival at 2 years were 69% and 72%. Our data suggest that BeEAM conditioning using bendamustine is safe and results in promising survival rates.     

Reconstructive materials for endodontically treated teeth

A review of the literature on restoration of endodontically treated teeth was presented. The advantages and disadvantages of current methods and materials were described, and a detailed technique was suggested for the newer glass-ionomer restorative materials. An all-purpose material suited for the restoration of endodontically treated teeth is still unavailable. Each patient requires individual consideration. However, Ketac Silver material may be an excellent choice in select patients.