Comparison of the clinical course of COVID-19 infection in sickle cell disease patients with healthcare professionals.

It is highly expected that COVID-19 infection will have devastating consequences in sickle cell disease (SCD) patients due to endothelial activation and decreased tissue and organ reserve as a result of microvascular ischemia and continuous inflammation. In this study, we aimed to compare the clinical course of COVID-19 in adult SCD patients under the organ injury mitigation and clinical care improvement program (BASCARE) with healthcare professionals without significant comorbid conditions. The study was planned as a retrospective, multicenter and cross-sectional study. Thirty-nine SCD patients, ages 18 to 64 years, and 121 healthcare professionals, ages 21 to 53, were included in the study. The data were collected from the Electronic Health Recording System of PRANA, where SCD patients under the BASCARE program had been registered. The data of other patients were collected from the Electronic Hospital Data Recording System and patient files. In the SCD group, the crude incidence of COVID-19 was 9%, while in healthcare professionals at the same period was 23%. Among the symptoms, besides fever, loss of smell and taste were more prominent in the SCD group than in healthcare professionals. There was a significant difference between the two groups in terms of development of pneumonia, hospitalization, and need for intubation (43 vs 5%, P < 0.00001; 26 vs 7%, P = 0.002; and 10 vs 1%, P = 0.002, respectively). Prophylactic low molecular weight heparin and salicylate were used more in the SCD group than in healthcare professionals group (41 vs 9% and 28 vs 1%; P < 0.0001 for both). The 3-month mortality rate was demonstrated as 5% in the SCD group, while 0 in the healthcare professionals group. One patient in the SCD group became continously dependent on respiratory support. The cause of death was acute chest syndrome in the first case, hepatic necrosis and multi-organ failure in the second case. In conclusion, these observations supported the expectation that the course of COVID-19 in SCD patients will get worse. The BASCARE program applied in SCD patients could not change the poor outcome.

Role of prophylactic and therapeutic red blood cell exchange in pregnancy with sickle cell disease: Maternal and perinatal outcomes.

BACKGROUND AND AIM: The incidence of fetomaternal complications during pregnancy is high for women with sickle cell disease (SCD), which is the most common hematologic genetic disorder worldwide. Prophylactic red blood cell exchange (pRBCX) has been shown to be efficient, safe, and feasible for preventing complications. The aim of this study was to observe maternal, perinatal, and neonatal outcomes of pregnancies in which pRBCX was. METHOD: This was a single-center, retrospective, cross-sectional study, which recruited 46 consecutive adult pregnant women with SCD between January 2012 and June 2019. Obstetric features, SCD-related complications, and fetomaternal outcomes were compared between the 27 patients who received prophylactic exchange and the 19 who did not (therapeutic exchange was performed in 7 and was not performed in 12 cases). RESULTS: Painful crises, preeclampsia, and preterm birth rates were significantly higher in the group that did not receive prophylactic exchange (control group; P = .001, P = .024, and P = .027, respectively). There was one maternal mortality in the control group (P = .41). Incidence of adverse fetal or maternal complications was significantly higher in the control group (P = .044 and P = .007, respectively). CONCLUSIONS: Our center's experience over a 7.5-year period, as described above, demonstrates that pRBCX in SCD affects the course of pregnancy positively by ameliorating negative fetomaternal outcomes.

Significance of Lymphocyte Count, Monocyte Count, and Lymphocyte-To-Monocyte Ratio in Predicting Molecular Response in Patients with Chronic Myeloid Leukemia: a Single-Centre Experience.

BACKGROUND: Chronic myeloid leukemia (CML) is a disease resulting from BCR-ABL gene fusion. It is possible to monitor treatment by molecular testing for BCR-ABL. The lymphocyte-to-monocyte ratio (LMR) is a commonly used marker associated with prognosis in various neoplasms. This study was performed to evaluate the relevance of absolute lymphocyte count (ALC), absolute monocyte count (AMC), and LMR in predicting molecular response status in patients with chronic phase CML. METHODS: Samples submitted to our hematology laboratory for BCR-ABL testing between April 2012 and October 2018 were retrospectively reviewed. Concurrent hemogram testing together with the results of quantitative reverse transcriptase-polymerase chain reaction were noted. Data were grouped according to molecular response status and the ALC, AMC, and LMR were compared among patient groups. RESULTS: A total of 224 samples from 95 patients were included in the study. Analysis revealed differences between groups when newly diagnosed patients were compared with patients undergoing treatment, regardless of response status. However, analyzing the groups according to molecular response status failed to reveal differences in ALC, AMC, or LMR. CONCLUSIONS: ALC, AMC, and LMR are not potential biomarkers for predicting molecular response status in patients with chronic phase CML.

The Impact of the Ferric Carboxymaltose on Hemoglobin and Ferritin Levels.

BACKGROUND: Anemia is a frequent disorder worldwide. Iron deficiency anemia (IDA) is the most common form of anemia. Although oral iron is the first choice for treatment, the efficacy of oral iron preparations may be limited. Ferric carboxymaltose (FCM) is a novel parenteral iron preparation which can rapidly replenish iron stores. The aim of the present study is to investigate the impact of FCM dose on hemoglobin (Hb) and ferritin levels and the frequency of hypersensitivity reactions. METHODS: This study was conducted with 765 IDA patients between September 1, 2016 and September 1, 2018. He-moglobin (Hb), serum ferritin, transferrin saturation values were examined at the time of diagnosis, Hb and ferritin values at first month. RESULTS: Post-treatment Hb and ferritin levels significantly increased. The mean Hb level alteration was 2.43 ± 1.2 g/dL, the median ferritin level alteration was 157.3 ng/mL. The mean Hb level was lower and the mean change in Hb level was higher in higher doses. Allergic reactions were more frequent in higher doses. CONCLUSIONS: Ferric carboxymaltose is a novel treatment option with a low risk of hypersensitivity reactions and well tolerated even in high doses.

Granulocyte-colony stimulating factor administration among hemoglobin S trait donors: A single center experience from the Eastern Mediterranean region.

BACKGROUND AND OBJECTIVE: Assessment of Hemoglobin S trait donors has gained importance together with the increased allogeneic peripheral stem cell transplant activity for sickle cell disease in the regions where the disease is prevalent. Outcomes of Granulocyte-Colony Stimulating Factor (G-CSF) administration are obscure for hemoglobin S trait donors. This study aims at investigating the incidence of hemoglobin S carrier status and outcomes of G-CSF administration among donors who live in Eastern Mediterranean region. MATERIAL AND METHOD: The cross-sectional, single-center cohort study was performed with 147 donors between January 2013 and March 2017. Prevalence of hemoglobin S trait was estimated and subjects with or without Hemogobin S trait were compared with regard to stem cell characteristics, early and late clinical outcomes after G-CSF administration. RESULTS: Eleven out of 147 donors (7.48%) were found as hemoglobin S trait. G-CSF administration was successfully completed and yielded good harvesting results in hemoglobin S trait donors. No statistically significant difference was found between groups with regard to early and late side effects, stem cell characteristics. Blood pressures and QTc values were within normal ranges in both groups. Groups were similar with regard to CD34 values. CONCLUSION: G-CSF seems safe in hemoglobin S trait donors. Their being eligible as donors would increase the chance of the patients for allogeneic stem cell transplantation in high prevalence regions. Further studies are required to reveal the safety profile of G-SCF in hemoglobin S carriers in different regions.

QTc prolongation during peripheral stem cell apheresis in healthy volunteers.

BACKGROUND AND AIM: Today, voluntary donation of peripheral blood stem cells by healthy donors for allogeneic hemopoietic cell transplantation is common worldwide. Such donations are associated with small but measurable risks of morbidity and mortality. Most complications are associated with citrate infusion during cell collection. We studied the effects of citrate infusion on the QTc and other vital parameters during and after peripheral stem cell apheresis in volunteers. METHOD: To ensure that donors were healthy, screening included taking a detailed medical history, physical examination, and laboratory measurements of plasma calcium and magnesium. Corrected QT (QTc) values were assessed using a 12-lead electrocardiographic platform that derived QTc values automatically. RESULTS: In all, 141 apheresis procedures were performed. The mean QTc values at baseline, at 2 and 4 h during the procedure, and at 30 min after the procedure, were 347.6 ± 59.5, 349.9 ± 52.8, 391.8 ± 54.0, and 404.8 ± 59.2 ms, respectively. The baseline and 2 h QTcs did not differ significantly, but the baseline QTc did differ significantly from the 4 h and 30 min after the procedure values. The plasma levels of calcium and magnesium did not significantly differ before and after the procedure. CONCLUSION: QTc prolongation may develop during leukopheresis, particularly if the procedure takes more than 2 h. Thus, to enhance donor safety, QTc measurement should be standard for all donors. In addition, any family history of sudden death should be noted, to prevent the development of possible fatal arrhythmia in susceptible donors.

Significance of electronic health records: A comparative study of vaccination rates in patients with sickle cell disease.

OBJECTIVE: In this study, we investigated the influence of electronic health records (EHR) and electronic vaccination schedule applications on the vaccination status of patients who were admitted to our Center for the treatment of sickle cell disease (SCD). METHODS: The vaccination status against influenza and pneumococcus infection was determined in 93 patients who were admitted to the hematology outpatient clinic, Baskent University Adana Hospital from April 2004 to March 2009. The vaccination status was then re-evaluated following establishment of EHR and electronic vaccination schedules in 2012. RESULTS: Of the 93 patients with SCD 21.5% (n = 20) were vaccinated against pneumococcus and 21.5% (n = 20) were regularly vaccinated against influenza. When the vaccination rates of 59 of 93 patients who presented for their regular control examinations were analyzed following establishment of EHR and vaccination schedules in 2012, these rates were 49.2% (n = 29) and 50.8% (n = 30) for influenza and pneumococcus, respectively, after EHR; there were 23.7% (n = 14) and 20.3% (n = 12), respectively, before EHR. A statistically significant difference was found between the vaccination rates before and after EHR (p < 0.05). CONCLUSION: Although viral and bacterial infections are life-threatening health problems in patients with SCD, the vaccination rates were low in high-risk patients. However, these rates increased after application of electronic vaccination schedules.

East Mediterranean region sickle cell disease mortality trial: retrospective multicenter cohort analysis of 735 patients.

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-ß(0) thalassemia (17 %), Hgb S-ß(+) 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.

Factors effecting influenza vaccination uptake among health care workers: a multi-center cross-sectional study.

BACKGROUND: The present study aimed to identify factors affecting vaccination against influenza among health professionals. METHODS: We used a multi-centre cross-sectional design to conduct an online self-administered questionnaire with physicians and nurses at state and foundation university hospitals in the south-east of Turkey, between 1 January 2015 and 1 February 2015. The five participating hospitals provided staff email address lists filtered for physicians and nurses. The questionnaire comprised multiple choice questions covering demographic data, knowledge sources, and Likert-type items on factors affecting vaccination against influenza. The target response rate was 20 %. RESULTS: In total, 642 (22 %) of 2870 health professionals (1220 physicians and 1650 nurses) responded to the questionnaire. Participants' mean age was 29.6 ± 9.2 years (range 17-62 years); 177 (28.2 %) were physicians and 448 (71.3 %) were nurses. The rate of regular vaccination was 9.2 % (15.2 % for physicians and 8.2 % for nurses). Increasing age, longer work duration in health services, being male, being a physician, working in an internal medicine department, having a chronic disease, and living with a person over 65 years old significantly increased vaccination compliance (p < 0.05). We found differences between vaccine compliant and non-compliant groups for expected benefit from vaccination, social influences, and personal efficacy (p < 0.05). Univariate analysis showed differences between the groups in perceptions of personal risks, side effects, and efficacy of the vaccine (p < 0.05). Multivariate analysis found that important factors influencing vaccination behavior were work place, colleagues' opinions, having a chronic disease, belief that vaccination was effective, and belief that flu can be prevented by natural ways. CONCLUSION: Numerous factors influence health professionals' decisions about influenza vaccination. Strategies to increase the ratio of vaccination among physicians and nurses should consider all of these factors to increase the likelihood of success.

Medication adherence to oral iron therapy in patients with iron deficiency anemia.

OBJECTIVE: This study aimed at investigating the factors affecting medication adherence in patients who use oral iron therapy due to iron deficiency anemia. METHODS: A total of 96 female patients in fertile age with mean age of 30±10.1 years (range 18-53) who were admitted to Family Medicine Clinic between 01 January and 31 March 2015 and who had received iron therapy within the recent three years were enrolled in the study. Data were collected through a questionnaire form. RESULTS: Of the patients, 39 (40,6%) were detected not to use the medication regularly or during the recommended period. A statistically significant relationship was found between non-adherence to therapy and gastrointestinal side effects and weight gain (p<0.05). CONCLUSION: Medication adherence is deficient in patients with iron deficiency anemia. The most important reason for this seems gastrointestinal side effects, in addition to weight gain under treatment.

Gonadal Status and Sexual Function at Long-Term Follow-up after Allogeneic Stem Cell Transplantation in Adult Patients with Sickle Cell Disease.

OBJECTIVES: Patients with adult sickle cell disease and severe sequelae are treated with nonmyeloablative allogeneic hematopoietic stem cell transplant. So far, data on gonadal effects are lacking for older cured patients. We assessed the gonadal reserve and sexual function of patients cured of sickle cell disease with transplant and with anti-T-lymphocyte globulin and posttransplant cyclophosphamide-containing regimen within the context of the Baskent Organ Damage Mitigation and Medical Care Development Program. MATERIALS AND METHODS: All adult patients (≥18 years) with sickle cell disease who underwent peripheral stem cell transplant from September 2013 to July 2019 and were graft-versus-host disease free for 2 years and not immunosuppressed were invited to participate in this prospective observational study. Of 61 eligible patients, 43 participants (~10% from international registries) were included (median age at transplant was 29 years; range, 18-45 years). Gonadal status, risk of gonadal damage posttransplant, conception, and sexual function posttransplant were evaluated. RESULTS: Allogeneic hematopoietic stem cell transplant was associated with increased risk of secondary amenorrhea (odds ratio of 93; 95% CI, 4.94-17.50; P = .002) and ovarian insufficiency (odds ratio of 37.8; 95% CI, 2.03 to -700.94; P = .014) but not with female sexual dysfunction. Secondary ovarian insufficiency developed in all women posttransplant. Transplant was associated with significant risk of azoospermia (odds ratio of 4.35; 95% CI, 1.02-18.45; P = .017). Moderate-to-severe erectile dysfunction developed in 2 men (10%). Among female participants, 1 had spontaneous conception that ended in miscarriage and 1 had term delivery after in vitro fertilization. Among male participants, 1 had a child by in vitro fertilization and 1 experienced spontaneous conception. CONCLUSIONS: Although spontaneous conception was shown in our patient group, gonadal damage was evident at >2 years posttransplant. This risk was associated with age in female patients. Better fertility preservation measures should be incorporated into medical care development programs.

Factors Associated With Overall Survival in Acute Myeloid Leukemia Patients Before and After Hematopoietic Stem Cell Transplant.

OBJECTIVES: Our aim was to identify factors associated with overall survival and the efficacy of postrelapse treatment protocols and to determine whether pretransplant consolidation therapy and minimal residual disease status pose a survival benefit. MATERIALS AND METHODS: Patients with acute myeloid leukemia who underwent stem cell transplant between 2007 and 2018 were enrolled retrospectively. The effects of pretransplant cytogenetic and minimal residual disease status, pretransplant consolidation therapies, development of graft-versus-host disease, postrelapse treatment protocols, and type of con-ditioning regimens on overall survival were analyzed. RESULTS: In 76 study patients, the cumulative overall 1- and 5-year relapse probabilities were 67.8% and 58.7%, respectively. Overall survival rates at 3 and 5 years in patients with and without relapse were 23.5% and 0% and 95.9% and 91.1% (P < .001), respectively. Although mean postrelapse overall survival was better with intensive salvage plus donor lymphocyte infusion, no significant differences were shown versus other therapies (intensive salvage, nonintensive salvage, intensive salvage or nonintensive salvage plus donor lymphocyte infusion, or supportive therapy). Twenty-three patients (30.3%) died during the study period with a median survival of 9.6 months. Patients with favorable, intermediate, and unfavorable cytogenetic status showed overall survival of 46.6 ± 10.4, 54.6 ± 4.4, and 36.9 ± 5.9 months (P = .807). Patients with and without minimal residual disease and patients who received or did not receive consolidation therapy had similar overall survival. Relapse was an independent predictor of overall survival (increased mortality risk of 26.22). Patients who developed graft-versus-host disease showed decreased relapse. CONCLUSIONS: Relapse is the most important predictor of overall survival and is associated with poor prognosis. Pretransplant minimal residual status and cytogenetic status showed no effect on relapse rates and overall survival, and consolidation therapy did not improve outcomes.

Comparison of Outcomes of Transplant and Nontransplant High-Risk Chronic Lymphocytic Leukemia Patients in the Novel Targeted Therapy Era.

OBJECTIVES: Our aim was to compare patients with high-risk chronic lymphocytic leukemia referred to our transplant center who underwent allogeneic stem cell transplant with those who did not receive this treatment. Factors compared included demographics, clinical characteristics, and survival rates in the novel targeted therapy era. MATERIALS AND METHODS: All 33 patients with high-risk chronic lymphocytic leukemia who were referred to the hematopoietic stem cell transplant center were enrolled in this retrospective, single-center nonrandomized study. Outcomes of patients who received allogeneic stem cell transplant were compared with those of nontransplant patients. Chemoimmunotherapy and ibrutinib were given when indicated. Factors related to overall and progression-free survival were assessed. RESULTS: Thirteen patients underwent allotransplant, and transplant was not done in 20 patients for various reasons. Demographic and clinical features of the transplant and nontransplant groups were similar. The estimated cumulative overall survival was 72.6 ± 15 and 84.3 ± 13 months in the nontransplant and transplant groups, respectively. The 5-year overall and progression-free survival rates in the transplant and nontransplant groups were 57.3%/36.0% and 40%/20.6%, respectively. In the nontransplant group, overall survival of those who used ibrutinib was longer than overall survival in patients in the transplant group who used the same drug, but the difference was not statistically significant. Although not significant, overall survival in patients who did not use ibrutinib was longer in the transplant group than in the nontransplant group. Cox regression analyses showed that transplant, relapse, and Binet stage were independent predictors of overall survival. CONCLUSIONS: In patients who do not use ibrutinib, allogeneic stem cell transplant improved survival compared with nontransplant patients. Addition of ibrutinib provided comparable life expectancies, showing that allogeneic stem cell transplant and ibrutinib may have complementary roles. Transplant is still an independent predictor of overall survival.

Organ damage mitigation with the Baskent Sickle Cell Medical Care Development Program (BASCARE).

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 ±â€Š7 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.

Comparative efficacy in red blood cell exchange transfusions with different apheresis machines in patients with sickle cell disease.

The purpose of this study is to compare the efficacy and side effects of two red blood cell exchange (RBCX) transfusion systems in sickle cell disease (SCD). The data is collected retrospectively from the January 2010 to March 2015. 447 RBCX transfusions were performed to 165 patients. Side effects, clinical and technical efficacy were compared in between procedures with Cobe Spectra (CS) and Spectra Optia (SO) systems. Furthermore a subgroup analyses was performed for 40 patients who had RBCX transfusions with both system at least two times. Vasoocclusive crises, preoperative period and foot ulcers (49.6, 13, and 15.2% respectively) were the common indications of RBCX transfusion. While the levels of post-RBCX HbS and the actual fraction of cells remaining (FCRa) were found significantly higher in the SO compared to CS system (p = 0.018 and p = 0.016 respectively), the rate of targeted hemoglobin S (HbS) levels (< 30%) were same in both. The length of procedure and replacement volume were significantly lower in SO compared to CS system (p = 0.025 and 0.044 respectively). Subgroup analyses of 40 patients did not reveal any statistically significant differences regarding post-procedure HbS levels, FCRa levels, replacement volume and procedure duration. The inter-rater correlation coefficient for FCRa was calculated to be 0.82. Serious adverse reactions were not observed from either system. Both systems are efficiently achieved the targeted post-procedure HbS level. The recently introduced SO apheresis system is as effective and safe as the CS system, which has been used for all indications of SCD for years.

Effectiveness of Visual Methods in Information Procedures for Stem Cell Recipients and Donors.

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.

Development of Acute Promyelocytic Leukemia in a Patient With Gouty Arthritis on Long Term Colchicine.

Colchicine is a frequently used drug in rheumatological diseases. Acute promyelocytic leukemia developed in a patient who used colchicine for gouty arthritis since 10 years is presented and the possible relation between the long term use of colchicine and hematological malignancies is discussed.

Active Invasive Fungal Infection in a Patient With Severe Aplastic Anemia.

Severe aplastic anemia is almost always fatal unless treated. Invasive fungal infections, particularly those caused by Aspergillus species, have long been recognized as a major cause of death in severe aplastic anemia. However, there are few specific reports about infections and their therapy in patients with aplastic anemia. Despite improvements in the last few years, the response rate of new antifungal drugs, such as voriconazole and liposomal amphotericin B, has only about a 30% recovery rate in patients with severe neutropenia and persistent fever. There is a paucity of data available about hematopoietic stem cell transplant under active invasive fungal infection in the literature. Therefore, we aimed to discuss the treatment scenarios for 2 severe aplastic anemia patients who have invasive fungal infections.

Anorectal Complications During Neutropenic Period in Patients with Hematologic Diseases.

BACKGROUND: Neutropenic 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. METHODS: A total of 79 patients whose neutropenic period (absolute neutrophil count <500/mcL) continued for 7 days, or longer were included in the study. RESULTS: A 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). CONCLUSION: Anorectal 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.

A Rare Complication Developing After Hematopoietic Stem Cell Transplantation: Wernicke's Encephalopathy.

Thiamine is a water-soluble vitamin. Thiamine deficiency can present as a central nervous system disorder known as Wernicke's encephalopathy, which classically manifests as confusion, ataxia, and ophthalmoplegia. Wernicke's encephalopathy has rarely been reported following hematopoietic stem cell transplantation. Herein, we report Wernicke's encephalopathy in a patient with acute myeloid leukemia who had been receiving prolonged total parenteral nutrition after haploidentical allogeneic hematopoietic stem cell transplantation. To the best of our knowledge, this is the first case reported from Turkey in the literature.