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1.
J Clin Immunol ; 44(7): 157, 2024 Jul 02.
Article in English | MEDLINE | ID: mdl-38954121

ABSTRACT

Molecular diagnosis of inborn errors of immunity (IEI) plays a critical role in determining patients' long-term prognosis, treatment options, and genetic counseling. Over the past decade, the broader utilization of next-generation sequencing (NGS) techniques in both research and clinical settings has facilitated the evaluation of a significant proportion of patients for gene variants associated with IEI. In addition to its role in diagnosing known gene defects, the application of high-throughput techniques such as targeted, exome, and genome sequencing has led to the identification of novel disease-causing genes. However, the results obtained from these different methods can vary depending on disease phenotypes or patient characteristics. In this study, we conducted whole-exome sequencing (WES) in a sizable cohort of IEI patients, consisting of 303 individuals from 21 different clinical immunology centers in Türkiye. Our analysis resulted in likely genetic diagnoses for 41.1% of the patients (122 out of 297), revealing 52 novel variants and uncovering potential new IEI genes in six patients. The significance of understanding outcomes across various IEI cohorts cannot be overstated, and we believe that our findings will make a valuable contribution to the existing literature and foster collaborative research between clinicians and basic science researchers.


Subject(s)
Exome Sequencing , High-Throughput Nucleotide Sequencing , Humans , Male , Female , Immunologic Deficiency Syndromes/genetics , Immunologic Deficiency Syndromes/diagnosis , Immunologic Deficiency Syndromes/immunology , Genetic Predisposition to Disease , Child , Child, Preschool , Mutation/genetics , Genetic Testing/methods , Infant , Exome/genetics , Adolescent
3.
Am J Blood Res ; 11(4): 427-437, 2021.
Article in English | MEDLINE | ID: mdl-34540352

ABSTRACT

Coronavirus disease 2019 (COVID-19) has led to a global pandemic that has also challenged the management of various other life-threatening conditions, such as malignant disorders. In this study, we present the clinical features and treatment outcomes of twenty-seven COVID-19 positive patients with leukemia across seven different centers in Istanbul. From March 1st to December 31st 2020, 116 patients were diagnosed with acute leukemia. Thirty-two cases with acute lymphocytic leukemia (ALL), 82 cases with acute myeloid leukemia (AML), and 2 cases with mixed phenotype acute leukemia (MPAL) were identified. Of the 27 patients with the COVID-19 infection, seven patients had ALL, 19 patients had AML and one patient had MPAL. The mortality rate was 37% among the patients with AML, whereas there were no deaths in the ALL group. The mortality rate of AML patients with the COVID-19 infection was higher compared to cases without the infection (P<0.05). We could not detect any significant difference in the ALL cohort. This study, which includes one of the largest acute leukemia series in literature proved that acute myeloid leukemia patients with the COVID-19 infection have worse outcomes than patients without the infection. The high mortality among patients with acute leukemias hospitalized with COVID-19 highlight the need for aggressive infection prevention, increased surveillance and protective isolation and even modification of the therapy, in case of minimal residual disease (MRD) negativity.

4.
J Cancer Res Ther ; 17(2): 565-573, 2021.
Article in English | MEDLINE | ID: mdl-34121709

ABSTRACT

INTRODUCTION: Complications in patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT) cause serious morbidity and mortality. Predicting patients at risk in advance and changing the symptomatic care and/or preparation regimen according to this risk assessment have been emphasized recently. Several single-nucleotide polymorphisms have been studied, and some were found to be responsible for early complications. Glutathione S-transferase P1 (GSTP1) is an enzyme involved in the detoxification process that reduces oxidative stress by reducing the number of free oxygen radicals. AIM: This study aimed to investigate the relationship between GSTP1 polymorphism and early complications of allo-HSCT, iron parameters, overall survival (OS), and transplantation-related mortality (TRM). MATERIALS AND METHODS: A total of 50 patients diagnosed with acute myeloid leukemia (n = 23) or acute lymphoblastic leukemia (n = 27) who underwent allo-HSCT between May 2008 and February 2011 at Gazi University Faculty of Medicine, Stem Cell Transplantation Unit, were included. RESULTS: Of the 50 patients, 24 (48%) were women and 26 (52%) were men. The median age of the patients was 26 (16-74) years. GSTP1 polymorphism was detected in 23 (46%) patients, and 27 (54%) had no polymorphism (wild type). The two groups were compared in terms of early toxicity after transplantation, according to the preparation regimen. The group with GSTP1 polymorphism was found to have a high transferrin saturation index (P < 0.05). Patients with no GSTP1 polymorphism showed a high grade III-IV anemia ratio (P < 0.05). The presence of sinusoidal obstruction syndrome and graft-versus-host disease was similar in both groups (P > 0.05). OS and TRM were higher in the GSTP1 polymorphism group, but no statistical difference was found between the two groups (P > 0.05). CONCLUSIONS: TSI was higher in the GSTP1 polymorphism group. GSTP1 polymorphism had no effect on early transplantation complications. Although the OS and TRM ratios were higher in the GSTP1 polymorphism group, no statistically significant difference was found between the groups. Further studies with larger sample size are needed.


Subject(s)
Anemia/genetics , Glutathione S-Transferase pi/genetics , Graft vs Host Disease/genetics , Hematopoietic Stem Cell Transplantation/adverse effects , Hepatic Veno-Occlusive Disease/genetics , Adolescent , Adult , Aged , Anemia/epidemiology , Anemia/prevention & control , Female , Genetic Predisposition to Disease , Graft vs Host Disease/epidemiology , Graft vs Host Disease/prevention & control , Hepatic Veno-Occlusive Disease/epidemiology , Hepatic Veno-Occlusive Disease/prevention & control , Humans , Leukemia, Myeloid, Acute/genetics , Leukemia, Myeloid, Acute/therapy , Male , Middle Aged , Polymorphism, Single Nucleotide , Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Prospective Studies , Time Factors , Transplantation Conditioning , Transplantation, Homologous/adverse effects , Young Adult
5.
Turk J Haematol ; 38(3): 195-203, 2021 08 25.
Article in English | MEDLINE | ID: mdl-34057336

ABSTRACT

Objective: Although inhibition of the complement system at different steps is a promising therapy modality in patients with paroxysmal nocturnal hemoglobinuria (PNH), allogeneic hematopoietic stem cell transplantation (HCT) is still the only curative therapy, especially for patients with intractable hemolysis or bone marrow failure. The aim of this study is to evaluate the outcomes of allogeneic HCT in PNH patients with aplastic anemia (PNH-AA) or without. Materials and Methods: Thirty-five PNH/PNH-AA patients who were treated with allogeneic HCT in 10 transplantation centers in Turkey were retrospectively analyzed. Results: Sixteen (45.7%) and 19 (54.3%) patients were diagnosed with classical PNH and PNH-AA, respectively. The median age of the patients was 32 (18-51) years. The 2-year overall survival (OS) rate and rate of graft-versus-host disease-free, failure-free survival (GFFS) was 81.2% and 78.1%, respectively. The 2-year OS in cases of classical PNH and PNH-AA was 81.3% and 79.9%, respectively (p=0.87), and 2-year GFFS in cases of PNH and PNH-AA was 79% and 76% (p=0.977), without statistical significance. The OS and GFFS rates also did not differ between transplantations with matched sibling donors (MSDs) and matched unrelated donors (MUDs). Conclusion: Allogeneic HCT with MSDs or MUDs is a good option for selected patients with classical PNH and PNH-AA. In particular, patients with debilitating and refractory hemolysis and patients with bone marrow failure might form an excellent group of candidates for allogeneic HCT.


Subject(s)
Anemia, Aplastic , Hematopoietic Stem Cell Transplantation , Hemoglobinuria, Paroxysmal , Adult , Anemia, Aplastic/therapy , Hemoglobinuria, Paroxysmal/complications , Hemoglobinuria, Paroxysmal/therapy , Hemolysis , Humans , Middle Aged , Retrospective Studies , Turkey/epidemiology
6.
Physiother Theory Pract ; 37(1): 52-63, 2021 Jan.
Article in English | MEDLINE | ID: mdl-30958713

ABSTRACT

Background: A limited number of studies have reported impairments in physical activity, exercise capacity and quality of life (QOL) in allogeneic hematopoietic stem cell transplantation (allogeneic-HSCT) recipients. We aimed to compare dyspnea, exercise capacity, physical activity and QOL in allogeneic-HSCT recipients with age-gender matched healthy individuals, since this has not been investigated hitherto. Methods: A total of 80 allogeneic-HSCT recipients (>100 days status post-transplantation) (38.88 ± 13.25 years) and 60 healthy individuals (35.92 ± 10.83 years) were compared. Exercise capacity [6-minute walk test (6-MWT)], physical activity level (total and active energy expenditure, moderate and severe physical activity duration, number of steps, average metabolic equivalent, lying down and sleeping duration) [metabolic holter], QOL [European Organization for Research and Treatment of Cancer QOL Questionnaire (EORTCQOL)], dyspnea [Modified Medical Research Council Dyspnea scale] and pulmonary functions [spirometry] were evaluated. Clinical trials #NCT03606005. Results: Six-MWT distance, energy expenditure, physical activity duration, number of steps, average metabolic equivalent, global health status, functional and social function subscales of EORTCQOL were significantly lower in recipients compared with controls; dyspnea score, lying down, sleep durations, symptom and fatigue subscales of EORTCQOL were significantly higher in recipients compared with controls (p < 0.05). Conclusion: Dyspnea during daily living activities, exercise capacity, physical activity level and QOL are considerably impaired in allogeneic-HSCT recipients during post-engraftment period. To improve impaired outcomes, allogeneic-HSCT recipients should be oriented to cardiopulmonary rehabilitation programs.


Subject(s)
Dyspnea/physiopathology , Exercise Tolerance/physiology , Exercise/physiology , Hematopoietic Stem Cell Transplantation , Quality of Life , Survivors , Transplant Recipients , Adult , Cross-Sectional Studies , Female , Humans , Male , Middle Aged , Spirometry , Surveys and Questionnaires , Walk Test
7.
Rheumatol Int ; 41(1): 7-18, 2021 01.
Article in English | MEDLINE | ID: mdl-32588191

ABSTRACT

Hemophagocytic syndrome (HPS) or hemophagocytic lymphohistiocytosis (HLH) is an acute and rapidly progressive systemic inflammatory disorder characterized by cytopenia, excessive cytokine production, and hyperferritinemia. Common clinical manifestations of HLH are acute unremitting fever, lymphadenopathy, hepatosplenomegaly, and multiorgan failure. Due to a massive cytokine release, this clinical condition is considered as a cytokine storm syndrome. HPS has primary and acquired (secondary, reactive) forms. Its primary form is mostly seen in childhood and caused by various mutations with genetic inheritance and, therefore, is called familial HLH. Secondary HLH may be caused in the presence of an underlying disorder, that is, secondary to a malignant, infectious, or autoimmune/autoinflammatory stimulus. This paper aims to review the pathogenesis and the clinical picture of HLH, and its severe complication, the cytokine storm, with a special emphasis on the developed classification criteria sets for rheumatologists, since COVID-19 infection has clinical symptoms resembling those of the common rheumatologic conditions and possibly triggers HLH. MED-LINE/Pubmed was searched from inception to April 2020, and the following terms were used for data searching: "hemophagocytic syndrome" OR "macrophage activation syndrome" OR "hemophagocytic lymphohistiocytosis", OR "cytokine storm". Finally, AND "COVID-19" was included in this algorithm. The selection is restricted to the past 5 years and limited numbers of earlier key references were manually selected. Only full-text manuscripts, published in an English language peer-reviewed journal were included. Manuscript selection procedure and numbers are given in Fig. 2. Briefly, the database search with the following terms of "Hemophagocytic syndrome" OR "Macrophage activation syndrome" OR "Hemophagocytic lymphohistiocytosis" OR "Cytokine storm" yielded 6744 results from inception to April 2020. The selection is restricted to the past 5 years and only limited numbers of earlier key references were selected, and this algorithm resulted in 3080 manuscripts. The addition of (AND "COVID-19") resulted in 115 publications of which 47 studies, together with four sections of an online book were used in the final review. No statistical method was used. HLH is triggered by genetic conditions, infections, malignancies, autoimmune-autoinflammatory diseases, and some drugs. In COVID-19 patients, secondary HLH and cytokine storm may be responsible for unexplained progressive fever, cytopenia, ARDS, neurological and renal impairment. Differentiation between the primary and secondary forms of HLH is utterly important, since primary form of HLH requires complicated treatments such as hematopoietic stem cell transplantation. Further studies addressing the performance of HScore and other recommendations in the classification of these patients is necessary.


Subject(s)
Cytokine Release Syndrome/diagnosis , Lymphohistiocytosis, Hemophagocytic/diagnosis , Macrophage Activation Syndrome/diagnosis , COVID-19/classification , COVID-19/diagnosis , Cytokine Release Syndrome/etiology , Cytokine Release Syndrome/physiopathology , Diagnosis, Differential , Humans , Lymphohistiocytosis, Hemophagocytic/complications , Lymphohistiocytosis, Hemophagocytic/physiopathology , Macrophage Activation Syndrome/physiopathology , Pandemics , Rheumatology/methods , SARS-CoV-2
8.
Exp Clin Transplant ; 18(1): 98-105, 2020 02.
Article in English | MEDLINE | ID: mdl-28411358

ABSTRACT

OBJECTIVES: Acute kidney injury is a relatively frequent complication of allogenic hematopoietic stem cell transplant, resulting in increased risk of morbidity and mortality. Early diagnosis and management of acute kidney injury is of great importance for prevention of poor outcomes in these transplant recipients. MATERIALS AND METHODS: Fifty consecutive patients, hospitalized for allogenic hematopoietic stem cell transplant at the Bone Marrow Transplantation Unit of Gazi University Faculty of Medicine, were included in this prospective study. Serial measurements of serum creatinine and creatinine clearance were obtained before administration of conditioning regimen and at 0, 7, 14, 21, and 28 days after start of conditioning. Blood and urine samples were also obtained for the measurement of serum cystatin C and urine neutrophil gelatinase-associated lipocalin levels before conditioning and 24 hours before each serum creatinine measurement. RESULTS: During the median 25 days of follow-up, acute kidney injury developed in 19 patients: 10 patients had stage 1, 7 had stage 2, and 2 had stage 3 acute kidney injury according to the Acute Kidney Injury Network classification. There were significant positive correlations between serum cystatin C levels and serum creatinine levels and negative correlations with creatinine clearance levels at each time point (P < .001), whereas no statistically significant associations were observed with urinary neutrophil gelatinase-associated lipocalin levels. Both univariate and multivariate Cox regression models showed a statistically significant association between serum cystatin C levels and development of acute kidney injury, whereas urine neutrophil gelatinase-associated lipocalin levels did not show any significant associations. CONCLUSIONS: Serum cystatin C levels might be a useful marker for early detection of acute kidney injury in adult allogenic hematopoietic stem cell transplant recipients. Close monitoring of kidney function by sensitive biomarkers might provide early recognition and timely management of acute kidney injury in high-risk patient populations.


Subject(s)
Acute Kidney Injury/diagnosis , Cystatin C/blood , Hematopoietic Stem Cell Transplantation/adverse effects , Acute Kidney Injury/blood , Acute Kidney Injury/urine , Adolescent , Adult , Biomarkers/blood , Biomarkers/urine , Creatinine/blood , Early Diagnosis , Female , Humans , Immunosuppressive Agents/therapeutic use , Lipocalin-2/urine , Male , Middle Aged , Predictive Value of Tests , Prospective Studies , Risk Assessment , Risk Factors , Time Factors , Transplantation Conditioning , Transplantation, Homologous/adverse effects , Treatment Outcome , Turkey , Young Adult
9.
Transpl Infect Dis ; 20(4): e12912, 2018 Aug.
Article in English | MEDLINE | ID: mdl-29679523

ABSTRACT

BACKGROUND: Immune-compromised patients with latent TB infection (LTBI) are at risk for TB reactivation and should receive prophylaxis. Whereas the tuberculin skin test (TST) has limitations particularly in immune-compromised patients. AIMS: This retrospective study was conducted to determine the incidence of TB infection in adult HSCT recipients whose preventive therapy for LTBI was determined according to the guidance of targeted TST. PATIENTS AND METHODS: Five hundred and fifty-eight consecutive HSCT recipients (287 autologous and 271 allogeneic) who survived ≥100 days post-transplantation were included in this analysis. RESULTS: Tuberculin skin test results were available in 493 of 558 transplants (88.3%). The incidence of negative TST was 54.5% (269 of 493 patients). One multiple myeloma patient with a history of TB and negative TST result and was not on INH prophylaxis developed reactivation of TB infection. None of the recipients under INH prophylaxis (151 of 558 transplants; 27.1%) and none of the 224 patients with TST ≥5 mm developed TB infection. DISCUSSION: Despite the limitations of being a retrospective analysis and variable prophylaxis thresholds of TST, there are some remarkable results of this analysis. We had no TB infection in the allogeneic HSCT recipients. The high incidence of negative TST results may be attributed to the underlying immune-deficiency. TST may not be a reliable guide for predicting TB reactivation risk in hematology patients. CONCLUSION: Tuberculin skin test may have a high rate of false-negative and false-positive results in HSCT recipients. The general guidelines for targeted TST to guide treatment of LTBI may not apply to all regions and situations. More reliable methods are required to predict and treat LTBI in these specific conditions.


Subject(s)
Antibiotic Prophylaxis , Hematopoietic Stem Cell Transplantation/adverse effects , Immunocompromised Host , Transplantation Conditioning/adverse effects , Tuberculosis/epidemiology , Adolescent , Adult , Aged , Antibiotics, Antitubercular/therapeutic use , Female , Graft Rejection/prevention & control , Hematologic Neoplasms/surgery , Humans , Incidence , Male , Middle Aged , Retrospective Studies , Tuberculin Test , Tuberculosis/diagnosis , Tuberculosis/immunology , Tuberculosis/prevention & control , Turkey/epidemiology , Young Adult
10.
J Oncol Pharm Pract ; 24(4): 281-289, 2018 Jun.
Article in English | MEDLINE | ID: mdl-29284356

ABSTRACT

Background Autologous hematopoietic stem cell transplantation (AHSCT) remains the standard of care for younger patients with multiple myeloma (MM). Currently, high-dose melphalan (HDM) is recommended as conditioning regimen before AHSCT. Preclinical data suggest that combining bortezomib and melphalan has synergistic effect against multiple myeloma cells. Bortezomib and HDM (Bor-HDM) combination as conditioning regimen has been investigated by many other investigators. Objective In this retrospective study, we aimed to compare transplant-related toxicities and hematologic recovery of HDM and Bor-HDM conditioning regimens. Method We retrospectively evaluated hematologic recovery and toxicity profile in patients with MM who received AHSCT with either HDM ( n = 114) or Bor-HDM ( n = 53) conditioning regimen. Results Nonhematologic toxicities were comparable between HDM and Bor-HDM conditioning regimen, except mucositis and diarrhea being more frequent in the Bor-HDM group. Neutrophil and platelet engraftment time and duration of hospital stay were significantly shorter for HDM regimen. Conclusions In this retrospective analysis, we observed engraftment kinetics and duration of hospitalization were significantly worse in Bor-HDM conditioning regimen with manageable toxicities. Randomized studies are needed to further compare Bor- HDM regimen to HDM in terms of response rates, toxicities, and transplant-related mortality.


Subject(s)
Antineoplastic Agents/administration & dosage , Bortezomib/administration & dosage , Hematopoietic Stem Cell Transplantation/methods , Melphalan/administration & dosage , Multiple Myeloma/therapy , Transplantation Conditioning/methods , Adult , Aged , Antineoplastic Agents, Alkylating/administration & dosage , Dose-Response Relationship, Drug , Female , Humans , Male , Middle Aged , Multiple Myeloma/diagnosis , Retrospective Studies , Transplantation, Autologous
11.
Br J Haematol ; 180(1): 100-109, 2018 01.
Article in English | MEDLINE | ID: mdl-29094350

ABSTRACT

The outcomes of adult patients transplanted for Fanconi anaemia (FA) have not been well described. We retrospectively analysed 199 adult patients with FA transplanted between 1991 and 2014. Patients were a median of 16 years of age when diagnosed with FA, and underwent transplantation at a median age of 23 years. Time between diagnosis and transplant was shortest (median 2 years) in those patients who had a human leucocyte antigen identical sibling donor. Fifty four percent of patients had bone marrow (BM) failure at transplantation and 46% had clonal disease (34% myelodysplasia, 12% acute leukaemia). BM was the main stem cell source, the conditioning regimen included cyclophosphamide in 96% of cases and fludarabine in 64%. Engraftment occurred in 82% (95% confidence interval [CI] 76-87%), acute graft-versus-host disease (GvHD) grade II-IV in 22% (95% CI 16-28%) and the incidence of chronic GvHD at 96 months was 26% (95% CI 20-33). Non-relapse mortality at 96 months was 56% with an overall survival of 34%, which improved with more recent transplants. Median follow-up was 58 months. Patients transplanted after 2000 had improved survival (84% at 36 months), using BM from an identical sibling and fludarabine in the conditioning regimen. Factors associated with improved outcome in multivariate analysis were use of fludarabine and an identical sibling or matched non-sibling donor. Main causes of death were infection (37%), GvHD (24%) and organ failure (12%). The presence of clonal disease at transplant did not significant impact on survival. Secondary malignancies were reported in 15 of 131 evaluable patients.


Subject(s)
Fanconi Anemia/therapy , Hematopoietic Stem Cell Transplantation , Adolescent , Adult , Cause of Death , Fanconi Anemia/diagnosis , Fanconi Anemia/mortality , Graft vs Host Disease/etiology , Hematopoietic Stem Cell Transplantation/adverse effects , Hematopoietic Stem Cell Transplantation/methods , Humans , Middle Aged , Neoplasms, Second Primary/etiology , Prognosis , Retrospective Studies , Risk Factors , Survival Analysis , Tissue Donors , Transplantation Conditioning , Transplantation, Homologous , Treatment Outcome , Young Adult
12.
Br J Haematol ; 178(1): 61-71, 2017 07.
Article in English | MEDLINE | ID: mdl-28382618

ABSTRACT

This phase II study explored the effects of bortezomib consolidation versus observation on myeloma-related bone disease in patients who had a partial response or better after frontline high-dose therapy and autologous stem cell transplantation. Patients were randomized to receive four 35-day cycles of bortezomib 1·6 mg/m2 intravenously on days 1, 8, 15 and 22, or an equivalent observation period, and followed up for disease status/survival. The modified intent-to-treat population included 104 patients (51 bortezomib, 53 observation). There were no meaningful differences in the primary endpoint of change from baseline to end of treatment in bone mineral density (BMD). End-of-treatment rates (bortezomib versus observation) of complete response/stringent complete response were 22% vs. 11% (P = 0·19), very good partial response or better of 80% vs. 68% (P = 0·17), and progressive disease of 8% vs. 23% (P = 0·06); median progression-free survival was 44·9 months vs. 21·8 months (P = 0·22). Adverse events observed ≥15% more frequently with bortezomib versus observation were diarrhoea (37% vs. 0), peripheral sensory neuropathy (20% vs. 4%), nausea (18% vs. 0) and vomiting (16% vs. 0). Compared with observation, bortezomib appeared to have little impact on bone metabolism/health, but was associated with trends for improved myeloma response and survival.


Subject(s)
Antineoplastic Agents/therapeutic use , Bortezomib/therapeutic use , Consolidation Chemotherapy/methods , Multiple Myeloma/drug therapy , Osteolysis/drug therapy , Adult , Aged , Antineoplastic Agents/administration & dosage , Antineoplastic Agents/adverse effects , Biomarkers/blood , Bortezomib/administration & dosage , Bortezomib/adverse effects , Drug Administration Schedule , Female , Follow-Up Studies , Humans , Kaplan-Meier Estimate , Male , Middle Aged , Multiple Myeloma/complications , Multiple Myeloma/physiopathology , Osteolysis/etiology , Osteolysis/physiopathology , Stem Cell Transplantation , Treatment Outcome
13.
Cancer Invest ; 35(3): 195-201, 2017 Mar 16.
Article in English | MEDLINE | ID: mdl-28112977

ABSTRACT

We hypothesized the levels of free light chains obtained before and after autologous stem cell transplantation can be useful in predicting transplantation outcome. We analyzed 70 multiple myeloma patients. Abnormal free light chain ratios before stem cell transplantation were found to be associated early progression, although without any impact on overall survival. At day +30, the normalization of levels of involved free light chain related with early progression. According to these results almost one-third reduction of free light chain levels can predict favorable prognosis after autologous stem cell transplantation.


Subject(s)
Biomarkers, Tumor/blood , Immunoglobulin Light Chains/blood , Multiple Myeloma/blood , Adult , Aged , Combined Modality Therapy , Disease Progression , Disease-Free Survival , Female , Hematopoietic Stem Cell Transplantation , Humans , Kaplan-Meier Estimate , Male , Middle Aged , Multiple Myeloma/mortality , Multiple Myeloma/therapy , Prognosis , Retrospective Studies , Transplantation, Autologous , Treatment Outcome
14.
J Clin Apher ; 32(5): 295-301, 2017 Oct.
Article in English | MEDLINE | ID: mdl-27614935

ABSTRACT

High-dose chemotherapy followed by autologous stem cell transplantation (ASCT) to rescue hematopoiesis is considered standard care for patients with a relapsed chemosensitive lymphoma, but diagnosis of lymphoma has been a risk factor for poor mobilization in several studies. The aim of this prospective noninterventional clinical audit was to review the mobilization strategies used by EBMT centers in relapsed lymphoma and to evaluate their efficacy. Between 2010 and 2014, 275 patients with relapsed lymphoma from 30 EBMT centers were prospectively registered. Almost all patients were mobilized with chemotherapy plus G-CSF (96%), but there was a large variation in chemotherapy schedules. Thirty (11%) of them were poor mobilizers (<2 × 106 CD 34+ cells/kg body weight) at the first mobilization. Poor mobilization was not associated with gender, age, bone marrow involvement at diagnosis, primary diagnosis, number of previous chemotherapy lines, previous radiotherapy or mobilization with G-CSF alone. The use of high dose cyclophosphamide alone was associated with mobilization failure (P = 0.0006), whereas the use of a platinum-containing regimen was associated with a good mobilization outcome (P = 0.013). Because failure rate is low, we can conclude from this study that PBSC mobilization failure in relapsed lymphomas is not an important problem in the EBMT centers.


Subject(s)
Hematopoietic Stem Cell Mobilization/methods , Lymphoma/blood , Lymphoma/therapy , Peripheral Blood Stem Cells/pathology , Adolescent , Adult , Aged , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Clinical Audit , Combined Modality Therapy , Female , Humans , Lymphoma/drug therapy , Male , Middle Aged , Peripheral Blood Stem Cell Transplantation , Prospective Studies , Recurrence , Transplantation, Autologous , Treatment Failure , Young Adult
15.
Turk J Pediatr ; 58(1): 113-115, 2016.
Article in English | MEDLINE | ID: mdl-27922248

ABSTRACT

We report herein on our observation of recessive congenital methemoglobinemia (type I), an autosomal recessive disorder, in immediate generations (in a mother and her daughter). Molecular analysis revealed a mechanism of inheritance not reported previously, despite the high probability of occurrence in autosomal recessive disorders. This report is also the first publication describing an extremely rare mutation (Arg50Gln) causing this disorder in the Turkish population.


Subject(s)
Cytochrome-B(5) Reductase/genetics , Methemoglobinemia/congenital , Adolescent , Female , Humans , Male , Methemoglobinemia/diagnosis , Methemoglobinemia/genetics , Middle Aged , Mutation , Turkey
16.
Turk J Haematol ; 33(1): 34-40, 2016 Mar 05.
Article in English | MEDLINE | ID: mdl-26376938

ABSTRACT

OBJECTIVE: Chemo/radiotherapy-induced free oxygen radicals and reactive oxygen derivatives contribute to the development of early and late transplantation-related pulmonary and extra-pulmonary complications in hematopoietic stem cell transplantation (HSCT) recipients. It has been proposed that an increase in fractional exhaled nitric oxide (FeNO) level indicates oxidative stress and inflammation in the airways. The aim of this prospective study is to evaluate the pre-transplantation FeNO levels in HSCT patients and to search for its role in predicting post-transplantation pulmonary complications and mortality. MATERIALS AND METHODS: HSCT patients were included in the study prospectively between October 2009 and July 2011. Pre-transplantation FeNO levels were measured with a NIOX MINO® device prior to conditioning regimens. All patients were monitored prospectively for post-transplantation pulmonary complications with medical history, physical examination, chest X-ray, and pulmonary function tests. RESULTS: A total of 56 patients (33 autologous, 23 allogeneic) with mean age of 45±13 years were included in the study, among whom 40 (71%) were male. Pre-transplantation FeNO level of the whole study group was found to be 24±13 (mean ± standard deviation) parts per billion (ppb). The FeNO level in allogeneic HSCT recipients was 19±6 ppb while it was 27±15 ppb in autologous HSCT recipients (p=0.042). No significant correlation was found between the pre-transplantation chemotherapy and radiotherapy protocols and baseline FeNO levels (p>0.05). Post-transplantation pulmonary toxicity was identified in 12 (21%) patients and no significant relationship was found between baseline FeNO levels and pulmonary toxicity. The survival rate of the whole study group for 1 year after transplantation was 70%. No significant relationship was identified between baseline FeNO values and survival (FeNO 19±7 ppb in patients who died and 26±15 ppb in the survivors; p=0.114). CONCLUSION: Pre-transplantation FeNO measurement does not seem to have a role in predicting post-transplantation pulmonary complications and mortality.


Subject(s)
Breath Tests , Bronchiolitis Obliterans/etiology , Hematopoietic Stem Cell Transplantation/adverse effects , Nitric Oxide/analysis , Pneumonia/etiology , Adult , Antibiotic Prophylaxis , Antineoplastic Agents/adverse effects , Bronchiolitis Obliterans/diagnosis , Bronchiolitis Obliterans/metabolism , Female , Graft vs Host Disease/etiology , Graft vs Host Disease/prevention & control , Hematopoietic Stem Cell Transplantation/mortality , Humans , Immunosuppressive Agents/adverse effects , Immunosuppressive Agents/therapeutic use , Inflammation , Lung/drug effects , Lung/radiation effects , Male , Middle Aged , Oxidative Stress , Pneumonia/diagnosis , Pneumonia/metabolism , Prospective Studies , Radiotherapy/adverse effects , Transplantation Conditioning/adverse effects , Transplantation Conditioning/mortality
17.
Support Care Cancer ; 24(2): 647-659, 2016 Feb.
Article in English | MEDLINE | ID: mdl-26135532

ABSTRACT

PURPOSE: Respiratory muscles are known to be weakened and are a cause of reduced exercise capacity in both recipients and candidates of allogeneic hematopoietic stem cell transplantation (allo-HSCT). Effects of inspiratory muscle training (IMT) in this patient population have not been comprehensively investigated so far. The current study was planned to investigate the effects of IMT during allo-HSCT on early transplantation-related outcomes. METHODS: This is a prospective, randomized controlled, double-blinded study. Thirty-eight allo-HSCT recipients, 20 of whom were allocated to the treatment group (40 % of maximal inspiratory pressure (MIP)) and 18 to the control group (5 % of MIP), received IMT for 6 weeks. Pulmonary functions, dyspnea, respiratory (MIP, maximal expiratory pressure (MEP)) and peripheral muscle strength, maximal exercise capacity using modified incremental shuttle walking test (MISWT) and submaximal exercise capacity using 6-min walking test (6-MWT), fatigue, depression, and quality of life were evaluated before and after IMT. RESULTS: The distance covered during MISWT (61.94 m) and 6-MWT (29.30 m), respiratory muscle strength (MIP 34.99 cmH2O, MEP 12.69 cmH2O), depression (-0.95), and modified Borg dyspnea scores (-0.11) showed a significant improvement in the treatment group compared to controls (p ≤ 0.05). CONCLUSIONS: Inspiratory muscle training is a safe and effective intervention which improves respiratory muscle strength and exercise capacity and decreases depression and dyspnea in allo-HSCT recipients. These positive changes might be further enhanced by prolonging the duration of training or inclusion of more recipients with inspiratory muscle weakness. CLINICAL TRIAL REGISTRATION NUMBER: NCT02270346.


Subject(s)
Breathing Exercises/methods , Hematopoietic Stem Cell Transplantation/methods , Inspiratory Capacity/physiology , Respiratory Muscles/physiology , Adolescent , Adult , Aged , Double-Blind Method , Hematopoietic Stem Cell Transplantation/adverse effects , Humans , Male , Middle Aged , Physical Therapy Modalities , Prospective Studies , Quality of Life , Transplantation, Homologous , Young Adult
18.
Case Rep Hematol ; 2015: 780159, 2015.
Article in English | MEDLINE | ID: mdl-26060589

ABSTRACT

Background. The sickling of red blood cells causes a constellation of musculoskeletal, cardiovascular, and pulmonary manifestations. A 32-year-old gentleman with sickle cell anemia (SCA) had been suffering from recurrent acute chest syndrome (ACS). Aim. To examine the effects of inspiratory muscle training (IMT) on pulmonary functions, respiratory and peripheral muscle strength, functional exercise capacity, and quality of life in this patient with SCA. Methods. Functional exercise capacity was evaluated using six-minute walk test, respiratory muscle strength using mouth pressure device, hand grip strength using hand-held dynamometer, pain using Visual Analogue Scale, fatigue using Fatigue Severity Scale, dyspnea using Modified Medical Research Council Scale, and health related quality of life using European Organization for Research and Treatment of Cancer QOL measurement. Results. A significant improvement has been demonstrated in respiratory muscle strength, functional exercise capacity, pain, fatigue, dyspnea, and quality of life. There was no admission to emergency department due to acute chest syndrome in the following 12 months after commencing regular erythrocytapheresis. Conclusion. This is the first report demonstrating the beneficial effects of inspiratory muscle training on functional exercise capacity, respiratory muscle strength, pain, fatigue, dyspnea, and quality of life in a patient with recurrent ACS.

19.
Leuk Lymphoma ; 56(12): 3295-300, 2015.
Article in English | MEDLINE | ID: mdl-25899403

ABSTRACT

Extra-nodal NK/T lymphoma (ENKTL) is rare and more frequently encountered in East Asia. The role of high-dose therapy and autologous stem cell transplantation (HDT-ASCT) for ENKTL is unclear. Twenty-eight evaluable patients who had undergone HDT-ASCT in Europe from 2000-2009 were studied. The median age was 47 years and patients had received a median of two lines of prior therapy. Some 57% of patients were not in complete remission or beyond first complete remission at HDT-ASCT. The 1-year non-relapse mortality (NRM) was 11%; 2-year progression-free survival (PFS) and overall survival (OS) rates were 41% and 52%, respectively. Notably, the 2-year PFS and OS for those with stage III/IV disease were 33% and 40%, respectively, with no relapses beyond 1-year post-HDT-ASCT. This is the largest analysis of HDT-ASCT for patients with ENKTL reported from the Western hemisphere. Survival is comparable to East Asian cohorts and outcomes are encouraging for patients with advanced disease.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Hematopoietic Stem Cell Transplantation , Lymphoma, Extranodal NK-T-Cell/therapy , Adult , Aged , Combined Modality Therapy , Europe/epidemiology , Female , Follow-Up Studies , Hematopoietic Stem Cell Transplantation/adverse effects , Hematopoietic Stem Cell Transplantation/methods , Humans , Lymphoma, Extranodal NK-T-Cell/diagnosis , Lymphoma, Extranodal NK-T-Cell/epidemiology , Lymphoma, Extranodal NK-T-Cell/mortality , Male , Middle Aged , Neoplasm Staging , Registries , Retreatment , Transplantation Conditioning , Transplantation, Autologous , Treatment Outcome , Young Adult
20.
Acta Haematol ; 133(4): 372-380, 2015.
Article in English | MEDLINE | ID: mdl-25824293

ABSTRACT

BACKGROUND/AIM: Low-molecular-weight heparin (LMWH) has been shown to prolong survival among patients with solid tumors, but its role among myeloma patients is unknown. PATIENTS: Data from the GIMEMA (Gruppo Italiano Malattie Ematologiche dell'Adulto), Nordic and Turkish myeloma study groups comparing melphalan and prednisolone with (MPT, n: 404) or without thalidomide (MP, n: 393) are analyzed for effects of LMWH. Forty percent (159/394) of the patients on MPT and 7.4% (29/390) in the MP arm received LMWH. RESULTS: Thalidomide improved response and progression-free survival (PFS). Regardless of thalidomide treatment, response rate was higher among those receiving LMWH vs. none vs. other anticoagulants (58.1 vs. 44.9 vs. 50.4%, p = 0.01). PFS was significantly longer (median 32 vs. 21 and 17 vs. 17 months, p = 0.004) only among international scoring system (ISS) I patients receiving MPT ± LMWH vs. MP ± LMWH. The group of MPT patients who also received LMWH had a better OS compared to those who did not [45 months, 95% confidence interval (CI) 27.7-62.3, vs. 32 months, 95% CI 26.1-37.9; p = 0.034]. When multivariate analysis was repeated in subgroups, thalidomide was no longer a significant factor (response, PFS) among those receiving LMWH. CONCLUSION: Addition of LMWH to MPT, in particular in patients with low ISS, suggests additive effects, but the results are limited by the retrospective design of our study.


Subject(s)
Anticoagulants/therapeutic use , Antineoplastic Agents/therapeutic use , Heparin, Low-Molecular-Weight/therapeutic use , Multiple Myeloma/drug therapy , Thalidomide/therapeutic use , Aged , Aged, 80 and over , Disease-Free Survival , Drug Therapy, Combination , Female , Follow-Up Studies , Humans , Kaplan-Meier Estimate , Male , Melphalan/therapeutic use , Middle Aged , Multiple Myeloma/mortality , Prednisone/therapeutic use , Retrospective Studies , Treatment Outcome , Turkey
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