RESUMO
BACKGROUND: Since well-designed prospective comparative trials are lacking, haploidentical hematopoietic stem cell transplantations approach should be based on the expertise of a particular center. In this study, we aimed to report the results and outcomes of patients who underwent haploidentical hematopoietic stem cell transplantation. METHODS: : Thirty-nine patients who underwent transplantation in our clinic between 2015 and 2022 were retrospectively analyzed. Primary end point of this study is to find out the survival rates of the patients. RESULTS: The overall survival of patients was 29.9 ± 4.9 months. The disease-free survival of the patients was 37.8 ± 5.7 months. The 3-year overall survival rate of the patients was %50 and the 3-year disease-free survival rate of the patients was %53. Nineteen patients were nonsurvivors among a total of 39 patients. Busulfan-fludarabine-thiotepa was the most frequently used conditioning regimen for transplantation. Busulfan-fludarabin-antithymocyte globulin regimen is the second preferred conditioning regimen. Cyclosporine- cyclophosphamide-mycophenolate mofetil was the most widely used graft-versus-host disease prophylaxis regimen. Sixteen patients had graft-versus-host disease, 28% of the patients had acute graft-versus-host disease, and 13% had chronic graft-versus-host disease. Gastrointestinal system consists of the most involved organs in graft-versus-host disease since 15% of the patients had gastrointestinal graft-versus-host disease. First-degree relatives (parent/child) were the most frequent donor source for haploidentical hematopoietic stem cell transplantation. Sepsis was the most frequent reason of death among transplant patients. DISCUSSION: In our center, we prefer to use high dose posttransplantation cyclophosphamide after haploidentical hematopoietic stem cell transplantation for graft-versus-host disease prophylaxis. With this approach, our center's overall survival and disease-free survival rates are comparable and compatible with the literature findings.
Assuntos
Doença Enxerto-Hospedeiro , Neoplasias Hematológicas , Transplante de Células-Tronco Hematopoéticas , Criança , Humanos , Bussulfano/uso terapêutico , Estudos Retrospectivos , Estudos Prospectivos , Transplante de Células-Tronco Hematopoéticas/métodos , Ciclofosfamida/uso terapêutico , Ciclosporina/uso terapêutico , Doença Enxerto-Hospedeiro/prevenção & controle , Ácido Micofenólico/uso terapêutico , Neoplasias Hematológicas/terapiaRESUMO
BACKGROUND: Thrombocytopenia is a common complication following hematopoietic stem cell transplantation (HSCT). Eltrombopag has been used in thrombocytopenia treatment after HSCT in recent years. Herein, we present our experience of 25 patients treated with eltrombopag for post-HSCT thrombocytopenia. METHODS: Fifteen autologous hematopoietic stem cell transplantation (AHSCT) and 10 allogenic hematopoietic stem cell transplantation (allo-HSCT) recipients treated with eltrombopag for treatment of prolonged isolated thrombocytopenia (PIT) or secondary failure of platelet recovery (SFPR) in the stem cell transplantation unit of Hacettepe University Hematology Department between 2017 and 2021 were included in the study. The primary endpoint of this study is eltrombopag response in patients diagnosed with PIT or SFPR. Platelet count above 50,000/mm3 for five consecutive days without platelet transfusion was considered as eltrombopag response. Overall survival (OS) analyses were calculated based on the time between HSCT and death from any cause. The patients who were alive at the last follow-up were censored at this time for calculation of OS analyses. RESULTS: AHSCT (66.7% (10/15)) and allo-HSCT (50% (5/10)) recipients responded to eltrombopag for the treatment of post-HSCT thrombocytopenia. There was no excess toxicity related to the eltrombopag use. The median response duration of allo-HSCT recipients and AHSCT recipients were 41 (13-104) days and 50 (7-342) days, respectively. There was a statistically significant OS duration difference between the responders and nonresponders in allo-HSCT and AHSCT recipients with p values of 0.005 and 0.02, respectively. DISCUSSION: Eltrombopag is promising for the treatment of thrombocytopenia after AHSCT and allo-HSCT in terms of efficacy and safety.
Assuntos
Transplante de Células-Tronco Hematopoéticas , Trombocitopenia , Benzoatos/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Hidrazinas/uso terapêutico , Pirazóis , Estudos Retrospectivos , Trombocitopenia/tratamento farmacológico , Trombocitopenia/etiologiaRESUMO
OBJECTIVE: Aplastic anemia (AA) is a life-threatening disorder and may be associated with significant morbidity and mortality Currently, the first treatment option is allogeneic hematopoietic stem cell transplant (allo-HSCT) for patients younger than 40 years. Bone marrow is recommended as the stem cell source due to less graft versus host disease (GVHD) risk and better outcomes than peripheral blood (PB)-derived stem cell. The aim of this study is to share the data of AA patients who have underwent PB-derived allo-HSCT in our bone marrow transplantation center. METHODS: Twenty-seven patients who underwent PB-derived allo-HSCT from human leukocyte antigen matched sibling donors were analyzed retrospectively. RESULTS: The median follow-up time was 95.2 months (range, 4.8-235 months). The 10-year survival was 89 %. The median neutrophil and platelet engraftment time was 11 days (range, 9-16 days) and 13 days (range, 11-29 days), respectively. Primary platelet engraftment failure was observed in 1 patient (3.7 %). Acute and chronic GVHD observed in 2 (7.4 %) and 3 (11.1 %) patients, respectively. Neutropenic fever was observed in 13 (44.8 %) of patients until the engraftment after allo-HSCT. One patient died due to CMV infections, two died due to septic shock secondary to fungal infection. CONCLUSION: Although there is no prospective data directly comparing BM with PB as stem cell source in AA, observational studies indicates better OS with BM. PB can be used in certain situations such as higher risk for graft failure and donor preference. This study demonstrated that PB-derived stem cell seems to be a reasonable alternative to BM.
Assuntos
Anemia Aplástica/terapia , Transplante de Células-Tronco Hematopoéticas/métodos , Transplante de Células-Tronco de Sangue Periférico/métodos , Adolescente , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto JovemRESUMO
Background/aim: The disease caused by SARS-CoV-2 was named as COVID-19. There is as yet insufficient information about the effects of HSCT on the clinical course of COVID-19. In the present study, we aimed to investigate the clinical course of COVID-19 in patients who had undergone HSCT. Materials and methods: We analyzed baseline characteristics, clinical course and findings of COVID-19, hospitalization and death rates, overall survival, and case fatality rates of HSCT recipients diagnosed with COVID-19 retrospectively. Results: 57.6% of the patients underwent AHSCT, and 42.4% underwent allo-HSCT. 60.6%, 27.3%, and 12.1% of the patients had mild, moderate, and severe COVID-19 or critical illness, respectively. Overall, 45.5% were hospitalized, 12.1% required intensive care, and 9.1% necessitated invasive mechanical ventilation. The total CFR was 9.1% in HSCT recipients, 22.2% in patients with active hematologic malignancy, and 4.2% in patients without active hematologic malignancy. Conclusion: It can be concluded that mortality of HSCT recipients is lower in patients whose primary disease is in remission compared to ones that are not in remission. Further studies with larger group patients are needed in order to delineate the effects of COVID-19 on HSCT patients.
Assuntos
COVID-19/mortalidade , COVID-19/fisiopatologia , Transplante de Células-Tronco Hematopoéticas/mortalidade , Hospitalização/estatística & dados numéricos , Transplantados/estatística & dados numéricos , Adulto , Idoso , COVID-19/terapia , Feminino , Transplante de Células-Tronco Hematopoéticas/estatística & dados numéricos , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , SARS-CoV-2 , Índice de Gravidade de Doença , Turquia/epidemiologiaRESUMO
BACKGROUND AND AIM: High-dose chemotherapy followed by autologous stem cell transplantation (ASCT) has been considered the standard of treatment care for patients with multiple myeloma (MM). Insufficient mobilization and harvest of peripheral stem cells can be a major obstacle for performing ASCT. This is resulting in a lacking opportunity of cure in patients with MM. The aim of this study was to evaluate the factors which influence mobilization failure in patients with MM. MATERIALS AND METHODS: This study has been performed in a retrospective manner. Two hundred and thirty-four patients with diagnosed MM who underwent stem cell mobilization after induction chemotherapy at Hacettepe University Hospital between the years of 2003 and 2018 were evaluated. RESULTS: A total of 234 patients were included in this study. The median age was 54 (32-76) years at the time of diagnosis. In 209 of 234 patients (89.3%) first mobilization trial was successful. At univariate analysis, among parameters identifiable before mobilization, male gender (pâ¯=â¯0.03), number of chemotherapy cycle before stem cell mobilization (pâ¯<â¯0.001), second ASCT (pâ¯<â¯0.001) and immunomodulatory treatment before stem cell mobilization (pâ¯<â¯0.001) predicted mobilization failure. At multivariate analysis, number of chemotherapy cycle before stem cell mobilization (pâ¯=â¯0.03), second ASCT (pâ¯<â¯0.001) and immunomodulatory treatment before stem cell mobilization (pâ¯=â¯0.02) retained independent predictive power. CONCLUSION: Detectable different clinical characteristics of MM patients before initiating mobilization may be predictors of poor mobilization. Therefore, the mobilization protocol should be evaluated on a patient basis. Minimization of exposure to chemotheraputic agents in MM patients, especially immunomodulatory agents, may increase CD34+ cell harvest yields.
Assuntos
Mobilização de Células-Tronco Hematopoéticas/métodos , Mieloma Múltiplo/terapia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
Acute respiratory syndrome coronavirus 2 (SARS-CoV-2) first identified in Wuhan, China; and spread all over the world. Reverse-transcription polymerase chain reaction (RT-PCR) test for SARS-CoV-2 usually returns to negative in 20 days post-infection, but prolonged positivity has been reported up to 63 days. A case whose viral shedding lasted 60 days is reported from China. Herein we report a patient with a history of autologous stem cell transplantation (ASCT) for lymphoma whose RT-PCR test remained positive for SARS-CoV-2 for 74 days. The prolonged RT-PCR positivity, despite convalescent plasma infusion, may suggest that the given antibodies may be ineffective in terms of viral clearance. In patients with hematological malignancies or immunosuppression, such as ASCT, may lead to prolonged viral shedding, and strict isolation is warranted for long-term SARS-CoV-2 infection control.
Assuntos
COVID-19/terapia , COVID-19/virologia , Linfoma/virologia , SARS-CoV-2/fisiologia , Eliminação de Partículas Virais/fisiologia , Humanos , Imunização Passiva , Masculino , Pessoa de Meia-Idade , Soroterapia para COVID-19RESUMO
During the ongoing COVID-19 pandemic due to the SARS-CoV-2 virus of which evidence-based medical paradigms cannot be easily applied; difficult clinical decisions shall be required particularly in the 'difficult-to-treat' cases of high risk group with associated comorbidities. Convalescent immune plasma therapy is a promising option as a sort of 'rescue' treatment in COVID-19 immune syndrome, where miraculous antiviral drugs are not available yet. In this report, we aim to convey our experience of multi-task treatment approach with convalescent immune plasma and anti-cytokine drug combination in a COVID-19 patient with extremely challenging comorbidities including active myeloid malignancy, disseminated tuberculosis and kidney failure.
Assuntos
COVID-19/complicações , COVID-19/terapia , Síndromes Mielodisplásicas/complicações , Síndromes Mielodisplásicas/virologia , Tuberculose/complicações , Tuberculose/virologia , Temperatura Corporal , COVID-19/diagnóstico por imagem , COVID-19/imunologia , Humanos , Imunização Passiva , Contagem de Linfócitos , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/diagnóstico por imagem , SARS-CoV-2/fisiologia , Tomografia Computadorizada por Raios X , Tuberculose/diagnóstico por imagem , Soroterapia para COVID-19RESUMO
Background and Aim: Recently, acute promyelocytic leukemia (APL) has shifted from the most hazardous to the best curable type of acute myeloid leukemia. Anthracyclines, all-trans retinoic acid (ATRA) and arsenic derivatives are the most important developments for the treatment of APL. ATRA promotes the terminal differentiation of malignant promyelocytes to mature neutrophils. We aimed to compare platelet and neutrophil recovery time after induction chemotherapy in patients with acute myeloid leukemia (AML) and APL.Materials and Methods: Two hundred and fifteen patients with AML and APL, who were diagnosed and treated in our tertiary care center between the years of 2001 and 2018 were evaluated.Results: One hundred and eighty one AML patients (84.2%) and 34 (15.8%) APL patients were included in this study. The time between neutrophil nadir after induction chemotherapy and neutrophil recovery was longer in APL patients than in AML patients [30.5 (4-52) vs. 20 (5-58), p < 0.001]. The time between platelet nadir after induction chemotherapy and platelet recovery was longer in APL patients than in AML patients [21.5 (4-42) vs. 17 (4-45), p = 0.02].Conclusion: Neutrophil and platelet recovery times were longer in APL patients than in AML patients in our present study. In 60 days, mortality rate was higher in APL patients than AML patients. Non-relapse mortality (NRM) rate was similar between two groups. There was a significant difference between two groups in terms of NRM causes. Platelet and neutrophil recovery time is very important because infection is the most important cause of NRM.
Assuntos
Quimioterapia de Indução , Leucemia Promielocítica Aguda , Adolescente , Adulto , Idoso , Células da Medula Óssea , Feminino , Humanos , Cinética , Leucemia Promielocítica Aguda/tratamento farmacológico , Leucemia Promielocítica Aguda/metabolismo , Contagem de Leucócitos , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Estudos RetrospectivosRESUMO
Background/aim: High-dose melphalan and autologous hematopoietic stem cell transplantation (AHSCT) is the standard treatment strategy for multiple myeloma (MM) patients who are eligible for it. The recommended dose of CD34+ hematopoietic progenitor cells (HPCs) for adequate engraftment is above 2 × 106/kg. The aim of this study was to evaluate the relationship between the dose of CD34+HPCs and survival in MM patients who underwent AHSCT at a tertiary care center. Materials and methods: Enrolled in this study were 271 MM patients who underwent AHSCT between 2003 and 2019. Clinical characteristics of the patients, disease status pre-AHSCT, reinfused CD34+ cell doses, and neutrophil and platelet engraftment days were recorded, retrospectively. The patients were divided into 2 groups according to whether the dose of reinfused CD 34+ HPCs was <5 × 106/kg or ≥5 × 106/kg. The groups were compared in terms of engraftment and overall survival (OS) times. Results: The median age of the patients was 54.8 (3376) years. The median dose of infused CD34+ HPCs was 5.94 × 106/kg (1.4759.5 × 106/kg). The median follow-up period was 54 months (4211). The median OS of the patients was 103 months (11144). The median neutrophil and platelet engraftment time was 10 (824) and 11 (740) days. Doses of <5 × 106/kg and ≥5 × 106/kg CD34+ HPC were reinfused in 38.1% and 61.9% of the patients, respectively. There was a negative significant correlation between the reinfused CD34+cell level and neutrophil/platelet engraftment times (r = 0.32, P < 0.001; r = 0.27, P < 0.001, respectively). The median OS times were observed as 103 months (11144) and 145 months (123166) for patients who had been administered <5 × 106/kg and ≥5 × 106/kg of CD34+ HPCs, respectively (P = 0.009). Conclusion: The increased amount of CD34+ autologous hematopoietic stem cell dose after high dose melphalan chemotherapy in MM patients shortened the platelet and neutrophil engraftment time and increased OS. Early platelet engraftment and administration of a CD34+ HPC count that is ≥5 × 106/kg can be considered as predictors of better survival in patients.
Assuntos
Antineoplásicos Alquilantes/uso terapêutico , Sobrevivência de Enxerto/fisiologia , Transplante de Células-Tronco Hematopoéticas/métodos , Células-Tronco Hematopoéticas , Melfalan/uso terapêutico , Mieloma Múltiplo/terapia , Adulto , Idoso , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Transplante AutólogoRESUMO
Background/aim: COVID-19 (Coronavirus disease of 2019) is an infectious disease outbreak later on declared as a pandemic, caused by the SARS-CoV-2 (severe acute respiratory syndrome coronavirus-2). It spreads very rapidly and can result in severe acute respiratory failure. The clinical studies have shown that advanced age and chronic diseases increase the risk of infection. However, influence of the blood groups on COVID-19 infection and its outcome remains to be confirmed. The aim of this study is to investigate whether there exists a relationship between the blood groups of the patients and risk of SARS-CoV-2 infection and the clinical outcomes in COVID-19 patients Material and method: 186 patients with PCR confirmed diagnosis of COVID-19 were included in this study. Age, sex, blood groups, comorbidities, need for intubation and intensive care unit follow up and mortalities of the patients were analyzed retrospectively. 1881 healthy individuals, who presented to the Hacettepe University Blood Bank served as the controls. Results: The most frequently detected blood group was blood group A (57%) amongst the COVID-19 patients. This was followed by blood group O (24.8%). The blood group types did not affect the clinical outcomes. The blood group A was statistically significantly more frequent among those infected with COVID-19 compared to controls (57% vs. 38%, P < 0.001; OR: 2.1). On the other hand, the frequency of blood group O was significantly lower in the COVID-19 patients, compared to the control group (24.8% vs. 37.2%, P: 0.001; OR: 1.8). Conclusions: The results of the present study suggest that while the blood group A might have a role in increased susceptibility to the COVID-19 infection, the blood group O might be somewhat protective. However, once infected, blood group type does not seem to influence clinical outcome.
Assuntos
Sistema ABO de Grupos Sanguíneos , COVID-19/sangue , COVID-19/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , COVID-19/mortalidade , COVID-19/terapia , Estudos de Casos e Controles , Feminino , Humanos , Unidades de Terapia Intensiva/estatística & dados numéricos , Intubação Intratraqueal/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Prognóstico , Respiração Artificial/estatística & dados numéricos , Estudos Retrospectivos , Fatores de Risco , SARS-CoV-2 , Índice de Gravidade de Doença , Adulto JovemRESUMO
Background/aim: Ruxolitinib, a JAK/STAT signaling pathway inhibitor targeted drug, has been approved for the controlling of disease symptoms and splenomegaly in patients with myeloproliferative neoplastic diseases. Recently, it has been proposed that ruxolitinib-induced JAK/STAT pathway inhibition in myelofibrosis is associated with an elevated frequency of aggressive B-cell lymphomas. However, the biological basis and significance of this pharmacobiological adverse event is unknown. The aim of this bioinformatics study is to detect any possible confounding effects of ruxolitinib on the genesis of lymphoproliferative disorders. Materials and methods: The gene expression data were retrieved from the E-MTAB-783 Cancer Genome Project database. Gene expression data for all available genes in 26 cell lines belonging to various types of lymphomas were chosen for use in this in silico analysis. Results: We identified genes that were significant in developing resistance to ruxolitinib in lymphoma cell lines. Conclusion: Based on the results of our present study, ruxolitinib may potentially lead to the pathological expression of the transcription factors important in lymphoma genesis, neoplastic commitment on the progenitor lymphoid cells, inhibition of repressor transcriptions protective for lymphoma development, inhibition of apoptosis, promotion of neoplastic proliferation, transcriptional activation, and proliferation of malignant neoplastic B cells.
Assuntos
Regulação Neoplásica da Expressão Gênica/efeitos dos fármacos , Inibidores de Janus Quinases/efeitos adversos , Transtornos Linfoproliferativos/induzido quimicamente , Transtornos Linfoproliferativos/genética , Pirazóis/efeitos adversos , Transdução de Sinais/efeitos dos fármacos , Apoptose , Linhagem Celular Tumoral , Simulação por Computador , Humanos , Janus Quinase 1 , Janus Quinase 2 , Inibidores de Janus Quinases/farmacologia , Nitrilas , Pirazóis/farmacologia , PirimidinasRESUMO
Background/aim: Polycythemia Vera (PV) is a myeloproliferative disorder characterized by overproduction of morphologically normal red blood cells (RBCs), granulocytes, and platelets, a phenotype that is caused by a mutation (V617F) in Janus kinase 2 (JAK2). However, JAK2 V617F is also found in approximately 50% of patients with essential thrombocytosis and primary myelofibrosis, rendering its presence nonspecific as a diagnostic test. An increased red cell mass is a major criterion for the diagnosis of PV according to World Health Organization (WHO) 2016 criteria. High hemoglobin (Hgb) or Hematocrit (Hct) are universally used as indicators of an increased red cell mass for the diagnosis of PV. However, conditions such as iron deficiency (ID) with decreased mean cell volume may mask the diagnosis due to nonelevated Hct level. The aim of this study was to investigate the clinical characteristics of the patients with unclassifiable non-CML classical myeloproliferative disease with microcytosis (MPD/M) and nonelevated Hgb and Hct levels at diagnosis and to determine if some of these cases could be real PV cases masked due to ID-related microcytosis. Materials and methods: There were 23 MPD/M cases among 208 non-CML classical MPD cases (11%). Among 22 patients who had adequate test results related to the cause of microcytosis, ID and beta-thalassemia trait (TT) were the apparent causes of microcytosis in 15 and 1 cases, respectively. Results: Clinicopathological correlations revealed consistently positive JAK2 V617F mutation status (20/20, 100%), frequently elevated RBC count (17/23, 73.9%), and PV-compatible bone marrow findings (10/12, 83.3%). These findings are compatible with PV instead of essential thrombocytopenia or primary myelofibrosis. In spite of frequent cytoreductive treatment, 3 patients developed increased Hgb/Htc levels during median 58.2 (27963) months' follow-up. Conclusion: These data show that the majority of MPD/M cases are PV patients masked due to ID-related microcytosis.
Assuntos
Eritrócitos Anormais , Deficiências de Ferro , Distúrbios do Metabolismo do Ferro/sangue , Distúrbios do Metabolismo do Ferro/diagnóstico , Transtornos Mieloproliferativos/sangue , Policitemia Vera/sangue , Policitemia Vera/diagnóstico , Adulto , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
Background/aim: High-dose chemotherapy followed by autologous stem cell transplantation (ASCT) has become the standard approach for patients with relapsed/refractory Hodgkin's lymphoma (HL) or non-Hodgkin's lymphoma (NHL). In this study, we report the outcome of the mitoxantrone-melphalan conditioning regimen for lymphoma. Materials and methods: The study group included 53 patients who were relapsed/refractory HL (n = 14) and NHL (n = 39) and received mitoxantrone and melphalan followed by ASCT. The transplant regimen consisted of mitoxantrone (60 mg/m2) and melphalan (180 mg/m2) followed by peripheral blood stem cell infusion (PBSC). Results: Prior to high-dose chemotherapy, 37.7% of the patients were in complete remission (CR) and 45.3% were in partial remission (PR), and 17% had stable or progressive disease. After high-dose chemotherapy and PBSC, 44 out of 51 patients achieved CR (86.2%). CR was achieved in 24 out of 33 patients (72.7%) who were transplanted in a marginally active phase of the disease. At a median followup of 25.4 months (1.8131.3 months) after ASCT, 13 patients relapsed/ progressed and 8 patients died. The estimated 2-year overall survival (OS) was 81.9%, and event-free survival (EFS) was 59.3%. Conclusion: High-dose chemotherapy followed by ASCT is an effective conditioning regimen in relapsed/refractory lymphoma patients who are undergoing ASCT.
Assuntos
Antineoplásicos/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/métodos , Linfoma , Melfalan/uso terapêutico , Mitoxantrona/uso terapêutico , Adolescente , Adulto , Idoso , Feminino , Humanos , Linfoma/tratamento farmacológico , Linfoma/epidemiologia , Linfoma/patologia , Linfoma/cirurgia , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND AND AIM: This is a retrospective study aiming to investigate the effect of the number of high dose cytarabine-based chemotherapy (HiDAC) courses in patients with acute myeloid leukemia before allogenic stem cell transplantation (ASCT). MATERIALS AND METHODS: A total of 110 patients with acute myeloid leukemia who received ASCT between 2001 and 2018 were included in the study. RESULTS: Of the 110 patients, 25 (23%) patients received one course of HiDAC, 42 (38%) patients received two courses of HiDAC, 34 (31%) patients received three courses of HiDAC and 9 (8%) patients received four courses of HiDAC. Median follow-up for survivors was 71 months (range 4-186) for all patients. The 3-year overall survival for patients who received one course of HiDAC and patients who received more than one course of HiDAC were 49% and 70%, respectively (p = 0.29). The 3-year disease free survival (DFS) for patients who received one course of HiDAC and patients who received more than one course of HiDAC were 38% and 66%, respectively (p = 0.05). There was no statistically significant difference in OS between patients who received one or more than one consolidation chemotherapy. But there was nearly a statistically significant difference between patients who received one or more than one consolidation chemotherapy in DFS. CONCLUSION: In conclusion, the administration of more than one consolidation chemotherapy may provide longer DFS, however the number of consolidation chemotherapy is not associated with statistically significant differences in overall outcomes.
Assuntos
Quimioterapia de Consolidação , Citarabina/uso terapêutico , Leucemia Mieloide Aguda/tratamento farmacológico , Transplante de Células-Tronco , Adulto , Idoso , Citarabina/farmacologia , Intervalo Livre de Doença , Relação Dose-Resposta a Droga , Feminino , Humanos , Leucemia Mieloide Aguda/patologia , Masculino , Pessoa de Meia-Idade , Indução de Remissão , Transplante Homólogo , Resultado do Tratamento , Adulto JovemRESUMO
There are very few data about the relationship between acute myeloid leukemia (AML) prognosis and bone marrow recovery kinetics following chemotherapy. In this study, we aimed to assess the prognostic importance and clinical associations of neutrophil and platelet recovery rates and rebound thrombocytosis (RT) or neutrophilia (RN) in the postchemotherapy period for newly diagnosed AML patients. De novo AML patients diagnosed between October 2002 and December 2013 were evaluated retrospectively. One hundred patients were suitable for inclusion. Cox regression analysis using need for reinduction chemotherapy as a stratification parameter revealed RT as the only parameter predictive of OS, with borderline statistical significance (p = 0.06, OR = 7; 95% CI 0.92-53), and it was the only parameter predictive of DFS (p = 0.024, OR = 10; 95% CI 1.3-75). In order to understand whether RT or RN was related to a better marrow capacity or late consolidation, we considered neutrophil recovery time and platelet recovery time and nadir-first consolidation durations in all patients in the cohort. Both the marrow recovery duration and the time between marrow aplasia and first consolidation were shorter in RT and RN patients. To our knowledge, this is the first study to report a correlation between RT/RN and prognosis in AML.
Assuntos
Leucemia Mieloide Aguda , Trombocitose , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Humanos , Leucemia Mieloide Aguda/sangue , Leucemia Mieloide Aguda/diagnóstico , Leucemia Mieloide Aguda/tratamento farmacológico , Contagem de Leucócitos , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Prognóstico , Indução de Remissão , Estudos Retrospectivos , Trombocitose/sangue , Trombocitose/diagnóstico , Trombocitose/tratamento farmacológicoRESUMO
BACKGROUND AND AIM: Legg-Calve-Perthes disease (LCPD) is still an enigma. Hemostatic abnormalities have been indicated in the pathogenesis. We had previously demonstrated enhanced tissue factor pathway inhibitor response, increased global fibrinolytic capacity, and an increase in thrombomodulin in patients with LCPD compared with healthy individuals. These studies emphasized the role of vascular endothelium in pathogenesis of the LCPD. P-selectin is expressed on activated platelets and endothelial cells, and E-selectin is expressed on activated endothelial cells. The aim of this study was to assess circulating E-selectin and P-selectin levels in LCPD patients, which might reflect an endothelium activation and/or injury. MATERIALS AND METHODS: The study included 85 pediatric patients. Group I consisted of 55 patients with LCPD and group II (control) consisted of 30 healthy children. Peripheral venous blood concentrations of E-selectin and P-selectin levels were measured with a commercially available assay. RESULTS: Mean age was 8.41±2.73 years in group I and 8.83±2.92 years in group II. Both E-selectin and P-selectin levels were higher in LCPD patients in comparison with the age-matched controls. E-selectin was 54.92±18.84 pg/mL in group I, 45.54±15.31 pg/mL in group II and P-selectin was 46.40±20.35 pg/mL in group I, 36.92±9.84 pg/mL in group II (P=0.022 and P=0.019, respectively). CONCLUSIONS: On the basis of our results, two important endothelium and platelet markers, E-selectin and P-selectin, are upregulated in LCPD. Our results suggested that activated platelets and possibly endothelial activation, as reflected by enhanced P-selectin/E-selectin kinetics, might contribute to the microvascular thrombosis and/or inflammation of LCPD.
Assuntos
Selectina E/sangue , Endotélio Vascular/metabolismo , Doença de Legg-Calve-Perthes/metabolismo , Selectina-P/sangue , Ativação Plaquetária/fisiologia , Adolescente , Biomarcadores/sangue , Plaquetas/metabolismo , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Microvasos/metabolismo , Solubilidade , Trombose/metabolismo , Vasculite/metabolismoRESUMO
OBJECTIVE: The prominent functions of the local renin-angiotensin system (RAS) in primitive hematopoiesis further support the hypothesis that local autocrine bone marrow RAS could also be active in neoplastic hematopoiesis. The aim of this study is to examine critical RAS elements in normal CD34+ hematopoietic stem cells and multiple myeloma (MM)-related progenitor cells. MATERIALS AND METHODS: The study group comprised the total bone marrow cells (CBM) of 10 hematologically normal people, the CD34+ stem cell samples (CD34+CBM) of 9 healthy donors for allogeneic peripheral stem cell transplantation, and the CD34+ stem cell samples (CD34+MM) of 9 MM patients undergoing autologous peripheral stem cell transplantation. We searched for the gene expression of the major RAS components in healthy hematopoietic cells and myeloma cells by quantitative real-time polymerase chain reaction analysis. RESULTS: RENIN, angiotensinogen (ANGTS), and angiotensin converting enzyme-I (ACE I) mRNA expression levels of CBM were significantly higher than those in myeloma patients (p=0.03, p=0.002, and p=0.0008, respectively). Moreover, RENIN and ANGTS mRNA expression levels were significantly higher in CD34+ stem cell samples of healthy allogeneic donors compared to those in myeloma patients (p=0.001 and p=0.01). However, ACE I expression levels were similar in CD34+CBM and CD34+MM hematopoietic cells (p=0.89). CONCLUSION: Although found to be lower than in the CBM and CD34+CBM hematopoietic cells, the local RAS components were also expressed in CD34+MM hematopoietic cells. This point should be kept in mind while focusing on the immunobiology of MM and the processing of autologous cells during the formation of transplantation treatment protocols.
RESUMO
Cardiac magnetic resonance imaging is the gold standard to detect cardiac iron overload in patients with beta-thalassemia. The aim of this study was to evaluate cardiac iron overload using four-dimensional transthoracic echocardiography in thalassemia patients with and without cardiac involvement detected by T2* value and to compare the two techniques. This cross-sectional and observational study was conducted in 44 patients diagnosed with thalassemia major. Left ventricular systolic function was assessed using four-dimensional speckle tracking echocardiography-derived global longitudinal (GLS), circumferential, radial, and area strain indices. Left ventricular ejection fraction, volumes, and mass index were similar between the patients with T2* values less than 20 ms as compared to those with T2* values greater than 20 ms. However, patients with lower T2* values had significantly higher GLS values (- 17.0% vs. - 19.8%, p < 0.001) compared with those with higher T2* values. GLS demonstrated a sensitivity of 91.7% and a specificity of 71.9% at a cut-off value of - 18.5%; however, sensitivity was 75%, and the specificity was 84.4% at a cut-off value of - 17.5%. For - 18.5%, the positive predictive value was 55%, and the negative predictive value was 95.8%; for - 17.5%, these values were 64.2 and 90%, respectively. This novel echocardiographic method, tested for the first time in our study in comparison with cardiac MRI in an adult patient group, has been shown to predict cardiac iron overload in thalassemia patients in the subclinical period without LVEF decline. Four-dimensional GLS is a marker with high sensitivity and negative predictive value.
Assuntos
Sobrecarga de Ferro , Talassemia , Disfunção Ventricular Esquerda , Talassemia beta , Adulto , Humanos , Talassemia beta/complicações , Talassemia beta/diagnóstico por imagem , Volume Sistólico , Estudos Transversais , Função Ventricular Esquerda , Sobrecarga de Ferro/diagnóstico por imagem , Sobrecarga de Ferro/etiologia , Imageamento por Ressonância Magnética/métodos , Ecocardiografia Quadridimensional , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/etiologiaRESUMO
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.
Assuntos
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
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.