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© 2021 Edizioni Scientifi che per l'Informazione su Farmaci e Terapia.The prognosis is poor for relapsed or refractory (R/R) classical Hodgkin Lymphoma (cHL) patients. The brentuximab vedotin (Bv) and bendamustine (B) combination has been used as a preferable salvage regimen in R/R cHL patient trials. We retrospectively evaluated response rates, toxicities, and the survival in R/R cHL patients treated with the BvB combination. In a multi-centre real-life study, 61 R/R HL patients received intravenous doses of 1.8 mg/kg Bv on the first day plus 90 mg/m2 B on the first and second days of a 21-day cycle as a second-line or beyond-salvage regimen. Patients’ median age at BvB initiation was 33 (range: 18–76 years). BvB was given as median third-line treatment for a median of four cycles (range: 2–11). The overall and complete response rates were 82% and 68.9%, respectively. After BvB initiation, the median follow-up was 14 months, and one- and two-year overall survival rates were 85% and 72%, respectively. Grade 3/4 toxicities included neutropenia (24.6%), lymphopenia (40%), thrombocytopenia (13%), anaemia (13%), infusion reactions (8.2%), neuropathy (6.5%), and others. The BvB combination could be given as salvage regimen aiming a bridge to autologous stem cell transplant (ASCT), in patients relapse after ASCT or to transplant-ineligible patients with manageable toxicity profiles.

In Turkish literature there are very few studies regarding multiple primary malignancies (MPM). The aim of this study was to analyze the synchronous and the metachronous malignancies that occurred with a hematologic malignancy. All the patients with a hematologic malignancy were enrolled in this cross-sectional, definitive retrospective study. Data were obtained from the medial records. Patients characteristics including demographic features, treatment protocols and overall survival (OS) were recorded. Among 663 patients with a hematologic malignancy, there were 26 patients with MPMs (3.9%). Synchronous malignancies constitute 0.9% and metachronous malignancies were present in 3%. In men diffuse large B-cell lymphoma (DLBCL) and non-small cell lung carcinoma (NSCLC) and in women breast and acute myeloid leukemia were the most common primary and secondary MPMs respectively. The mean cumulative OS of all patients with MPMs was 246.3±33.4 months and the 5 years-OS was 91.3%. In synchronous MPMs the most frequent concomitant tumors were DLBCL and NSCLC. In metachronous tumors the median time interval between first and second malignancies was 69.5 months (range: 31-312). In four patients there were three MPMs. After radiotherapy three patients developed breast, thyroid and skin cancers and in one patient who received radioiodine for the treatment of thyroid carcinoma, DLBCL had developed. The chemotherapeutic agents applied for the primary malignancies consisted of alkylating agents, antimetabolites, anthracyclines, topoisomerase II inhibitors, monoclonal antibodies and mitotic inhibitors. In 75% of the patients with DLBCL who had received R-CHOP chemotherapy regimen, NSCLC had developed during the follow-up period. In conclusion secondary malignancies with hematologic malignancies are not rare and the clinicians should keep the possibility of secondary malignancies in mind and be suspicious during diagnostic evaluations. Warning with regard to the risk of development of secondary malignancies due to the primary treatment should be given to any patient with a hematologic malignancy. [Med-Science 2020; 9(1.000): 94-9]

Objectives The use of unrelated donors as a source of stem cells for patients with blood disorders continues to increase. Approximately 5% to 7% of unrelated stem cell donors are asked to donate stem cells a subsequent time to the same or a different patient. We investigated donors who accepted to be a donor for the second time between 2015 and 2021; donors were evaluated in terms of procedure-related complications, product quality, and donor follow-up in a JACIEaccredited (Joint Accreditation Committee of the International Society for Cellular Therapy and European Society for Blood and Marrow Transplantation). Materials and methods Stem cell collections were performed in accordance with relevant standard operating procedures from healthy volunteer donors. Data on sequence of peripheral blood stem cell, bone marrow, and donor lymphocyte collection procedures; presence of complications during procedures; time between 2 donations; need for granulocyte colonystimulating factor again; and first and second donation types were noted. Data on donor and stem cell products were determined using the hospital information management system. Results Our study included 12 donors (9 men and 3 women) who donated a second time within the specified date range. In the evaluation of the second donation types, 7 were lymphocyte collection donations, 4 were peripheral blood stem cell donations, and 1 was a bone marrow stem cell donation. In shortterm and long-term follow-ups, there were no complications among the donors. In the second donations, targeted product values were reached. Conclusions Although it is safe to have a second donation from a donor for the same patient, collection centers may collect more products than requested from eligible donors.

Despite recent improvements in the treatment options, adult relapsed/refractory Acute Lymphoblastic Leukaemia (ALL) and lymphoblastic lymphoma (LBL) exhibit poorer cure rates than in childhood. Since, the mainstay difference of childhood multidrug regimens is L-Asparaginase, we sought to salvage adult patients with a protocol containing methotrexate, vincristine, conventional L-asparaginase, and dexamethasone (MOAD). In this study, we aimed to summarize our experience. Methods: Adult patients with relapsed/refractory ALL and LBL followed-up in our institution between 2017 and 2018 were reviewed and those treated with MOAD protocol were retrospectively included in the study. Clinical data, treatment responses, and adverse events were summarised. The protocol featured 28-day cycles of methotrexate 200 mg/m2 intravenously (IV) on days 1 and 15; vincristine 1.4 mg/m2 IV on days 1, 8, and 15; L-asparaginase 10,000 IU/m2 IV twice weekly; and dexamethasone 40 mg IV or orally on days 1–4 and 15–18. Results: A total of eight patients were enrolled, of median age 37 years (range: 21–58 years). Four patients were recovered after transplantation. Complete remission was evident in 38%. Two such patients underwent allogeneic hematopoietic stem cell transplantation after the protocol. Another patient with lymphomatous disease achieved partial remission and underwent successful transplantation. L-asparaginase did not trigger any clinically evident hypersensitivity reaction; the most common adverse events associated with the protocol were hypofibrinogenemia, anemia, and febrile neutropenia. Conclusions: The MOAD protocol was effective and tolerable, enabling to salvage before and after transplantation, particularly in patients with relapsed/refractory T-cell acute lymphoblastic leukemia and lymphoblastic lymphoma.

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.

Background Approximately half of patients with relapsed chemosensitive disease achieve robust responses with BEAM (BCNU, etoposide, cytarabine, and melphalan) and autologous stem cell rescue. The scarcity of comparative studies further limits alternative treatment protocols, such as the MITO/MEL (mitoxantrone, melphalan) protocol. Patients and Methods In this retrospective multicenter study, we compared the BEAM and MITO/MEL regimens used before autologous hematopoietic stem cell transplantation (ASCT) in terms of efficacy and side effects in patients with Hodgkin lymphoma. Data met international accreditation rules. Before ASCT, 108 patients received the MITO/MEL, and 34 patients received the BEAM. Results The median follow-up time was 36 months in the MITO/MEL group (range, 3-178) and 23 months in the BEAM group (range, 4-99). After ASCT, the 3-year expected overall survival and disease-free survival rates were 86.1% and 86.1% for the MITO/MEL group and 91.3% and 76.5% for the BEAM group, respectively. Although 50% of patients developed febrile neutropenia attacks in the MITO/MEL group, this rate was 91.1% in the BEAM group. The grade II and higher rates of hepatic, renal, gastrointestinal, and cardiac toxicities were similar in both groups. However, the rate of pulmonary toxicity was determined to be 1.9% in the MITO/MEL group and 29.4% in the BEAM group (P Conclusion The MITO/MEL conditioning regimen seems to be as effective as the BEAM regimen but has better tolerability in terms of pulmonary toxicity and may be used as an alternative option if necessary, depending on the comorbidity status of the patient.

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.

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.

Amaç: Birçok faktör hematopoetik kök hücre (HKH) engraftmanını etkiler. Bu çalışmada hematolojik kanseri olan hastalarda kemik iliğindeki myelofibrozis ve anjiogenezisin engraftman üzerine etkisi araştırılmıştır. Gereç ve Yöntem: Otolog kök hücre nakli yapılan 34 hasta (20 erkek, 14 kadın) verileri değerlendirildi. Nakilden önceki son kemik iliğindeki fibrozis 0-3 arasında derecelendirildi, anjiogenezis stereolojik metod ile ölçüldü. Hastalar anjiogenezis parametrelerinin yoğunluğuna göre de iki gruba ayrıldı. Bulgular: Hastaların çoğunluğu (%73.5) multipl myelom idi ve yarısında fibrozis saptandı. On bir hasta derece 1, 6 hasta derece 2 fibrozise sahipti. Trombosit ve eritrosit engraftman günleri açısından derece 2 fibrozisi olan grupla fibrozis saptanmayan grup arasında istatistiksel anlamlı farklılık vardı. Derece 1 ve 2 fibrozis gruplarında fibrozisi olmayanlara göre VSD ve NVES düzeyleri anlamlı olarak yüksekti. Toplam yaşam derece 2 fibrozisi olan grupta daha düşük olmakla birlikte istatistiksel anlamlılık yoktu. Sonuç: Kemik iliği myelofibrozisinin bağımsız bir risk faktörü olduğu saptanmıştır. Bunun toplam yaşamı etkilemeden otolog nakil sürecinde trombosit ve eritrosit engraftmanı üzerine negatif etkisi olabilir. Fibrozisin derecesi ile doğru orantılı olarak artmış anjiogenezis arasındaki ilişkiyi açıklamak için ileri çalışmalar gereklidir. Purpose: Hematopoietic stem cell (HSC) engraftment is influenced by many factors. We investigated the effects of bone marrow fibrosis and angiogenetic structure on engraftment in patients with hematological malignancies. Materials and Methods: Data were collected from 34 patients (20 males and 14 females) who underwent autologous HSC transplantation. Bone marrow myelofibrosis was graded from 0 to 3, angiogenesis was quantified using a stereological method in the most recent bone marrow biopsy before the transplantation. Patients were categorized into two groups according to intensity of angiogenesis parameters. Results: Half of the patients had fibrosis and majority had multiple myeloma (73.5%). Eleven patients had grade 1, six had grade 2 myelofibrosis. The engraftment day (ED) for platelets and erythrocytes was significantly different between the grade 2 fibrosis and non-fibrosis groups. VSD and NVES levels were significantly higher in the grades 1 and 2 fibrosis groups than the no fibrosis group. While the overall survival time was shorter in the grade 2 fibrosis group than the others, the difference was not statistically significant. Conclusion: Bone marrow fibrosis was found to be independent risk factor. It may have a negative effect on platelet and erythrocyte engraftment time of autologous transplantation process but this effect does not influence survival.

Amaç: Orak hücre hastalığının sık görüldüğü bölgemizde eritrosit alloimmmünizasyon sıklığını ve ilişkili durumları araştırmayı amaçladık.Gereç ve Yöntem: bu çalışma tek merkezli, zamansal kesitli ve geriye dönük kohort çalşıması olarak planlanmıştır. Toplamda 216 orak hücre hastalığı [Hemoglobin (Hb) SS, Hb S-β talasemi, Hb S-α talasemi] tanısı olan hasta çalışmaya dahil edilmiştir. Hastalar transfüzyon miktarına gore iki gruba ayrılmıştır. Yılda 6’dan daha az transfüzyon alan ya da eritroferez öyküsü olmayan hastalar Grup 1’e, yılda 6 ve daha fazla basit transfüzyon alan ya da eritroferez işlemine alınan hastalar Grup 2’ye dahil edilmiştir.Bulgular: Çalışmamıza 216 hasta dahil edilmiştir. Transfüzyon tedavisi alan toplam 216 hastanın 67 (%31.0)’sinde, Grup 1’deki 56 hastanın 17’sinde (%30.4), Grup 2’deki 160 hastanın 50’sinde (%31.3) tespit edilmiştir. Hastalar alloimmünizasyon gelişimi açısından analiz edildiğinde, çalışmamız ne orak hücre komplikasyonlarının alloimmünizasyon gelişimi için ne de alloimmünizasyonun ölüm için bir risk faktörü olmadığını göstermiştir.Sonuç: Çalışmamamızda bulunan yüksek alloimmünizasyon sıklığı, tecrübeli merkezler dışında yapılan transfüzyonlarda alloimmünizasyonu önleyici politikalara yeteri kadar uyulmadığı konusunda fikir vermektedir. Bu nedenle orak hücre anemili hastalarda alloimmünizasyon, ayrıntılı eritrosit antijen tanımlama işlemi yapılarak azaltılabilir veya önlenebilir., Purpose: We aimed to investigate erythrocyte alloimmunization frequency and related factors in our region where SCD is common.Material and Methods: This study was planned as a single center, cross-sectional and retrospective cohort study. A total of 216 patients who had been followed up due to SCD [Hemoglobin (Hb) SS, Hb S-β thalassemia, Hb S-α thalassemia] were included in this study. Patients were divided to two groups according to amount of transfusion. The patients who had received less than 6 transfusions per year and who did not have the history of erythropheresis were allocated to Group 1, and the patients who had received 6 or more simple transfusion per year or who had undergone erythrocyte exchange were allocated to Group 2.Results: Of 216 SCD patients included in the study. Alloimmunization was detected in 67 (31.0%) out of 216 patients who underwent transfusion, and in 17 (30.4%) out of 56 patients in Group 1 and in 50 (31.3%) out of 160 patients in Group 2. When the patients were analyzed according to alloimmunization development, our study revealed that neither SCD complications are a risk factor for alloimmunization nor alloimmunization increases mortality rates.Conclusion: High alloimmunization frequency found in our study suggests the insufficient adherence of alloimmunization-prevention policies in RBC transfusions performed except experienced institutions. Therefore alloimmunization may be reduced or prevented through performing extended red cell typing among SCD 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.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.

Background The profile of leukocytes in bronchoalveolar lavage (BAL) fluid provides important information for diagnosing various lung diseases. A differential cell count of BAL is conventionally performed by evaluating centrifuged samples under a light microscope and enumerating the stained cells. Another rarely used method to identify BAL leukocytes is flow cytometry (FCM). However, there are no guidelines for standardizing this method and related literature is limited. This study aimed to evaluate the accuracy of FCM for identifying BAL leukocytes. Methods The BAL samples accepted to the hematology laboratory between 2014 - 2018 were retrospectively evaluated via light microscopy (LM) by a hematologist; while flow cytometric analyses with a monoclonal antibody panel composed of CD45/CD14/CD16 were noted by another doctor. The percentages of macrophages, lymphocytes, neutrophils and eosinophils determined by both methods were recorded for analysis. Correlations between the results from LM and FCM were investigated. In addition, compatibility between LM and FCM for denoting pathological values for each cell type was checked. Results Among 140 reviewed BAL samples, 76 were included for further analysis. Comparisons revealed strong correlations between FCM and LM for identifying macrophages, lymphocytes, neutrophils, and eosinophils. In addition, regarding the normal cutoff values for each leukocyte type, FCM and LM were similar in the identification of pathological changes of all cell types except eosinophils. Conclusions Flow cytometry was found to be feasible for use instead of LM and might become a more widely used technique to analyze BAL fluid in the future.

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.

No Abstract [Med-Science 2020; 9(1.000): 287-8]

Allogeneic hematopoietic stem cell transplant is a curative treatment option for many hematologic diseases. The existence of a fully compatible donor for recipients is the first condition for minimized transplant-related mortality and morbidity. The best donor for hematopoietic stem cell transplant is an HLA-matched sibling donor. The possibility of finding an HLA-matched sibling is less than 30% worldwide. Hematopoietic stem cell transplant is needed for an increasing number of patients every year, but the ability to find a fully compatible donor has limited its use.From August 2012 to May 2017, we screened 412 adult patients who required AHSCT and their families for HLA tissue groups who were seen at our center (Baskent University Adana Dr. Turgut Noyan Research and Medical Center Hematology Unit). To screen tissue groups at our center, we perform lowresolution typing for HLA-A, -B, -C, -DRB1, and -DQB. If an HLA genotype cannot be identified, verification typing is done using highresolution testing.We found matched family donors in 227 (55%) of 412 patients screened at our center. The ratio of HLAmatched related donors was 83% for 279 patients who received allogeneic stem cell transplant.The likelihood of finding eligible unrelated donors has been gradually increasing, in part due to the development of the National Bone Marrow Bank. However, a careful screening for related donors is still important. Our findings indicate the importance of careful examination of family genealogy and of careful family screening in our region.

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 (

Although it is presumed to be a curative strategy for intermediate and high risk acute myeloid leukemia (AML), many patients relapse after allogeneic hematopoietic stem cell transplantation. This prompt us to examine the ways to improve the outcomes. We retrospectively evaluated 76 AML patients who were transplanted between 2007-2017 years in our clinic. We tried to identify the factors associated with posttransplant relapse, postrelapse survival and if there was a survival benefit of pretransplant consolidation and minimal residual disease (MRD) negativity. We examined the effect of the acute-chronic graft versus host disease (GVHD) and salvage therapy after the posttransplant relapses. The mean age of the patients was 44.6±1.21 years (ranges 21-67). 42.1% were females and 57.9% were males. 43.3% of the patients were in complete remission (CR) MRD positive state before the transplantation whereas 35.5% were in CR MRD negative and 3.9% were in progressive disease state. In 13 patients who were in CR state, the MRD status were not known. 11 (14.5%) patients were considered as in favorable risk, 52 (68.4%) in intermediate risk and 13 (17.1%) in unfavorable risk with respect to cytogenetic analysis before the transplantation. The donors were HLA compatible relatives (77.6%), HLA compatible non-relatives (10.5%), haploidentical people (9.2%), one HLA incompatible relative (1.3%) and one HLA incompatible nonrelative (1.3%). 74 bone marrow transplantations (97.4%) were allogeneic and the remaining two (2.6%) were autologous. Myeloablative conditioning regimen was applied to 57 patients (75%) and 19 patients got (25%) reduced intensity conditioning regimen. GVHD developed in 51.3% after transplantation and 61.5% of these were chronic extensive. Relapse occurred in 27 patients (35.5%), hematological relapse being the most common (31.6%). The median time for the development of relapse was found to be 5.5 months (range: 1.5-37). The overall probability for the development of a relapse was found to be 48.7% (95% CI: 40.9-56.5)(Figure1A). 23 patients (30.3%) died during the study period with a median survival of 9.6 months (range: 1.6-45). In the studied population the overall survival probability was found to be 52.8% (95% CI: 45.4 - 60.2) [Figure 1B]. 36.4%, 28.8% and 30.8% of the patients with favorable, intermediate and unfavorable cytogenetic status died respectively during the study period. The comparison of the survival probability of the patients with favorable, intermediate and unfavorable cytogenetic status was depicted in Figure 2. The overall survival probability of the patients with favorable, intermediate and unfavorable cytogenetic status were 46.6% (95% CI: 26.2-66.9), 54.6% (95 % CI: 45.9 - 63.2) and 36.9 % (95% CI: 25.4 - 48.5) respectively (p=0.807). MRD status of 60 patients were known. At the end of the study period 75.8% of the CR MRD positive and 70.4% of the CR MRD negative patients remained alive. The comparison of the survival of patients in CR with respect to MRD status is shown in Figure 3. The overall survival probability of CR MRD positive patients was 56.3 % (95% CI: 45.3 - 67.3) and this rate was 52.5 % (95% CI: 40.8 - 64.3) in MRD negative patients (p=0.770) [Figure 3]. Patients who developed GVHD had similar overall survival probability with the patients who did not developed the disease; 47.0% vs 57.2%, p=0.115 (Figure 4A). Even the patients with chronic extensive GVHD had similar overall survival rates with the patients who had none or acute GVHD; 49.3 % vs 58.2%, p=0.27 (Figure 4B). 66.7% of the patients with a progressive disease before the transplantation died during the study period and this rate was 27.1% in the patients with CR (p=0.005). In conclusion the overall survival rate of the transplanted AML patients was 52.8% in the study group. The overall survival did not seem to be affected by pre-transplant MRD status, cytogenetic risk factors and administration of consolidation therapy. The only patients who had significantly worse results were the ones who had progressive disease before the transplantation. From this point it would be logical to make transplantation whenever the patient is in first CR regardless of the MRD status and a matched donor is found so that the toxic effects of the consolidation chemotherapy may be prevented. Disclosures No relevant conflicts of interest to declare.

Multiple myeloma (MM) is a disease of the elderly. It shows rapidly increasing incidence with increased age. Although MM is rare in young people, patients diagnosed before the age of 50 years account for 10% of the overall incidence and approximately 2% are diagnosed before 40 years of age. The aim of the study was to assess the clinical results of the young MM patients diagnosed at the age of ≤45 years. All the MM patients who were ≤45 years old at the time of diagnosis and who were admitted to our clinic between January 2004 and December 2017 were retrospectively assessed. There was a total of 31 patients and 14 (45.2%) of them were females and 17 (54.8%) of them were males. The mean age was 40.4±3.8 years. The most frequent myeloma types were Ig G Kappa (n:12, 38.7%) and Kappa light chain (n: 10, 32.3%). The remaining types were Ig A lambda (n:4, 12.9%), Ig G lambda (n:2, 6.5%), Lambda light chain (n: 2, 6.5%) and non-secretory myeloma (n:1, 3.2%). Before transplantation 12 patients (38.7%) were in complete remission, 11 patients (35.5%) were in very good partial remission, 4 patients (12.9%) were in partial remission state and 4 patients (12.9%) had refractory disease. VAD + VCD protocol was given to 12 (38.7%) patients. 6 (19.8%) patients had VCD, 3 (9.7%) got VCD + VRD, 2 (6.5%) got VAD and 8 (25.8%) got other chemotherapy protocols. Transplantation was done in 26 patients (83.9%). Overall 9 patients (29%) died during the study period. Second or tandem transplantation was performed to 10 patients and 30% (n:3) of these re-transplanted patients died. The overall survival for these patients was estimated to be 70% (95% CI: 64.2-75.8). In conclusion MM in patients younger than 45 years is rare. In these patients Ig G Kappa and Kappa Light chain types are the most frequent types. Heterogeneous of individual patients may exist within these young myeloma patients. For those who are expected to show a poor response to current treatments despite their young age, the efficacy of an alternative therapeutic strategy such as front line allo-HSTC should be further investigated. Disclosures No relevant conflicts of interest to declare.

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.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.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.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.Amaç: Hematopoietik kök hücre alıcıları ve donörlerden bilgilendirilmiş onam alınması, nakil sürecinin en önemli basamağıdır. Görsel yöntemlerden yararlanılması anlamayı kolaylaştırabilir. Bu prospektif randomize çalışmada sözel ve yazılı bilgilendirmeye ilave olarak audiovizüel yöntem kullanılmasının standart yönteme göre etkinliğinin araştırılması amaçlanmıştır. Gereç ve Yöntemler: On dakikalık kısa bir bilgilendirme animasyonu hazırlatıldı. Toplam 82 katılımcı rastgele 2 gruba ayrıldı: Grup 1 (görsel bilgilendirme yönteminin ilave edildiği grup) ve grup 2 (standart yöntem uygulanan grup). Bilgilendirme işlemi sonunda katılımcılara 20 soruluk bir anket uygulandı. Ayrıca, yeni yöntemin sonuçlarının kişiler arası farklılıktan etkilenip etkilenmediği test edildi. Bulgular: Güvenirlik testi ve faktör analizi anketin güvenilir ve geçerli olduğunu gösterdi. Tüm olgular için genel memnuniyet ortalama değeri 200 üzerinden 184,8±19,8 olarak bulundu. Yazılı bilgilendirilmiş onam form memnuniyeti ortalama puanları yönünden grup 1’deki olguların memnuniyetleri grup 2’ye oranla anlamlı olarak daha yüksek olduğu saptandı (p=0,039). Sözel olarak bilgi veren doktor ve bilgilendirme animasyonu memnuniyeti bakımından gruplar arasında fark saptanmadı. Olguların memnuniyet seviyesi yaş, eğitim durumu ve bilgilendiren kişiler arası farklılıktan etkilenmedi. Sonuç: Bu çalışma, görsel yöntemlerin kullanılmasının işlem ve muhtemel risklerinin daha iyi anlaşılmasına katkı sağlayabileceğini göstermektedir. Anahtar Sözcükler: Hematopoietik kök hücre, Donör, Bilgilendirilmiş onam, Audovizuel yöntem, Kemik iliği nakli.

Osler-Weber-Rendu Syndrome (hereditary hemorrhagic telengiectasis) is a hereditary disease with autosomal dominant inheritance characterized by muco-cutaneous telengiectasis, arterio-venous malformations in internal organs. The disease is manifested by telengiectasis in oral mucosa, ear, nasal mucosa, fingertips and finger-beds and recurrent hemorrhage. Epistaxis is among the typical findings of the disease. Coexistence with arterio-venous malformations is common. It may lead to gastrointestinal hemorrhage and neurologic problems due to mucosal telengiectasis. Herein, we presented a case who had recurrent iron deficiency anemia and diagnosed with Osler-Weber-Rendu Syndrome as the result of radiologic and endoscopic examinations performed due to the presence of oral telengiectasis.

İntrahepatik kolestaz, belirgin düzeyde artmış bilüribin ve akut hepatik yetmezlik ile karakterize, orak hücreli aneminin nadir bir komplikasyonu olup sıklıkla ölümcül gidişlidir. Bu raporda, intrahepatik kolestazı olan 2 homozigot hemoglobin S, bir S-β talesemi hastası bildirilmektedir. Otomatize eritrosit değişimi ile başarılı bir şekilde periferik kanda hemoglobin S düzeyi %30’un altına indirilmesine rağmen, kolestazın temel değerlendirme testlerinden olan bilüribin düzeyi birinci hastada 50 mg/dL, ikincisinde 30 mg/dL ve üçüncü hastada 10 mg/dL’nin üzerinde saptandı. Plazma değişimi işlemi uygulanan hastalardan biri dışında diğerlerinde klinik durumlarında iyileşme gözlendi. Bu vakalardan elde edilen veriler intrahepatik kolestazı olan orak hücreli anemi hastalarında eritrosit değişim işlemine yeterli yanıt alınamadığında plazma değişiminin klinik iyileşmede rol oynayabileceğini göstermektedir., Intrahepatic cholestasis, a rare complication of sickle cell anemia, is characterized by marked hyperbilirubinemia, acute hepatic failure, and an often fatal course. In this report, we present patients with homozygous hemoglobin S and one patient with sickle-β thalassemia disease who have intrahepatic cholestasis. Despite automated red blood cell exchange transfusion, which successfully decreased the hemoglobin S level to less than 30% of total hemoglobin level in the peripheral blood, basic biochemical laboratory examination revealed signs of cholestasis with a serum bilirubin level of up to 50 mg/dL in the first case, 30 mg/dL in the second, and 10 mg/dL in the third. The patients underwent plasma exchange, which improved their clinical condition except one. These cases suggest that plasma exchange may have a role in improving the clinical condition of patients with sickle cell disease complicated with intrahepatic cholestasis (SCIC) that fails to respond to automated red blood cell exchange therapy

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.

PubMedID: 27068408 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-ß0 thalassemia (17 %), Hgb S-ß+ thalassemia (15 %), and Hb S-? 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. © 2016, Springer-Verlag Berlin Heidelberg.

The curative treatment approach for diffuse large B-cell lymphoma (DLBCL) is controversial even in the rituximab (R) era. The aim of this study was to examine the FcγRIIIA gene polymorphism distribution of DLBCL patients who had been treated with R-CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy. Furthermore, we investigated the impact of FcγRIIIA gene polymorphism on the overall response rate (ORR) and overall survival (OS).Patients from 3 centers in the Aegean region of Turkey who had newly diagnosed CD20-positive DLBCL were enrolled in the study. The single nucleotide polymorphisms of the FcγRIIIA gene were analyzed by real time-PCR. The response to treatment was determined in the middle and at the end of the protocol. During 2 years of follow-up, the patients were clinically and radiologically evaluated for disease status every 3 months.Thirty-six patients were included in the study and the distributions of F/F, V/F, and V/V types of alleles of FcγRIIIA were 25%, 50%, and 25%, respectively. Twenty-seven patients were considered as evaluable according to ORR and OS. The patients' ORR was 87.5%, 100%, and 50% in the F/F, V/F, and V/V allele groups, respectively. We did not establish any statistically significant differences among the 3 alleles groups in respect to ORR (p=0.93). The OS within 2 years in the F/F, V/F, and V/V allele groups was 62.5%, 100%, and 100%, respectively. The OS in the F/F allele group was found to be lower than in the other 2 allele groups (p=0.01).The distribution of gene polymorphisms in our study group was similar to those of previous studies. While ORR was similar between the groups, our results highlight a lower OS in F/F patients compared to other allele groups of FcγRIIIA.Amaç: Diffüz büyük B hücreli non-Hodgkin lenfomada (DLBCL) kür sağlayıcı tedavi yaklaşımı rituximab çağında olmamıza rağmen tartışmalı bir konudur. Bu çalışmanın amacı R-CHOP (siklofosfamid, doksorubisin, vinkristin ve prednizon) rejimi alan DLBCL hastalarında FcγRIIIA gen polimorfizminin dağılımını incelemekti. Ayrıca FcγRIIIA gen polimorfizminin tüm yanıt oranları (ORR) ve tüm yaşam (OS) üzerine olan etkisini araştırmaktı. Gereç ve Yöntemler: Türkiye’nin Ege Bölgesi’ndeki üç merkezden yeni tanı almış CD-20 pozitif DLBCL hastaları çalışmaya dahil edildi. FcγRIIIA’daki tek gen polimorfizmi gerçek zamanlı-PCR ile incelendi. Tedaviye yanıt, planlanmış olan protokolün ortasında ve sonunda değerlendirildi. İki yıllık takip süresince her üç ayda bir hastalığın hem klinik, hem de radyolojik durumu ele alındı. Bulgular: Çalışmaya dahil edilen 36 hastada, FcγRIIIA’nın F/F, V/F ve V/V alellerinin dağılımı sırasıyla %25, %50 ve %25’ti. ORR ve OS verilerine göre 27 hasta değerlendirilebilir olarak kabul edildi. Hastaların ORR değerleri F/F, V/F ve V/V alel gruplarına göre sırasıyla %87,5; %100 ve %50 olarak hesaplandı. Hastaların ORR değerleri açısından üç alel grubu arasında istatistiksel olarak anlamlı fark saptanmadı (p=0,93). F/F, V/F ve V/V gruplarında iki yıllık OS %62,5, %100 ve %100 bulundu. F/F alel grubunun OS’si diğer iki alel grubundakinden daha düşük bulunmuştur (p=0,01). Sonuç: Gen polimorfizmi dağılımı sonuçlarımız önceki çalışmalarda bulunanlarla benzerdir. Gruplar arasında ORR değerleri arasında fark yokken, sonuçlarımız F/F hastalarının FcγRIIIA’nın diğer allel gruplarına göre daha kısa bir OS değerine sahip olduğunu göstermektedir.

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 -alpha(3.7) (63.3 %), --(MED) (11.7 %), --(20.5) (10.7 %), alpha 2(IVS1(-5nt)) (3.9 %), and alpha 2(polyA-2) (3.5 %). The most frequent genotypes were -alpha(3.7)/alpha alpha (35.8 %), -alpha(3.7)/-alpha(3.7)(18.9 %), -(20.5)/alpha alpha (11.5 %), and --(MED)/alpha alpha (10.4 %), respectively. There were statistically significant differences in hematological findings between -alpha(3.7)/-alpha(3.7) and --(MED)/alpha alpha, 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 -alpha(3.7) single gene deletion is particularly prevalent at Adana province in agreement to other studies from Turkey.

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.