Sickle cell disease (SCD), one of the most common genetic disorders worldwide, is characterized by hemolytic anemia and tissue damage from the rigid red blood cells. Although hydroxyurea and transfusion therapy are administered to treat the accompanying tissue injury, whether either one prolongs the lifespan of patients with SCD is unknown. SCD-related mortality data are available, but there are few studies on mortality-related factors based on evaluations of surviving patients. In addition, ethnic variability in patient registries has complicated detailed analyses. The aim of this study was to investigate mortality and mortality-related factors among an ethnically homogeneous population of patients with SCD. The 735 patients (102 children and 633 adults) included in this retrospective cohort study were of Eti-Turk origin and selected from 1367 patients seen at 5 regional hospitals. A central population management system was used to control for records of patient mortality. Data reliability was checked by a data supervision group. Mortality-related factors and predictors were identified in univariate and multivariate analyses using a Cox regression model with stepwise forward selection. The study group included patients with homozygous hemoglobin S (Hgb S) disease (67 %), Hb S-β(0) thalassemia (17 %), Hgb S-β(+) thalassemia (15 %), and Hb S-α thalassemia (1 %). They were followed for a median of 66 ± 44 (3-148) months. Overall mortality at 5 years was 6.1 %. Of the 45 patients who died, 44 (6 %) were adults and 1 (0.1 %) was a child. The mean age at death was 34.1 ± 10 (18-54) years for males, 40.1 ± 15 (17-64) years for females, and 36.6 ± 13 (17-64) years overall. Hydroxyurea was found to have a notable positive effect on mortality (p = 0.009). Mortality was also significantly related to hypertension and renal damage in a univariate analysis (p = 0.015 and p = 0.000, respectively). Acute chest syndrome, splenic sequestration, and prolonged painful-crisis-related multiorgan failure were the most common causes of mortality. In a multivariate analysis of laboratory values, only an elevated white blood cell count was related to mortality (p = 0.009). These data show that despite recent progress in the treatment of SCD, disease-related factors continue to result in mortality in young adult patients. Our results highlight the importance of evaluating curative treatment options for patients who have an appropriate stem cell donor in addition to improving patient care and patient education.

Objective:Obtaining informed consent from hematopoietic stem cell recipients and donors is a critical step in the transplantation process. Anxiety may affect their understanding of the provided information. However, use of audiovisual methods may facilitate understanding. In this prospective randomized study, we investigated the effectiveness of using an audiovisual method of providing information to patients and donors in combination with the standard model.Materials and Methods:A 10-min informational animation was prepared for this purpose. In total, 82 participants were randomly assigned to two groups: group 1 received the additional audiovisual information and group 2 received standard information. A 20-item questionnaire was administered to participants at the end of the informational session.Results:A reliability test and factor analysis showed that the questionnaire was reliable and valid. For all participants, the mean overall satisfaction score was 184.8±19.8 (maximum possible score of 200). However, for satisfaction with information about written informed consent, group 1 scored significantly higher than group 2 (p=0.039). Satisfaction level was not affected by age, education level, or differences between the physicians conducting the informative session.Conclusion:This study shows that using audiovisual tools may contribute to a better understanding of the informed consent procedure and potential risks of stem cell transplantation.

BackgroundNeutropenic patients are susceptible to any anorectal disease, and symptomatic anorectal disease afflicts 2–32% of oncology patients. Perianal infections are the most feared complication, considering the lack of natural defense against infectious microorganisms. When septic complications develop, the anorectal disease is potentially fatal, especially in neutropenic patients in whom mortality rates range between 11–57%. Although anorectal diseases are a frequent complication with potentially fatal outcomes among patients with hematologic diseases, sufficient data are not available in the literature. In this study, we aimed to investigate the anorectal complications developing during the neutropenic period in patients with hematologic diseases.MethodsA total of 79 patients whose neutropenic period (absolute neutrophil count <500/mcL) continued for 7 days, or longer were included in the study.ResultsA total of 34 patients out of 79 (43%) were detected to develop anorectal complications, of them 6 (7.6%) developed an anorectal infection. The patients were characterized according to the hematological disease and its status (active or not), the type of treatment and the presence of a history of an anorectal pathology before the onset of the hematologic disease. Nineteen (24.1%) patients had the history of anorectal disturbances before diagnosis of the hematologic disease, and recurrence of an anorectal pathology was found in 14 out of 19 patients(73.7%). In addition, the overall mortality rate was higher among the patients who developed anorectal complications compared to another group (41.2% vs. 22.2%, p=0.059).ConclusionAnorectal pathology is a common complication with high recurrence rate in neutropenic patients. Perianal infections are important as they can cause life-threatening outcomes although they are relatively rare among all anorectal complications. Therefore perianal signs and symptoms should be meticulously evaluated concerning early diagnosis and treatment.

To look over the distribution of the mutations in a large series from Adana province, Southern Turkey, and determine the genotype-phenotype correlation of the frequent mutations. Among the 2500 individuals with mild or moderate anemia, microcytosis, and normal iron levels that were referred to our Genetic Diagnosis Center, a population consisting of 539 individuals were included in the study and tested for alpha-thalassemia mutations by using reverse dot blot hybridization technique. Twelve different mutations were detected in 539 patients. Among the 12 different mutations found, the most frequent mutations were the -a 3.7 (63.3 %), -MED (11.7 %), -20.5 (10.7 %), a2 IVS1(-5nt) (3.9 %), and a2 polyA-2 (3.5 %). The most frequent genotypes were -a 3.7 /aa (35.8 %), -a 3.7 /-a 3.7 (18.9 %), -20.5 /aa (11.5 %), and -MED /aa (10.4 %), respectively. There were statistically significant differences in hematological findings between -a 3.7 /-a 3.7 and -MED /aa, even though both have two mutated genes in the genotype. Our results show that alpha-thalassemia mutations are highly heterogeneous as well as deletional and -a 3.7 single gene deletion is particularly prevalent at Adana province in agreement to other studies from Turkey.

Adult patients with sickle cell disease (SCD) are highly susceptible to stem cell transplant complications, including drug toxicity, graft versus host disease (GVHD), and graft rejection due to SCD-related tissue damage, endothelial activation, and inflammation. The scarcity of compatible stem cells for transplantation further limits treatment options, with only 43 cases of adult allogeneic peripheral blood stem cell transplantation (allo-PSCT) from human leukocyte antigen (HLA)-identical sibling donors reported in the international registry for the period 1986-2013. Herein we report remarkable outcomes in a cohort of adult SCD patients who underwent allo-PSCT using a fludarabine (Flu), busulfan (Bu), and anti-T-cell lymphocyte globulin (ATG)-based conditioning regimen in combination with very low dose total body irradiation (TBI), followed by post-transplant cyclophosphamide (Cy) and sirolimus as GVHD prophylaxis. We performed a single-center, retrospective study consisting of 20 consecutive patients (mean age 33.4 years) who underwent allo-PSCT from HLA-matched related donors with a conditioning regimen of Flu 150/Bu 3.2/Cy 29/ATG 30 (Fresenius)/TBI 200 between September 2013 and September 2017. Data were validated by an independent data audit group of the affiliated JACIE-accredited transplantation center. All patients experienced a sustained donor cell engraftment. Full donor chimerism (total cell) occurred within 180 days in all patients. Mean duration of follow-up was 13.8 months (range: 0.3-50 months), with 12 (60%) patients completing 12 months. No non-relapse mortality or graft rejection occurred. Successful treatment was achieved without the presence of graft loss, grade III-IV acute GVHD, extensive chronic GVHD, or other major complications. Allo-PSCT in combination with Flu 150/Bu 3.2/Cy 29/ATG 30(Fresenius)/TBI 200- Cy/Sirolimus therapy yielded encouraging outcomes with no mortality and low incidence of GVHD. Further controlled studies will be necessary to compare transplant protocols and long-term outcomes.

The Eastern Mediterranean is among the regions where sickle cell disease (SCD) is common. The morbidity and mortality of this disease can be postponed to adulthood through therapies implemented in childhood. The present study focuses on the organ damage-reducing effects of the Baskent Sickle Cell Medical Care Development Program (BASCARE), which was developed by a team who lives in this region and has approximately 25 years of experience. The deliverables of the program included the development of an electronic health recording system (PRANA) and electronic vaccination system; the use of low citrate infusion in routine prophylactic automatic erythrocyte exchange (ARCE) programs including pregnant women; the use of leukocyte-filtered and irradiated blood for transfusion; the use of magnetic resonance imaging methods (T2∗) for the management of transfusion-related hemosiderosis; and the implementation of an allogeneic hematopoietic stem cell transplantation protocol for adult patients. The sample was composed of 376 study subjects and 249 control subjects. The hospital's Data Management System and the central population operating system were used for data collection. BASCARE enabled better analysis and interpretation of complication and mortality data. Vaccination rates against influenza and pneumococcal disease improved (21.5% vs 50.8% and 21.5% vs 49.2%, respectively). Effective and safe ARCE with low citrate infusion were maintained in 352 subjects (1003 procedures). Maternal and fetal mortality was prevented in 35 consecutive pregnant patients with ARCE. Chelating therapy rates reduced from 6.7% to 5%. Successful outcomes could be obtained in all 13 adult patients who underwent allogeneic peripheral stem cell transplantation from a fully matched, related donor. No patients died by day 100 or after the first year. Cure could be achieved without graft loss, grades III to IV acute graft versus host disease, extensive chronic graft versus host disease, or other major complications. The BASCARE program significantly improved patient care and thereby prolonged the life span of SCD patients (42 ± 13 years vs 29 ± 7 years, P < .001). We may recommend using such individualized programs in centers that provide health care for patients with SCD, in accordance with holistic approach due to the benign nature but malignant course of the disease.

Therapeutic plasma exchange (TPE) has been shown to hasten recovery in Guillain-Barré syndrome (GBS). In this study, the objective was to show the outcome of disability grade in a retrospective analysis of data of clinical experience of TPE using the COBE Spectra Apheresis system and other treatment options in selected patients from a series of 56 patients with GBS at a single treatment centre in Turkey.Ten patients had the acute motor axonal neuropathy (AMAN) subtype; 46 had the acute inflammatory demyelinating polyneuropathy (AIDP) subtype of GBS. Three hundred and eighteen TPE procedures were performed taking 2 to 3 hours: in 6.3 % of them a peripheral catheter was used; in 93.7 % of them a central catheter was used. Replacement fluids were fresh frozen plasma (FFP), lactated Ringer's solution or 3 % hydroxyethyl starch (HES). Among the patients, 12 (21.4 %) who had severe disease course received additional treatment to TPE -this was intravenous immunoglobulin (IVIG) in 11 patients. One patient was treated with steroids after rheumatology consultation due to another autoimmune disease. After 2 weeks, the mean GBS disability scores had significantly decreased from 3.75±0.48 to 2.44±0.96 (p=0.0001) and mean Medical Research Council (MRC) muscle strength scores significantly increased from 2.07±0.89 to 3.54±0.88 (p=0.0001). No difference in efficacy was observed between AMAN and AIDP subtypes. Adverse events occurred in 20 procedures (6.3 %) of TPE and were mostly transient hypocalcaemia and allergic reactions that did not necessitate treatment discontinuation. Difficulty in venous access was observed in 3.14 % of procedures. TPE using the COBE Spectra Apheresis system provides effective treatment of GBS with an acceptable safety profile using various replacement fluids and is an essential part of disease management. Although the benefit is controversial, other treatment options may be applied as an additional therapy in selected patients.

Aim/Background: Malignant diseases impair not only the quality of life of the patient but also the caregivers. The present study aims to investigate the quality of life in caregivers of the patients with hematologic neoplasms and determine the relationship between the subscales of quality of life (QOL) and socio-demographic characteristics of caregivers. Methods: One hundred consecutive caregivers of the patients who were hospitalized at Bone Marrow Transplant Unit and Hematology Department between 01 May 2015 and 30 June 2015 were included in this prospective, single center, cross-sectional study. Socio-demographic data of the patients and the caregivers were collected using a questionnaire form, QOL was evaluated using short-form 36. Results: Quality of life was found to be impaired in 46% of the caregivers. Given that a score of 100 defines full health status, bodily pain (31.3) and mental role (35.9) subscale scores were found to be the most affected subscales and physical functioning (71.8) subscale was found to be the least affected. There was a statistically significant difference between the age of the caregiver and physical role. Physical role and social functioning were better in caregivers who have economic support. There was a difference between employed and unemployed caregivers with regard to physical role and pain subscales. Overall health perception subscale score was higher for the caregivers of male patients. Graduates of the university were found to experience less pain. Conclusions: Cancer impairs not only the life quality of the patient but also the caregiver. So, the patient and the caregiver should be evaluated together and family physicians should take place in the center of and coordinate the interventions required for improving the quality of life of the caregivers.&nbsp;Giriş ve Amaç: Malign hastalıklar sadece hastanın değil, ailenin de yaşam kalitesini etkiler. Bu çalışmada hematolojik neoplazmı olan hastaların bakım verenlerinin yaşam kalitesinin araştırılması ve yaşam kalitesi alt ölçekleri ile bakım verenlerin sosyo-demografik özellikleri arasındaki ilişkinin belirlenmesi amaçlanmıştır. Yöntem: Bu prospektif, tek merkez, kesitsel çalışmaya 01 Mayıs 2015 ile 30 Haziran 2015 tarihleri arasında Başkent Üniversitesi Kemik İliği Nakli Ünitesi ve Hematoloji Bölümü’nde yatırılan yüz ardışık hastanın bakım verenleri alınmıştır. Hastaların ve bakım verenlerin sosyo-demografik verileri anket formu kullanılarak toplanmıştır, yaşam kalitesi ise kısa-form 36 ile toplanmıştır. Bulgular: Bakım verenlerin %46’sında yaşam kalitesinin bozulduğu bulunmuştur. Tam sağlık durumunu belirten skorun 100 olduğu düşünüldüğünde, vücut ağrısı (31.3) ve mental rol (35.9) en fazla etkilenen alt ölçekler iken fiziksel işlevselliğin (71.8) en az etkilendiği bulunmuştur. Bakım verenin yaşı ile fiziksel rol arasında istatistiksel olarak anlamlı fark vardı. Ekonomik desteği olan bakım verenlerin fiziksel rol ve sosyal işlev alt ölçekleri daha iyiydi. Çalışan ve çalışmayan bakım verenler arasında fiziksel rol ve ağrı alt ölçekleri bakımından fark vardı. Genel sağlık algısı alt ölçeği skoru erkek hastaların bakım verenlerinde daha yüksekti. Üniversite mezunlarının daha az ağrı yaşadıkları bulunmuştur. Sonuç: Kanser sadece hastanın değil, aynı zamanda bakım vericinin de yaşam kalitesini etkiler. Bu nedenle hasta ve bakım vericisi birlikte değerlendirilmeli, aile hekimleri bakım vericilerin yaşam kalitesinin artırılması için gereken girişimlerde merkezde olarak koordinasyon sağlamalıdır.