Background: Hereditary Hemochromatosis (HH) is an inherited iron metabolism disorder characterized by excessive iron accumulation in the liver, heart, pancreas and other endocrine organs. Although it is often associated with mutations in the HFE gene, it can also be caused by mutations associated with HJV, TFR-2 and ferroportin. JAK2 V617F (+) Polycythemia Vera (PV) is the most common cause of acquired primary erythrocytosis and has been reported very rarely in patients with HH. Aims: We presented two polycythemia vera patients with a diagnosis of concomitant hereditary hemochromatosis. Methods: Presence of HFE gene mutation and jak-2 V617F mutation was determined by PCR test Results: Case 1: N.G, a 67-year-old male patient with a history of hypertension, insulin-dependent diabetes mellitus and cerebrovascular accident was diagnosed with PV in another center in 2007. The patient was JAK2 V617F (+) and the tests performed in our laboratory after phlebotomies were EPO: 7.8 mIU/ml, ferritin: 34 ng/dl, transferrin saturation: 49%. Bone marrow aspiration biopsy revealed hypercellularity with erythroid and megakaryocytic hyperplasia. Although Hb/Hct levels were within the targeted range with hydroxyurea 500 mg 2x1 treatment, he was followed up with frequent phlebotomies due to persistent pruritus. After phlebotomy, there was a significant improvement in his pruritus. He was using clopidogrel as antiaggregant treatment. During follow-up, there was deterioration in blood glucose regulation and an increase in insulin requirement. In June 2018, he developed widespread hyperpigmentation on his skin. Due to unregulated diabetes, hyperpigmentation, and high ferritin levels despite frequent phlebotomy, he was examined for HH. He was found to be heterozygous positive for the HFE gene H63D mutation. The patient is still being followed up with HU 2X1 and phlebotomy without any problem. Case 2: E.H, a 57-year-old male patient with a history of hypertension and type 2 diabetes mellitus was diagnosed with PV in 2015. He was JAK2 V617F(+) and at diagnosis EPO:3.7 mIU/ml, ferritin:1403 ng/ml, transferrin saturation:63%. Bone marrow aspiration biopsy revealed hypercellularity and erythroid hyperplasia. Since he was a low-risk patient under the age of 60 and had no history of thrombosis, he was followed up with ASA 100 mg/day and intermittent phlebotomies. Similar to the first case, persistent pruritus was present despite the normal Hb/Hct level. Since hyperferritinemia was not expected in polycythemia vera and the patient was diabetic, he was examined for HH. In the HFE gene analysis, the H63D mutation was found to be heterozygous. Due to the frequent need for phlebotomy, the patient is considered to start treatment with hydroxyurea, and the follow-up still continues. Summary/Conclusion: The association of HH and PV has been rarely reported in the literature, and its clinical significance is not clearly known. In the presence of unexplained high ferritin levels in PV, even if the clinical findings are unclear, screening for HH may be recommended as an appropriate approach. The role of HFE genotypes in the development of myeloproliferative diseases is unknown. Clinical data accumulation is needed to elucidate iron metabolism and prognostic features in PV patients with HFE mutations. Keywords: polycythemia vera, hereditary hemochromatosis, JAK2 V617F, HFE mutation
The present study investigated the efficacy and safety profile of daratumumab monotherapy in 42 patients with relapsed refractory multiple myeloma through a Turkish early access program. The current findings have confirmed the efficacy of daratumumab monotherapy in heavily pretreated patients with refractory multiple myeloma because of the deep and durable responses and favorable safety and tolerability profile. Background: In countries where frontline drug approval is limited to first-generation proteasome inhibitors or immunomodulatory drugs, relapses have been both more frequent and less durable. We investigated real world data on the efficacy and safety of daratumumab monotherapy among patients with relapsed refractory multiple myeloma (RRMM) from Turkey using a prospective early access program. Patients and Methods: A total of 42 patients with RRMM after a minimum of 3 previous lines of proteasome inhibitor/immunomodulatory drug-based treatments were included from 25 centers across Turkey. Daratumumab monotherapy was administered intravenously at a dose of 16 mg/kg weekly (cycles 1-2), on alternate weeks (cycles 3-6), and monthly thereafter. Results: The median daratumumab monotherapy duration was 5.5 months (range, 0.2-28.7 months). The overall response rate was 45.2%, including 14 (33.3%) partial responses, 4 (9.5%) very good partial responses, and 1 (2.4%) complete response. The median duration of response was 4.9 months. The median progression-free survival (PFS) was 5.5 (95% confidence interval, 2.6-8.4 months) with 12- and 18-month PFS rates of 35.7% and 31.0%, respectively. The median overall survival was not reached; the 12- and 18-month overall survival rates were 64.3% and 59.5%, respectively. The depth of response had a significant effect on PFS (log-rank test, P = .026). Overall, of the 76 adverse events reported, 33 (43.4%) were grade >= 3; only 4 (9.52%) were grade 3 infusion-related reactions. No infusion-related reactions or adverse events led to treatment discontinuation. Conclusion: The present findings from our daratumumab early access program have confirmed the efficacy and safety profile of daratumumab monotherapy in heavily pretreated Turkish patients with RRMM.
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.
The US Food and Drug Administration has warned that treatment with dipeptidyl peptidase (DPP)-4 inhibitors may promote serious arthralgia. However, the clinical evidence for this is relatively lacking.For this reason, a systematic review and meta-analysis of randomized controlled trials (RCTs) were carried out to determine the relationship between DPP-4 inhibitors and risk of arthralgia, and also to investigate any potential risk factors.An extensive electronic search for RCTs comparing DPP-4 inhibitors with any comparators was performed up to July 2016. Outcomes of interest were overall and serious arthralgia. Summary risk ratios (RRs) with 95% confidence intervals (CIs) were calculated.A total of 67 RCTs (involving 79,110 patients) was ultimately included. Pooled results showed that DPP-4 inhibitors were associated with a slightly but significantly increased risk of overall arthralgia (RR: 1.13, 95% CI: 1.04–1.22; P = 0.003) and a non-significant increased risk of serious arthralgia (RR: 1.44, 95% CI: 0.83–2.51; P = 0.20). Also, subgroup analyses showed that add-on/combination therapy and longer diabetes duration (> 5 years) were possible factors associated with the increased risk of overall arthralgia.These findings suggest that DPP-4 inhibitors can increase the risk of arthralgia. Thus, the benefits of glycaemic control must be weighed against the risk of arthralgia when prescribing DPP-4 inhibitors. Further studies are now needed to identify and confirm these risk factors.
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.
Aim: Anemia is a public health problem worldwide. Cost effectiveness and efficient use of resources are vitally important. Red blood cell distribution width, which can be obtained from a standard complete blood count, is a measure of the variability in size of circulating erythrocytes. The present study was performed to investigate whether red blood cell distribution width can be used to predict response to iron therapy. Methods: This study was conducted in 50 patients admitted to hematology and family medicine clinics. Complete blood count and reticulocyte count were determined on day 5; complete blood count was examined 1 month after commencement of therapy. Results: Statistically significant differences were detected between hemoglobin levels and red blood cell distribution width values at the time of diagnosis and on day 5 and after 1 month of therapy. A significant positive correlation was found between the increase in red blood cell distribution width and the increase in hemoglobin. Conclusion: Red blood cell distribution width may be used in place of reticulocyte count to predict response to iron therapy. Red blood cell distribution width is the best biomarker for this purpose as a component of complete blood count, and therefore it may be accepted as superior to reticulocyte count.
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.
Amaç: Bu çalışmanın amacı demir tedavisi alan hastalarda leptin ve kilo artışı arasındaki ilişkiyi saptamaktır.Gereç ve Yöntem: Toplamda 42 demir eksikliği anemisi tanısı konulan kadın hasta çalışmaya dahil edilmiştir. Tanı anında ve tedavi başlandıktan 1 ay sonra tam kan sayımı, serum demir, ansature demir bağlama kapasitesi ve ferritin seviyesi, tanı anında ve tedavi başlandıktan sonra 96. saatte serum hepsidin ve leptin seviyesi ölçüldü. İştahta meydana gelen değişiklikler ve vücut ağırlığı tanı anında ve tedavi başlandıktan 1 ay sonra değerlendirildi.Bulgular: Tanı anındaki ve tedavi başlandıktan sonraki serum hepsidin ve leptin seviyeleri arasında istatistiksel olarak anlamlı fark bulunmadı. 22 (%52.4) hastada iştah artışı olduğu tespit edildi. Tanı anında hastaların ortalama ağırlığı 69.6±17.9 kg olarak tespit edilmiş iken tedavi başlandıktan 1 ay sonra hastaların ağırlığı 70.7±17.5 kg olarak bulunmuştur ve değerler arasında istatistiksel olarak anlamlı fark tespit edilmiştir.Sonuç: Çalışmamız gözlemlerimiz ve hipotezimizle uyumlu olarak demir tedavisini takiben hastalarda iştah artışı ve ağırlık artışı meydana geldiği saptanmıştır. Leptin düzeyleri ile kilo artışı arasında bir ilişki bulunmamıştır. .
•Polymorphism of eNOS G894T is a risk factor for diabetes.•Polymorphism of VEGF C936T is a risk factor for diabetes.•T allele is not an independent risk factor for diabetic foot.
Granulocytic sarcoma (GS) is a rare extramedullary solid tumor composed of immature myeloid cell that is associated with acute myeloid leukemia or myelodysplastic syndrome. Especially, the involvement of the breast as a pattern of relapse after allogeneic bone marrow transplantation ( allo SCT) is extremely rare. We report the case of a 57-year-old woman who palpated a painless lump in the superior quadrant of her right breast. Her past medical history was a myelodysplastic syndrome ( MDS) treated with bone marrow transplantation. Imaging work-up by mammogram and ultrasonography ( US ) showed solitary mass suspect of malignancy in breast. Tru-cut biopsy was performed from the suspect palpable mass of the right breast. Histologic findings were compatible with a granulocytic sarcoma in breast. While she was also evaluated for mass in the breast, also investigated by endoscopy for the symptoms of gastrointestinal system. The endoscopic biopsy showed diffuse neoplastic infiltration similar the pathology of the breast. GS of the breast is a rare extramedullary involvement of hematologic diseases. There are no the specific radyologic features in the presentation with GS of the breast. Our case also showed that careful histopathological review along with all panel of immunohistochemistry is extremely important for diagnose.
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.
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. No relevant conflicts of interest to declare.
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).
Objectives: 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. Materials and Methods: 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 high-resolution testing. Results: 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. Conclusions: 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.
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.
Purpose: The aim of this study was to evaluate the relationship between leptin and weight gain in patients receiving iron treatment. Materials and Methods: A total of 42 female patients who were diagnosed with IDA were included in the study. Whole blood count, serum iron, serum unsaturated iron binding capacity (UIBC), serum ferritin level were measured at the time of diagnosis and on month one after treatment; serum hepcidin level and serum leptin level were measured at the time of diagnosis and 96 hours after commencement of parenteral therapy; body weight and appetite alterations were recorded at the time of diagnosis and on month one. Results: A statistically significant difference was not found between hepcidin and leptin values at the time of diagnosis and after treatment. Increased appetite was detected in 22 (52.4%) patients on month one after treatment. While mean weight of all patients was 69.6 +/- 17.9 kg at the time of diagnosis, it was 70.7 +/- 17.5 kg on month one after treatment and there was a statistically significant difference between two values. Conclusion: Our study revealed that appetite and body weight increased following iron therapy, consistently with our observations and hypothesis. However no significant relationship was found with leptin levels and weight increase.
New-onset diabetes mellitus after solid-organ transplant makes for complicated tacrolimus immunosuppression. However, tacrolimus-associated diabetic ketoacidosis has not been reported in bone marrow transplant. We report 24-year-old women, hospitalized with diabetic ketoacidosis, 70 days after undergoing a bone marrow transplant with tacrolimus immunosuppression. Clinicians should be wary about tacrolimus levels and the risk of hyperglycemic states after bone marrow transplant as with other solid-organ transplants.