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Immune thrombocytopenia (ITP) is characterized by reduced platelet count due to increased destruction and is categorized according to the time following diagnosis (newly diagnosed, persistent, chronic). First-line corticosteroid therapy is associated with transient response, high relapse rates, and considerable toxicity. TAPER (NCT03524612) is a Phase II, prospective, single-arm trial investigating whether eltrombopag can induce a sustained response off-treatment (SRoT) in adult patients with ITP after first-line corticosteroid failure. This study defines SRoT as an off-treatment period wherein platelet count remains above 30 × 109 /L in the absence of bleeding or rescue therapy. The primary endpoint was the proportion of patients who achieved SRoT until Month 12, which was 30.5% (n = 32/105; p < .0001 testing hypothesis H1: proportion >15%) following eltrombopag tapering and discontinuation, and median SRoT duration was ~8 months until Month 12. Median platelet count increased within 1 month of treatment and remained elevated until Month 12. Quality of life improved within 3 months and was maintained. Headache (21%) was the most common adverse event. None of the 4 deaths reported were considered treatment-related. In summary, ~one-third of patients achieved SRoT until Month 12 following eltrombopag tapering and discontinuation. An ad-hoc early-use analysis, stratified by ITP duration at baseline, assessed initial hematologic responses and safety. Results suggest that eltrombopag has similar efficacy in newly diagnosed and later stages of ITP. In follow-up until Month 24, a median SRoT duration of ~22 months was observed (n = 20). The safety profile was comparable across analyses and ITP duration groups and aligned with its well-established safety profile.
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Púrpura Trombocitopênica Idiopática , Trombocitopenia , Adulto , Humanos , Púrpura Trombocitopênica Idiopática/complicações , Estudos Prospectivos , Qualidade de Vida , Resultado do Tratamento , Trombocitopenia/induzido quimicamente , Benzoatos/efeitos adversos , Hidrazinas/efeitos adversos , Esteroides , CorticosteroidesRESUMO
Glofitamab is a CD3xCD20 bi-specific antibody with two fragments directed to the CD20 antigen and a single CD3-binding fragment. Encouraging response and survival rates were recently reported in a pivotal phase II expansion trial conducted in patients with relapsed/refractory (R/R) B-cell lymphoma. However, the real-world data of patients of all ages with no strict selection criteria are still lacking. Herein, this retrospective study aimed to evaluate the outcomes of diffuse large B-cell lymphoma (DLBCL) patients who received glofitamab via compassionate use in Turkey. Forty-three patients from 20 centers who received at least one dose of the treatment were included in this study. The median age was 54 years. The median number of previous therapies was 4, and 23 patients were refractory to first-line treatment. Twenty patients had previously undergone autologous stem cell transplantation. The median follow-up time was 5.7 months. In efficacy-evaluable patients, 21% and 16% of them achieved complete response and partial response, respectively. The median response duration was 6.3 months. The median progression-free survival (PFS) and overall survival (OS) was 3.3 and 8.8 months, respectively. None of the treatment-responsive patients progressed during the study period, and their estimated 1-year PFS and OS rate was 83%. The most frequently reported toxicity was hematological toxicity. Sixteen patients survived, while 27 died at the time of the analysis. The most common cause of death was disease progression. One patient died of cytokine release syndrome during the first cycle after receiving the first dose of glofitamab. Meanwhile, two patients died due to glofitamab-related febrile neutropenia. This is the largest real-world study on the effectiveness and toxicity of glofitamab treatment in R/R DLBCL patients. The median OS of 9 months seems promising in this heavily pretreated group. The toxicity related mortality rates were the primary concerns in this study.
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Evans syndrome is a rare condition characterized by simultaneous or sequential development of autoimmune hemolytic anemia and immune thrombocytopenia (and/or immune neutropenia). Coronavirus disease 2019 (COVID-19) may cause various hematologic conditions, such as coagulation abnormalities (e.g., bleeding or thrombosis) or cell count alterations (e.g., lymphopenia and neutrophilia). COVID-19 may also induce Evans syndrome via immune mechanisms. Here, we describe the case of a patient developing Evans syndrome shortly after COVID-19 infection. Immune thrombocytopenia and warm-type autoimmune hemolytic anemia developed simultaneously, and intravenous immunoglobulin and methylprednisolone were initially administered. Additionally, we intend to review all COVID-19-induced Evans syndrome cases currently present in the literature and emphasize the differences as well as the similarities regarding patient characteristics, relationship to COVID-19 infection, and treatment approach. Since autoimmune cytopenias are frequent in COVID-19 patients, clinicians should pay particular attention to profound and abrupt-onset cytopenias. In these circumstances, hemolysis markers such as lactate dehydrogenase, haptoglobulin, Coombs tests, etc. should be investigated, and the possibility of Evans syndrome should always be considered to ensure prompt and appropriate treatment. These factors are essential to ensure hematologic recovery and prevent complications such as thrombosis.
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Anemia Hemolítica Autoimune , COVID-19 , Púrpura Trombocitopênica Idiopática , Trombocitopenia , Anemia Hemolítica Autoimune/complicações , Anemia Hemolítica Autoimune/terapia , COVID-19/complicações , Humanos , Púrpura Trombocitopênica Idiopática/complicações , Púrpura Trombocitopênica Idiopática/terapia , Trombocitopenia/complicaçõesRESUMO
The AETHERA trial reported an increased progression-free survival (PFS) when brentuximab vedotin (BV) was used as maintenance therapy in high-risk Hodgkin lymphoma (HL) after autologous stem cell transplantation (ASCT). Thus, we aimed to determine the impact and safety of BV as maintenance after ASCT in real-world patients. Seventy-five patients with relapsed/refractory HL started on BV consolidation therapy after ASCT due to high risk of relapse, between January 2016 and July 2019, from 25 institutions, were included in the study. The median follow-up time was 26 months. The most common high-risk features were primary refractory or relapsed disease <12 months (n = 61), lack of complete response (CR) to the last salvage regimen (n = 51), and having had at least two salvage regimens (n = 29). At the time of analysis, 42 patients completed consolidation courses, and BV was discontinued in 33 patients. Fifty patients had an ongoing response (CR in 41, PR in 6, and SD in 3 patients), 25 had progressed. Ten died in the follow-up, eight with progressive disease and two due to infection while in CR. The 2-year PFS and OS rates were 67.75% (95% confidence interval [CI]: 0.55-0.77) and 87.61% (95% CI: 0.76-0.94), respectively. Seventeen patients (23%) received BV in the pre-ASCT treatment lines, and there was no survival difference between the BV-naïve and BV-exposed groups. The most common adverse events were neutropenia (27%) and peripheral neuropathy (21%). Sixteen patients (21.3%) experienced grade 3 or 4 toxicity. BV was discontinued due to adverse event in 12 patients. Consolidation with BV after ASCT can achieve a 2-year PFS of 67.75% (95% CI: 0.55-0.75) with an acceptable toxicity profile.
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Brentuximab Vedotin/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/métodos , Doença de Hodgkin/tratamento farmacológico , Condicionamento Pré-Transplante/métodos , Adolescente , Adulto , Idoso , Brentuximab Vedotin/farmacologia , Humanos , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto JovemRESUMO
The diabetic fot/ulcer is the cause of high morbidity and mortality in patients with diabetes mellitus (DM). Generally, medical treatment of diabetic foot/ulcer is ineffective and stem cell implantation is an important option in the treatment. Here, we present a 69 years old man admitted to hospital due to a 3 × 4 cm wound in the plantar surface of left foot. Autologous stem cells were applied intralesionally into diabetic ulcers. The lesion shrunken 50 % at the 16th week and there is a wound under the left foot at 32nd week. Intralesionally autologous stem cell application was useful and safe without adverse course in patients with diabetic foot/ulcer.
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Angiopatias Diabéticas/terapia , Pé Diabético/terapia , Transplante de Células-Tronco Hematopoéticas/métodos , Condicionamento Pré-Transplante/métodos , Transplante Autólogo/métodos , Úlcera/terapia , Idoso , Humanos , MasculinoRESUMO
INTRODUCTION: There are limited numbers of available retrospective studies on various hematological diseases treated with stem cell mobilization therapy. In the present study, we aimed to demonstrate the effects of serum lipid levels on peripheral blood CD34+ (PBCD34+) cell counts as well as the changes in serum lipid levels during stem cell mobilization process. METHOD: PBCD34+ cell counts were compared between hypercholesterolemic patients and healthy individuals. Additionally, total cholesterol (TChol), LDL-cholesterol (LDL-C), HDL-cholesterol (HDL-C), and triglyceride (TG) levels were measured from healthy donors who underwent stem cell mobilization, at different time points (prior to filgrastim [phase 1], prior to apheresis [phase II], and the first week following apheresis [phase III]. RESULTS: In the hypercholesterolemia group, the PBCD34+ cell count was found to be higher among patients with elevated LDL-C (2.6 ± 0.35/µL vs. 1.7 ± 0.17/µL, p = 0.003) and TChol (2.6 ± 0.34/µL vs. 1.7 ± 0.14/µL, p = 0.006) in comparison to the healthy controls. In the mobilization group, phase II HDL-C levels (35.3 ± 2.8 mg/dL) were found to be lower than both phase I (45.6 ± 2.1 mg/dL) and phase III (44.5 ± 2.6 mg/dL) (p = 0.007). Phase II TChol levels (183.5 ± 10.0 mg/dL) were lower than both phase I (216.8 ± 8.5 mg/dL) and phase III (212.2 ± 8.4 mg/dL) (p = 0.02). At phase II, there was an inverse correlation between PBCD34+ cell count and HDL-C (r = - 0.57, p = 0.003). DISCUSSION: Our results indicate that, while increased LDL-C level is the determinant of baseline PBCD34+ cell count, reduced HDL-C is the determinant of PBCD34+ cell count during mobilization process.
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Lipídeos/sangue , Células-Tronco de Sangue Periférico/metabolismo , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Fatores de RiscoRESUMO
Background/aim: The aim of this study is to assess the efficacy and safety of ruxolitinib in patients with myelofibrosis. Materials and methods: From 15 centers, 176 patients (53.4% male, 46.6% female) were retrospectively evaluated. Results: The median age at ruxolitinib initiation was 62 (2887) and 100 (56.8%) of all were diagnosed as PMF. Constitutional symptoms were observed in 84.7%. The median initiation dose of ruxolitinib was 30 mg (1040). Dose change was made in 69 (39.2%) patients. Forty seven (35.6%) and 20 (15.2%) of 132 patients had hematological and nonhematological adverse events, respectively. The mean spleen sizes before and after ruxolitinib treatment were 219.67 ± 46.79 mm versus 199.49 ± 40.95 mm, respectively (p < 0.001). There was no correlation between baseline features and subsequent spleen response. Overall survival at 1-year was 89.5% and the median follow up was 10 (155) months. We could not show any relationship between survival and reduction in spleen size (p = 0.73). Conclusion: We found ruxolitinib to be safe, well tolerated, and effective in real-life clinical practice in Turkey. Ruxolitinib dose titration can provide better responses in terms of not only clinical benefit but also for long term of ruxolitinib treatment.
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Nitrilas/uso terapêutico , Mielofibrose Primária , Pirazóis/uso terapêutico , Pirimidinas/uso terapêutico , Feminino , Humanos , Masculino , Mielofibrose Primária/tratamento farmacológico , Mielofibrose Primária/epidemiologia , Pirazóis/efeitos adversos , Estudos Retrospectivos , Turquia/epidemiologiaRESUMO
BACKGROUND: Ulcerative colitis (UC) is a chronic, inflammatory bowel diseases characterized by uncontrolled inflammatory condition of the colon and rectal mucosa marked by recurrent periods of remission and exacerbation. Vitamin D receptor (VDR) is a member of the steroid receptor family that mediates the effects of vitamin D by regulating transcription of multiple cellular genes. We aimed to evaluate vitamin d receptor level in biopsy specimen of patients with UC in this study. METHODS: VDR levels were retrospectively studied in colon biopsy specimens of UC patients. The Spearman's rho correlation analysis, The Kolmogorov-Smirnov, Mann Whitney U, and chi-square tests were used for statistical analysis. The p values below 0.05 were considered statistically significant. RESULTS: Study included 112 UC patients (65 male and 47 female) and 30 controls (19 female and 11 male) who had normal results in biopsy examinations carried out due to various reasons. VDR levels of UC patients were statistically lower than control subjects, and was not associated with duration of the disease and place of involvement. CONCLUSIONS: VDR is an important receptor in the pathogenesis of UC, and optimizing vitamin D levels could have a therapeutic role in UC.
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Colite Ulcerativa , Colo , Receptores de Calcitriol/metabolismo , Reto , Adulto , Biópsia/métodos , Colite Ulcerativa/metabolismo , Colite Ulcerativa/patologia , Colo/metabolismo , Colo/patologia , Correlação de Dados , Feminino , Humanos , Mucosa Intestinal/metabolismo , Mucosa Intestinal/patologia , Masculino , Pessoa de Meia-Idade , Reto/metabolismo , Reto/patologia , Reprodutibilidade dos Testes , TurquiaRESUMO
AIM: Anti-tumor necrosis factor-alpha (Anti-TNF-α) therapy has achieved an important position, are widely used for ankylosing spondylitis (AS) patients. TNF-α inhibition improves clinical outcomes and has differential effects on haematopoiesis. Information about effects on eosinophils is limited. The aim of our study is to determine the relationship between blood eosinophil counts in AS patients treated with TNF-α inhibitors. METHODS: Seventy-five patients diagnosed with AS according to modified New York criteria were enrolled in this study. Disease activity was assessed by BASDAI, and erythrocyte sedimentation rate, C-reactive protein of patients were evaluated. All data were analyzed with Spearman's correlation and Friedman's Two-Way by using SPSS version 19.0 statistical software, and pâ¯<â¯0.05 was considered as statistically significant. RESULTS: Seventy-five AS (F/M: 27/48, the mean age of 41⯱â¯10â¯years) patients were evaluated. On the 3rd month of treatment, there was a correlation between BASDAI and CRP (râ¯=â¯0.32, pâ¯=â¯0.005), but no correlation between BASDAI and ESR (râ¯=â¯0.21, pâ¯=â¯0.06). Blood eosinophil count was not correlated with BASDAI, ESR and CRP on pre-, post-therapy (pâ¯>â¯0.05). It counts lower before anti-TNF-α therapy compare with post-treatment (130/mm3, 140/mm3 and 190/mm3, respectively). There was no statistically significant difference between pre- and 3rd month (pâ¯>â¯0.05), while correlation was found between pre- and 6th month, and also 3rd month and 6th month (pâ¯<â¯0.001, pâ¯=â¯0.002, respectively) for blood eosinophil count. CONCLUSION: To the best of our knowledge, our study is the first to evaluate blood eosinophil counts and disease activity with anti-TNF-α therapy. Blood eosinophil count may be affected by TNF-α inhibition in patients with AS.
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Low cholesterol levels may be accompanied by solid tumors or hematological malignancies such as multiple myeloma. Decreased cholesterol levels have been reported in some experimental studies about chronic lymphocytic leukemia (CLL). It may be associated with tumoral cell metabolism. Herein, we examine blood lipid profiles of patients with newly diagnosed CLL (284 male, 276 female, mean age 64 ± 11 years) as defined by National Cancer Institute criteria. The control group consisted of 71 healthy subjects with mean age 55 ± 9 years (28 male, 43 females). 60% of patients with Binet A, while 25% were Binet C. Decreased levels of total cholesterol, high density lipoprotein (HDL) and low density lipoprotein (LDL) were observed in patients with CLL than control group (p < 0,001). There was no statistical significance between CLL and control group for triglycerides (TG) and very low density lipoprotein (VLDL), also between HDL-C, VLDL, TG and grades. Cholesterol may metabolized by abnormal lymphocytes in CLL patients.
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Linfócitos B/metabolismo , HDL-Colesterol/sangue , LDL-Colesterol/sangue , Leucemia Linfocítica Crônica de Células B , Idoso , Correlação de Dados , Feminino , Humanos , Leucemia Linfocítica Crônica de Células B/sangue , Leucemia Linfocítica Crônica de Células B/patologia , Metabolismo dos Lipídeos , Masculino , Pessoa de Meia-IdadeRESUMO
OBJECTIVES: Fabry's disease is an X-linked inherited, rare, progressive, lysosomal storage disorder, affecting multiple organs due to the deficient activity of α-galactosidase A (α-Gal A) enzyme. The prevalence has been reported to be 0.15-1% in hemodialysis patients; however, the information on the prevalence in chronic kidney disease not on dialysis is lacking. This study aimed to determine the prevalence of Fabry's disease in chronic kidney disease. METHODS: The patients older than 18 years, enclosing KDIGO 2012 chronic kidney disease definitions, not on dialysis, were enrolled. Dried blood spots on Guthrie papers were used to analyze α-Gal A enzyme and genetic analysis was performed in individuals with enzyme activity ≤1.2 µmol/L/h. RESULTS: A total of 1453 chronic kidney disease patients not on dialysis from seven clinics in Turkey were screened. The mean age of the study population was 59.3 ± 15.9 years. 45.6% of patients were female. The creatinine clearance of 77.3% of patients was below 60 mL/min/1.73 m2, 8.4% had proteinuria, and 2.5% had isolated microscopic hematuria. The mean value of patients' α-Gal A enzyme was detected as 2.93 ± 1.92 µmol/L/h. 152 patients had low levels of α-Gal A enzyme activity (≤1.2 µmol/L/h). In mutation analysis, A143T and D313Y variants were disclosed in three male patients. The prevalence of Fabry's disease in chronic kidney disease not on dialysis was found to be 0.2% (0.4% in male, 0.0% in female). CONCLUSION: Fabry's disease should be considered in the differential diagnosis of chronic kidney disease with unknown etiology even in the absence of symptoms and signs suggestive of Fabry's disease.
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Doença de Fabry/epidemiologia , Rim/patologia , Proteinúria/epidemiologia , Insuficiência Renal Crônica/complicações , alfa-Galactosidase/sangue , Adulto , Idoso , Estudos Transversais , Doença de Fabry/genética , Feminino , Humanos , Masculino , Programas de Rastreamento , Pessoa de Meia-Idade , Linhagem , Turquia , alfa-Galactosidase/genéticaRESUMO
OBJECTIVE: The aim of this study was to highlight the use of combined intravenous lipid emulsion (ILE) and plasma exchange (PE) therapies in multidrug toxicity. CLINICAL PRESENTATION AND INTERVENTION: A 45-year-old woman who attempted suicide by ingesting large quantities of amisulpride (28 g), diazepam (250 mg), valsartan (2,240 mg), aripiprazole (45 mg) and paliperidone (21 mg) was taken to the hospital of Adnan Menderes University School of Medicine. Upon arrival, she exhibited signs of cardiotoxicity and severe depression of the central nervous and respiratory systems. She was treated successfully with ILE for 4 h and PE therapy for 36 h, consecutively. She was discharged on the fourth day of hospitalization having fully recovered. CONCLUSION: The patient was successfully treated with the combination of ILE and PE.
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Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/terapia , Emulsões Gordurosas Intravenosas/uso terapêutico , Troca Plasmática , Tentativa de Suicídio , Amissulprida , Anti-Hipertensivos/efeitos adversos , Antipsicóticos/efeitos adversos , Aripiprazol/efeitos adversos , Diazepam/efeitos adversos , Feminino , Humanos , Hipnóticos e Sedativos/efeitos adversos , Hipotensão/terapia , Pessoa de Meia-Idade , Palmitato de Paliperidona/efeitos adversos , Sulpirida/efeitos adversos , Sulpirida/análogos & derivados , Resultado do Tratamento , Turquia , Valsartana/efeitos adversosRESUMO
OBJECTIVE: Anti-apoptotic proteins such as Bcl-2 and Bcl-xL may play a role in the survival of erythroid progenitor cells. Information about these proteins in patients with ß-thalassemia minor is limited. We aimed to determine the levels of serum Bcl-2 in patients with ß-thalassemia minor. MATERIALS AND METHODS: Ninety-seven patients (60 females and 37 males with mean age of 29±21 years) with ß-thalassemia minor were enrolled in this study. The diagnosis of ß-thalassemia minor was based on whole blood counts, family history, and HbA2 levels estimated by high-performance liquid chromatography. The control group comprised 23 healthy adults (17 females and 6 males with mean age of 58±9 years) without anemia. The levels of serum Bcl-2 were measured by enzyme-linked immunosorbent assay. Mann-Whitney U tests were used in statistical evaluation and p<0.05 was accepted as statistically significant. RESULTS: Although there was no statistically significant difference between patients with ß-thalassemia minor and the control group for the level of serum Bcl-2 (p>0.05), these levels were higher in ß-thalassemia minor patients than controls. CONCLUSION: There are damaged beta chains in ß-thalassemia minor. Therefore, it is expected that premature death of red blood cells may occur due to apoptosis. The mean age of the control group was higher than that of the ß-thalassemia minor group; this may be why Bcl-2 levels were higher in the ß-thalassemia minor group. It is known that older age constitutes a risk for increased apoptosis. Other proteins (Bad, Bax, etc.) and pathways [CD95 (Fas) ligand] associated with apoptosis should be evaluated in future studies including more patients.
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We aimed to investigate the association between follicular T helper cells (Tfh) and disease severity in systemic sclerosis (SSc), a chronic connective tissue disease characterized by progressive fibrosis. While Tfh cells have been extensively studied in other autoimmune diseases, their role in SSc remains poorly understood. A cohort of 50 SSc patients, diagnosed based on the ACR/EULAR 2013 classification criteria, was included in the study. Patient data, including demographic information, comorbidities, treatment history and organ involvement, were collected. Disease severity was assessed using the modified Rodnan skin score and Medsger disease severity index. Statistical analyses were performed, considering a p value of < 0.05 as statistically significant. 38% had SSc with limited skin involvement, while 62% had SSc with extensive skin involvement. However, there were no statistically significant differences observed in the levels of CD4+ CXCR5+ , CD4+ ICOS+ , CD4+ CD40L+ and CD4+ PD+ lymphocytes between the two groups. Notably, SSc patients with Raynaud's phenomenon, digital ulcer and lung involvement exhibited higher levels of CD4+ CXCR5+ lymphocytes compared to those without these manifestations. Furthermore, a significant positive correlation was observed between CD4+ CXCR5+ lymphocyte levels and the severity of lung disease according to the Medsger disease severity index. Based on these findings, we conclude that elevated levels of Tfh cells are associated with lung involvement in SSc and there is a significant correlation between Tfh cell levels and the severity of lung disease. These observations suggest a potential role for Tfh cells in the pathogenesis of lung involvement in SSc and may guide the development of targeted therapies for this aspect of the disease.
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Pneumopatias , Escleroderma Sistêmico , Humanos , Células T Auxiliares Foliculares , Interleucinas , Índice de Gravidade de DoençaRESUMO
Malignant diseases occurring in elderly patients follow a different course from younger patients and show different genetic structures. Therefore, in this retrospective study, the somatic gene variant profile and fusion gene profiles of elderly and young acute leukemia patients were determined to draw attention to the existing genetic difference, and the results were compared. In this study, the records of 204 acute leukemia patients aged 18+ who were referred to the Molecular Pathology Laboratory from the Hematology Clinic between 2018 and 2022 were reviewed retrospectively. Fusion gene detection in patients was performed with the HemaVision®-28Q Panel. The NGS Myeloid Neoplasms Panel was conducted using the MiniSEQ NGS platform according to the manufacturer's protocol. When all cases are evaluated together, the most frequently diagnosed acute leukemia is acute myeloid leukemia (85.8%). Both groups had a similar fusion gene profile; however, the fusion burden was higher in the elderly group. When the groups were evaluated in terms of somatic gene variations, there were differences between the groups, and the variation load was higher in the elderly group. Considering the different somatic gene variation profiles, it is understood that the genetic structure of tumor cells is different in elderly patients compared to young cases.
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Introduction: Primary immune thrombocytopenia (ITP) is an acquired disorder of platelets with complex and unclear mechanism of increased immune distruction or impaired production of platelets. While management of ITP is evolving, there is a need for guidance particularly in certain circumstances such as pregnancy, emergency and for patients requiring co-medications. We aimed to determine the tendencies of hematologists in Turkiye on such special conditions. Methods: As a modified Delphi method, Turkish National ITP Working Group founded under Turkish Society of Hematology developed a questionnaire consisting of statements regarding pregnancy, emergency and circumstances regarding co-treatment with antiaggregant or anticoagulants. 107 Hematologists working either in university or state hospitals voted for their agreement or disagreement of the statements for two consequential rounds. Results: Participant hematologists reached an agreement on the starting treatment in pregnant patients with platelets less than 30 x109/L and delivery of either normal or cesarian section to be safely performed above 50 x109/L. For emergency and rescue management of ITP, our panel have agreed against the use of high dose corticosteroids alone, preferred a combination with transfusion or IVIG. For patients who require interventions, platelet counts >50 x109/L were regarded as safe for low risk procedures as well as co-treatment with antiplatelets or anticoagulants. Conclusion: As National ITP study group, we have observed the need to increase the practice guidance in patients with primary ITP requiring additional treatments including invasive interventions, and co-treatments towards coagulation. Decisions on the management of ITP during pregnancy should be individualized. There is a certain lack of consensus on the thresholds of platelet counts as well as co-morbidities and co-medications. This lack of consensus may be due to the variations in the practices.
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Objective: Primary immune thrombocytopenia (pITP) is an acquired autoimmune disorder related to the increased destruction and/or impaired production of platelets. Its diagnosis and management are challenging and require expertise and the interpretation of international consensus reports and guidelines with national variations in availability. We aimed to assess the agreement of hematologists in Türkiye on certain aspects of both first-line and second-line management of patients with pITP. Materials and Methods: Applying a modified Delphi method, the Turkish National ITP Working Group (14 steering committee members), founded under the auspices of the Turkish Society of Hematology, developed a 21-item questionnaire consisting of statements regarding the first-line and second-line treatment of pITP. A total of 107 adult hematologists working in either university or state hospitals voted for their agreement or disagreement with the statements in two consecutive rounds. Results: The participants reached consensus on the use of corticosteroids as first-line treatment and with limited duration. Methylprednisolone was the corticosteroid of choice rather than dexamethasone. Use of intravenous immunoglobulin was not preferred for patients without bleeding. It was also agreed that thrombopoietin receptor antagonists (TPO-RAs) or rituximab should be recommended as second-line treatment and that splenectomy could be considered 12-24 months after diagnosis in patients with chronic pITP. Conclusion: The optimization of the dose and duration of TPO-RAs in addition to corticosteroids is necessary to improve the management of patients with pITP.
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Consenso , Púrpura Trombocitopênica Idiopática , Humanos , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/terapia , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Adulto , Técnica Delphi , Gerenciamento Clínico , Inquéritos e Questionários , Turquia/epidemiologia , Esplenectomia , Corticosteroides/uso terapêutico , Feminino , Guias de Prática Clínica como AssuntoRESUMO
OBJECTIVE: Fertility preservation stands before us as an issue of quality of life for cancer patients and their partners and families. Therefore, the object of the present study was to determine the extent of the knowledge that hematologists have about fertility preservation and to understand their attitudes and practices regarding this matter. MATERIALS AND METHODS: A total of 25 hematologists participated in a survey. The questionnaire included questions on sociodemographic characteristics and awareness concerning the subject of fertility preservation, as well as questions designed to determine the extent of the knowledge that hematologists had on the subject and to understand their attitudes and practices in this context. RESULTS: Of the participants in the study, all expressed their awareness of the adverse effects that the various treatments they were prescribing could have on fertility; 2 (8%) revealed that they had never heard of the concept of fertility preservation. Of the participants, 19 (76%) indicated that they did not have adequate knowledge about fertility preservation, but 22 (88%) fortunately expressed a need for acquiring more knowledge about the subject. Of the respondents, 23 (92%) said that they did not have any brochures or published resources on this subject and stated their belief that if hematologists did have such documents, they would have more opportunity to discuss the various fertility preservation options with patients. All of the participants in the survey supported the idea of the Turkish Society of Hematology publishing a guidebook on this subject and organizing a session on fertility preservation in their regular congress. CONCLUSION: Meeting the needs of hematologists for training and knowledge in the subject of fertility preservation and ensuring the development of appropriate attitudes and practices in this area is an important issue. The Turkish Society of Hematology may play a significant key role. CONFLICT OF INTEREST: None declared.