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Objective: This study was planned to evaluate long-term post-transplant complications in patients who underwent transplantation with the diagnosis of Fanconi anemia (FA) in childhood in our bone marrow transplantation unit and who were still being followed. It was predicted that the results would show the critical importance of determining disease-specific post-transplant long-term follow-up plans and putting them into practice in terms of early detection of complications and improving the survival rates and quality of life of FA patients. Materials and Methods: In this single-center, cross-sectional study, according to current recommendations, we analyzed the long-term outcomes of 36 patients with FA with a median age of 18.1 years (range: 6.1-36 years, male/female ratio: 24/12) who underwent HSCT in the Pediatric Bone Marrow Transplantation Unit between 1995 and 2019 and survived at least 1 year following the transplantation. Results: The median long-term follow-up time was 8 years (range: 1-25 years). Gonadal dysfunction was detected in approximately 35% of our patients; more specifically, 31% of the patients had hypergonadotropic hypogonadism and 4% had hypogonadotropic hypogonadism. When the patients were evaluated for growth impairment, 7 of 12 patients who had reached their final adult heights and 12 of 21 patients who had not yet completed their growth had height standard deviation (SD) scores below -2 SDs. Three patients (9%) developed subclinical hypothyroidism, 2 (6%) had overt hypothyroidism, and 1 (3%) had central hypothyroidism. Although none of our patients fully met the criteria for metabolic syndrome, 23% had insulin resistance and 39% had dyslipidemia. Evaluation of organ dysfunctions revealed that nearly 50% of the patients had obstructive and 21% had restrictive changes in their pulmonary function tests. Hepatosteatosis was detected in 15% of the patients and mild valve dysfunction was detected in 50% of evaluable patients. Three patients developed secondary malignancies. Squamous cell cancer developed in 2 patients and basal cell cancer in 1 patient. Conclusion: A risk-defined multidisciplinary approach for the long-term follow-up of children with FA undergoing HSCT is essential for early detection and management of late effects. [ABSTRACT FROM AUTHOR]

Amaç: BK virus (BKV) ile ilişkili hemorajik sistit (HS), hematopoetik kök hücre transplantasyonu (HKHT) yapılan hastalarda yaygın görülen bir komplikasyondur. Bu çalışmanın amacı, HKHT yapılan çocuk hastalarda BKV enfeksiyonu insidansının araştırılmasıdır. Gereç ve Yöntemler: Çalışmaya Ekim 2015 ile Eylül 2017 tarihleri arasında izlenen, yaşları 16 ay ile 16 yıl arasında olan toplam 51 hasta dahil edilmiştir. Hastalar BKV DNA'nın idrar ve kanda tespiti için kantitatif gerçek-zamanlı polimeraz zincir reaksiyonu (Anaolia Geneworks, Türkiye) testiyle monitörize edilmiştir. Bulgular: Hastaların 46'sına allojenik HKHT ve 5'ine otolog HKHT yapılmıştır. Allojenik nakil yapılan 46 hastanın 26'sında (%56,5) ve otolog nakil yapılan 5 hastanın 1'inde (%20,0) olmak üzere toplam 27 (%52,9) hastanın idrar ve/veya kanında BKV DNA pozitifliği saptanmıştır. Allojenik HKHT yapılan 46 hastanın 12 (%26,1)'sinde preemptif tedavi için gereken idrarda >107 kopya/ml BKV viral yük düzeyi tespit edilmiştir. Preemptif tedavi uygulanan 12 hastanın 9'unda (%75,0) HS gelişmesi önlenirken 3'ünde (%25,0) HS gelişmiş ve tedaviyle iyileşmiştir. HS gelişmeden önceki iki hafta içinde BKV virurisi >109 kopya/ml olarak tespit edilmiş ve HS prediktif tanısı için prognostik bir gösterge olarak kabul edilmiştir. BKV viremisi 1 hastada sistit gelişmesinden önceki iki hafta içinde >104 kopya/ml olarak tespit edilmiştir. Sonuç: Yüksek riskli hastalarda HS prediktif tanısı için BKV enfeksiyonu, özellikle HKHT hastalarında BKV virüri taraması önerilir.

Objective: Angiogenic factors (AFs) released under endothelial stress are reflective of tissue healing, while some may also contribute to tissue damage/inflammation. We investigated whether alterations in the pre-transplant levels of AFs were associated with the risk of acute graft-versus-host disease (aGvHD). Materials and Methods: The pre-conditioning plasma levels of angiopoietin-2 (Ang2), endoglin, and follistatin were measured for 37 patients together with inflammatory markers. The index values defined were evaluated to better identify the alterations. Results: The patients had higher pre-conditioning levels of Ang2, endoglin, and follistatin compared to controls. The patients with aGvHD had higher Ang2 index and lower albumin index scores in comparison to those without aGvHD. Multivariate analysis revealed that the pre-transplant Ang2 index was an independent risk factor for aGvHD development. Conclusion: Pre-transplant evaluation of plasma Ang2 levels along with inflammatory status even before conditioning is associated with endothelial vulnerability. The pre-transplant Ang2 index could be a promising candidate to estimate the risk of aGvHD. [ABSTRACT FROM AUTHOR]

Objective: A soluble form of suppression of tumorigenicity 2 (sST2) has emerged as a biomarker for acute graft-versus-host disease (GVHD) and non-relapse mortality (NRM). We prospectively monitored sST2 levels during the early phase of hematopoietic stem cell transplantation (HSCT) and evaluated the clinical association with transplant-related complications including acute GVHD. Materials and Methods: Thirty-two adult Japanese patients who received a first allogeneic HSCT were enrolled in this study. Levels of sST2 were measured at fixed time points (pre-conditioning, day 0, day 14, day 21, and day 28). Results: The median age was 50.5 years (range=16-66). With a median follow-up of 21.5 months (range=0.9-35.4), 9 patients developed grade II-IV acute GVHD. Median sST2 levels on the day of HSCT were higher than baseline and reached the maximum value (92.7 ng/mL; range=0-419.7) on day 21 after HSCT. The optimal cut-off value of sST2 on day 14 for predicting grade II-IV acute GVHD was determined as 100 ng/mL by ROC analysis. The cumulative incidence of acute GVHD was 56.7% and 16.5% in the high- and low-sST2 groups, respectively (p<0.01). Multivariate analyses showed that high sST2 levels at day 14 were associated with a higher incidence of acute GVHD (hazard ratio=9.35, 95% confidence interval=2.92-30.0, p<0.01). The cumulative incidence of NRM was increased in the high- sST2 group (33% vs 0%, p<0.01), but all the patients died of non-GVHD complications. Among 6 patients in the high-sST2 group without grade II-IV GVHD, 5 patients developed veno-occlusive disease (VOD) and one also had thrombotic microangiopathy (TMA). Conclusion: The early assessment of sST2 after HSCT yielded predictive values for the onset of acute GVHD and other transplant-related complications including VOD and TMA. [ABSTRACT FROM AUTHOR]

Objective: Hematopoietic stem cell transplantation (HSCT) is a choice of treatment for malignant and non-malignant diseases. After HSCT, some complications may develop in patients. Cardiac complications are particularly important. The aim of this study was to investigate whether systolic pulmonary artery pressure (PAP) is a marker for overall survival (OS) in HSCT patients. Materials and Methods: In our study, 428 HSCT patients were evaluated. Ejection fraction (EF) and PAP values were investigated during symptom-oriented echocardiography in the pre-HSCT and post-HSCT periods. Results: Pre-HSCT EF values were similar between the groups. In patients with autologous HSCT (auto-HSCT) (PAP >25 mmHg), it was found that the 5-year mortality rate was 48.6%, while in the other group (PAP <25 mmHg) the 5-year mortality was 25.5%. There was a significant association between 5-year mortality rate and PAP level (p<0.046) in the auto-HSCT group. OS was 38% in the pre-auto- HSCT period with PAP values of >25 mmHg, while OS was 61% in the pre-auto-HSCT period with PAP values of <25 mmHg (p<0.001). We determined that there was a statistically significant difference between OS and PAP levels in patients with auto-HSCT. Five-year mortality rate and OS were not significantly different in patients undergoing allogeneic HSCT (allo-HSCT) (p>0.05). Conclusion: Our results suggest that pre-HSCT PAP value is an important risk factor for mortality and OS in patients undergoing auto-HSCT. [ABSTRACT FROM AUTHOR]

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Objective: Haematopoietic stem cell recipients need central venous catheters (CVCs) for easy administration of intravenous fluid, medications, apheresis, or dialysis procedures. However, CVCs may lead to infectious or non-infectious complications such as thrombosis. The effect of these complications on transplantation outcome is not clear. This manuscript presents the complication rates of double-lumen tunnelled CVCs and their effect on transplantation outcome. Materials and Methods: Data from 111 consecutive patients, of whom 75 received autologous and 36 received allogeneic peripheral blood stem cell transplantations, were collected retrospectively. The data were validated by the Record Inspection Group of the related JACIE-accredited transplantation centre. Results: Thrombosis developed in 2.7% of recipients (0.9 per 1000 catheter days). Catheter-related infection was identified in 14 (12.6%) patients (3.6 per 1000 catheter days). Coagulase-negative Staphylococcus was the most common causative agent. Engraftment time, rate of 100-day mortality, and development of grade II-IV graft-versus-host disease were not found to be associated with catheter-related complications. Conclusion: These results indicate that adverse events related with tunnelled CVCs are manageable and have no negative effects on transplant outcome. [ABSTRACT FROM AUTHOR]

Hematopoietic Stem Cell Transplantation (HSCT) is a method used for the treatment of patients with severe aplastic anemia, leukaemia and some other malignancies. As it causes many adverse sequelaes (anorexia, nausea, vomiting, diarrhea, and mucositis), adequate oral diet is usually unachievable and artificial nutrition support is required. In order to reduce the risk of infections, the protective benefit of "low-microbial / neutropenic diets" is suggested to use in immunosuppressed patients. Traditionally, total parenteral nutrition (TPN) has been the chosen method for the nutritional support of patients undergoing HSCT. Secondly enteral nutrition (especially with enteral tube feeding - ETF) is an alternative method for nutritional support. It is important that dietary guidance for HSCT patients to minimize the risk of microbial exposure, optimize nutrient consumption and energy intake is very important. [ABSTRACT FROM AUTHOR]

Hematopoietic stem cell transplantation (HSCT) is used for various hematologic malignancies seen in children and adults. There may be several complications before, during, and after the HSCT. Just one of them is endocrinologic complications, since endocrine system (particularly the pituitary gland, thyroid gland, adrenal glands, and gonads) is highly sensitive against various stress. Chemotherapy and/or total body irradiation used as preparative regimens and immunosuppressive agents (especially corticosteroids) used for the graft-versus-host disease can cause hormonal disorders. Time elapsed after the HSCT, transplantation type (autologous or allogeneic), preparative regimen choice, age, and gender determines the complications. A multidisciplinary management containing a specialist of endocrinology for these patients is preferred. In this report, we reviewed the endocrinologic complications that observed after the HSCT in children and adults referring to the recent literatures. J Clin Exp Invest 2012; 3(1): 149-156 [ABSTRACT FROM AUTHOR]