RESUMO
Current treatment modalities can cure up to 70-80 % of patients with classical Hodgkin lymphoma. Approximately, 20-30 % of patients require further treatment options. Brentuximab vedotin has been approved for the treatment of relapsed and refractory Hodgkin lymphoma. In the present study, we report the experience with brentuximab vedotin as single agent in 58 patients with relapsed or refractory Hodgkin lymphoma. The objective response rate was 63.5 % with 13 complete responders (26.5 %) among 49 patients evaluated at the early phase of treatment (2-5 cycles). Upon treatment prolongation (≥6 cycles), 37 patients achieved a final objective response rate of 32.4 % with 21.6 % of complete and 10.8 % of partial response. Overall survival at 12 months was 70.6 %, and progression-free survival at 12 months was 32.8 %. Median overall survival could not be reached and median progression-free survival was 7 months. While the median duration of response was 9 months in the whole cohort, it was 11.5 months in the complete responders. Complete response rates in patients treated with >3 chemotherapy regimens before brentuximab vedotin were significantly lower (p = 0.016). Fourteen patients were subsequently transplanted. In conclusion, brentuximab vedotin provided a bridge to transplantation in approximately one quarter of the patients. The declining response rates during the course of treatment suggest that transplantation should be implemented early during brentuximab vedotin treatment.
Assuntos
Resistencia a Medicamentos Antineoplásicos , Doença de Hodgkin/tratamento farmacológico , Imunoconjugados/uso terapêutico , Adolescente , Adulto , Brentuximab Vedotin , Resistencia a Medicamentos Antineoplásicos/efeitos dos fármacos , Feminino , Doença de Hodgkin/epidemiologia , Doença de Hodgkin/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Resultado do Tratamento , Turquia , Adulto JovemRESUMO
PURPOSE: Metabolic syndrome (MetS) is considered as a proinflammatory and prothrombotic state with atherogenic risk factors including dyslipidemia, obesity and glucose intolerance. Oxidative stress is a unifying basis of several disorders including diabetes mellitus (DM) and MetS. We therefore designed this cross-sectional study to investigate the potential interaction among iron metabolism, inflammation and endothelial plexus in MetS and DM patients. METHODS: A total of 62 patients [median age 54 (23-76) years; male/female 16/46] and 18 healthy controls [median age 38 (30-64) years; male/female 6/12] were included in the study. Patient population was classified as MetS (n = 30) and DM (n = 32). RESULTS: Leukocyte count (p = 0.002) and osteopontin (OPN) levels (p = 0.008) were significantly higher, while C-reactive protein (CRP) (p = 0.056) and IL-6 (p = 0.059) represented a relative increase in the patient group. Leptin, endothelin 1 (ET1), hepcidin, nitric oxide synthase (NOS), erythrocyte sedimentation rate (ESR), iron, transferrin saturation (TS) and ferritin levels were not significantly different between the patient and control groups. Endothelin 1 was found to be higher in the DM group compared to MetS group (p = 0.15, p = 0.049). Leukocyte count, leptin, hepcidin, OPN, NOS, IL-6, ESR, CRP, iron, TS and ferritin levels were not different between DM and MetS groups. A positive correlation was demonstrated between leptin and OPN (p = 0.001, r = 0.360), ferritin and hepcidin (p < 0.01, r = 0.633), IL-6 and CRP (p = 0.023, r = 0.319), leptin and NOS (p = 0.005, r = 0.309) and OPN and NOS (p < 0.001, r = 0.803). There was a negative correlation between hepcidin and NOS (p = 0.009, r = -0.289). When the study cohort was divided into two particular groups based on median ferritin and hepcidin levels, hepcidin (p = 0.002), ALT (p = 0.001) and LDL (p = 0.049) levels were higher in the high-ferritin group. Nitric oxide synthase levels (p = 0.033) were lower, whereas ferritin levels (p = 0.004) were higher in the high-hepcidin group. CONCLUSION: Mechanisms involved in the vicious circle of MetS including inflammation, endothelial vasculature and iron metabolism remain to be elucidated. The role of iron metabolism in this complex interaction should be confirmed with further studies.
Assuntos
Diabetes Mellitus/metabolismo , Endotélio Vascular/metabolismo , Ferro/metabolismo , Síndrome Metabólica/metabolismo , Estresse Oxidativo/fisiologia , Adulto , Idoso , Proteína C-Reativa/metabolismo , Estudos Transversais , Feminino , Ferritinas/sangue , Intolerância à Glucose/sangue , Hepcidinas/sangue , Humanos , Inflamação/metabolismo , Interleucina-6/sangue , Leptina/sangue , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
AIM: Pulmonary function tests (PFT) have an important role in the assessment of pulmonary and nonpulmonary complications of hematopoetic stem cell transplantation (HSCT). In this study the relationship between PFTs and DLCOadj values and the complications of HSCT was investigated. The possible role of iron overload in the deterioration of the PFTs after HSCT was also searched. METHODS: One hundred and fifty one patients who had undergone allogeneic HSCT between years 2003 through 2008, and had the records of PFTs prior to and at 1, 3, 6, 9 and 12 months after transplantation were included in the study. Prospectively collected data of these patients were analysed retrospectively. RESULTS: Although no significant difference was identified in other PFT parameters, a significant decrease in DLCOadj was determined after 1st and 3rd months of HSCT. A significant correlation was found between pretransplant DLCOadj value <%70 and sinusoidal obstruction syndrome (SOS) (P=0.001, r=0.323), but in multivariate analysis pretransplant DLCOadj was not an independent predictor of SOS; only total body irradiation (TBI) (OR: 3.673, %95 CI: 0.880-15.804), the day of platelet engraftment (OR=1.093, %95 CI: 1.029-1.161) and serum ferritin (OR=1.001, %95 CI: 1.000-1.001) were significant. Advancing age and serum ferritin levels >600 ng/mL were the independent risk factors for pretransplant DLCOadj <%70 (OR: 0.970, %95 CI: 0.941-0.999 and OR: 2.355, %95 CI: 1.058-5.241 respectively). CONCLUSION: Although a significant correlation exists between pretransplant DLCOadj values and post-transplant SOS development, pretransplant DLCOadj was not an independent predictor of SOS. Increased serum ferritin levels were common both for pretransplant DLCO decrease and post-transplant SOS development. Iron induced endothelial damage may be the common pathophysiologic mechanism causing lung and liver vulnerability, and DLCOadj may be a non-invasive method of demonstrating this vulnerability.
Assuntos
Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Hepatopatia Veno-Oclusiva/etiologia , Sobrecarga de Ferro/complicações , Testes de Função Respiratória , Adulto , Fatores Etários , Feminino , Ferritinas/sangue , Doença Enxerto-Hospedeiro/prevenção & controle , Transplante de Células-Tronco Hematopoéticas/mortalidade , Hepatopatia Veno-Oclusiva/diagnóstico , Humanos , Sobrecarga de Ferro/fisiopatologia , Masculino , Análise Multivariada , Capacidade de Difusão Pulmonar/fisiologia , Curva ROC , Estudos Retrospectivos , Fatores de Risco , Transplante Homólogo/efeitos adversos , Irradiação Corporal TotalRESUMO
Influenza A H1N1 virus, causing a pandemic since spring 2009, has been an important cause of morbidity and mortality worldwide. Patients with hematological malignancies and hematopoietic stem cell transplant (HCT) recipients are in a high-risk group and might require hospitalization more commonly because of H1N1 infection. Early demonstration of H1N1 influenza virus and commencing antiviral therapy promptly can be life saving particularly in immunosuppressed patients. We retrospectively reviewed the data of 10 HCT recipients who were diagnosed with influenza H1N1 infection at the Stem Cell Transplantation Unit of Gazi University Hospital in Turkey, from October through December 2009. All patients, except 1, were started empirically on oseltamivir on admission, after nasopharyngeal and oropharyngeal sampling for H1N1 virus. Four of the patients, 2 of whom developed pneumonia, required hospitalization. One of the patients with pneumonia died of respiratory failure caused by bacterial co-infection. The course of the remaining patients was uneventful. In conclusion, HCT recipients infected with H1N1 during the influenza H1N1 pandemic did not necessarily have an adverse prognosis, particularly with prompt administration of the appropriate antiviral therapy.
Assuntos
Antivirais/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Vírus da Influenza A Subtipo H1N1 , Influenza Humana/tratamento farmacológico , Influenza Humana/virologia , Oseltamivir/uso terapêutico , Adulto , Feminino , Humanos , Influenza Humana/etiologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto JovemRESUMO
Abnormal protein bands (APB) unrelated to the original monoclonal protein occasionally appear in serum immunofixation samples from patients with multiple myeloma (MM) following haematopoietic stem cell transplantation (HCT). To investigate the significance of APB, medical records and serum immunofixation patterns of 53 MM patients, who had undergone HCT (49 autologous and 4 allogeneic) at the stem cell transplantation unit of Gazi University Faculty of Medicine, were reviewed. Patients were staged according to Durie-Salmon and International staging systems (ISS) and disease response was determined according to European Bone Marrow Transplantation (EBMT) criteria. Fourteen (26.4%) of the 53 patients developed APBs after HCT. The median time for the appearance and duration of APB was 3 (range 1-24) and 5.5 (range 1.5-14) months, respectively. Probability of overall survival (OS) at the end of the follow-up was 77 and 61.4% in patients with and without APB, respectively (p = 0.334). The median duration of follow-up (767 days (range, 220-2905) vs. 726 days (range, 120-1780) p = 0.545) was not different in patients with and without APB. Probability of progression free survival (PFS) at the end of follow-up was 28.8% in patients with and 27.7% in patients without APB (p = 0.835). PFS (910 days (range 180-2905) vs. 730 days (range 90-1765) p = 0.835) was longer in patients with APB, though without statistical significance. Thus, the occurrence of APB post-transplantation is not associated with any adverse long-term consequences and does not require treatment modification.
Assuntos
Transplante de Células-Tronco Hematopoéticas/métodos , Cadeias kappa de Imunoglobulina/sangue , Cadeias lambda de Imunoglobulina/sangue , Mieloma Múltiplo/sangue , Adolescente , Adulto , Idoso , Progressão da Doença , Feminino , Seguimentos , Transplante de Células-Tronco Hematopoéticas/estatística & dados numéricos , Humanos , Imunoglobulina G/sangue , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/patologia , Mieloma Múltiplo/cirurgia , Estadiamento de Neoplasias , Modelos de Riscos Proporcionais , Fatores de Tempo , Transplante Autólogo , Transplante Homólogo , Resultado do Tratamento , Adulto JovemRESUMO
Women with thrombophilic disorders may have pregnancy complications including fetal loss (FL); however, the impact of thromboprophylaxis with low molecular weight heparin (LMWH) and previous obstetric history remains to be clarified. Our aim was to compare the effects of LMWH in three groups of women with thrombophilia who had varying numbers of prior fetal losses. We prospectively investigated the live birth rates in 66 women with thrombophilic conditions, treated with a LMWH (enoxaparin, 40 mg daily). Groups 1, 2 and 3 included patients with 0-1, 2-3 and ≥ 4 pregnancy losses, respectively. Groups 1, 2 and 3 had 90.47%, 83.78% and 50.00% live birth frequencies, respectively. Group 3 had a significantly lower chance of a live birth compared with Group 1, regardless of type of thrombophilic disorder. FL aetiology in thrombophilic patients with multiple miscarriages appears multifactorial, and thromboprophylaxis alone may not be sufficient.
Assuntos
Aborto Habitual/etiologia , Anticoagulantes/uso terapêutico , Enoxaparina/uso terapêutico , Morte Fetal/etiologia , Trombofilia/complicações , Aborto Habitual/tratamento farmacológico , Adulto , Feminino , Morte Fetal/prevenção & controle , Humanos , Nascido Vivo , Gravidez , Trombofilia/tratamento farmacológico , Adulto JovemRESUMO
BACKGROUND: Whether thalidomide induces a sensory ganglionopathy or a length-dependent axonal neuropathy is disputed. Moreover no agreement exists concerning the effects of thalidomide dosage on the clinical and electrophysiological findings. OBJECTIVE: We examined the effect of age, gender disease duration, total cumulative dose on the clinical and electrophysiologic parameters. METHODS: Fifteen patients who had previously received 100 mg/day of thalidomide for the treatment of multiple myeloma were evaluated retrospectively. Clinical findings and nerve conductions studies were evaluated using a modified total neuropathy scoring system. RESULTS: Sensory symptoms (p = 0.033, r = 0.552) and objective sensory findings (p = 0.002, r = 0.730) worsened with higher thalidomide doses. There was no effect of age, gender and disease duration, neither on clinical symptoms and objective findings, nor on electrophysiologic data. Twelve patients (80%) developed the electrophysiological findings of neuropathy. Six (40%) had pure sensory and 4 (26.6%) had sensori-motor peripheral neuropathy, while 4 (26.6%) had carpal tunnel syndrome. Sural sensory nerve action potential (SNAP) amplitudes were more prominently reduced compared to SNAPs obtained from the upper extremities. Sural SNAP amplitude showed a tendency toward reduction as the total cumulative dose, although it is not statistically significant (respectively; p = 0.187). Significantly reduced ulnar peroneal and tibial compound muscle action potential amplitudes, slow motor nerve conduction velocities of the ulnar and peroneal nerves were found in the study group compared to reference norms (p < 0.05). CONCLUSION: Our results suggest that thalidomide produces a dose dependent peripheral neuropathy, mainly localized to the peripheral nerves in a length dependent manner. The patient must be monitored closely to prevent irreversible consequences.
Assuntos
Eletrofisiologia/métodos , Imunossupressores/efeitos adversos , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Talidomida/efeitos adversos , Potenciais de Ação/efeitos dos fármacos , Adolescente , Adulto , Idoso , Eletromiografia , Feminino , Humanos , Imunossupressores/farmacologia , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/tratamento farmacológico , Condução Nervosa/efeitos dos fármacos , Estatística como Assunto , Talidomida/farmacologia , Adulto JovemRESUMO
Abnormalities in liver function tests are common in hematopoietic SCT (HSCT) recipients. We retrospectively investigated the role of liver biopsy in determining the cause of elevated liver enzymes and its impact on the management of patients in the post-HSCT setting. A total of 24 consecutive liver biopsies were obtained from 20 patients from September 2003 to December 2007. A definite histopathologic diagnosis was obtained in 91.7% of the biopsies. Iron overload (IO) was found in 75% and GVHD in 54.2% of the patients. The initial clinical diagnosis of GVHD was confirmed in 56.5% and refuted in 43.5% of the allogeneic HSCT recipients. The median number of post transplant transfusions, percent transferrin saturation and ferritin levels were found to be higher in patients who had histologically proven hepatic IO (p1=0.007, p2=0.003 and p3=0.009, respectively). Regression analysis showed a significant correlation between serum ferritin levels and histological grade of iron in the hepatocytes. Our data suggest that hepatic IO is a frequent finding in the post-HSCT setting, which contributes to hepatic dysfunction and it should be considered in the differential diagnosis, particularly in patients with high serum ferritin levels.
Assuntos
Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Sobrecarga de Ferro/etiologia , Hepatopatias/patologia , Fígado/patologia , Adulto , Antineoplásicos/uso terapêutico , Biópsia , Feminino , Doença de Hodgkin/tratamento farmacológico , Doença de Hodgkin/cirurgia , Humanos , Sobrecarga de Ferro/parasitologia , Leucemia/tratamento farmacológico , Leucemia/cirurgia , Hepatopatias/etiologia , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/tratamento farmacológico , Mieloma Múltiplo/cirurgia , Estudos Retrospectivos , Transplante Homólogo/efeitos adversos , Adulto JovemRESUMO
Increasing use of more aggressive treatment approaches in patients with hematologic malignancies leads to an increased frequency of invasive fungal infections, which is a major cause of transplant-related mortality in hematopoietic stem cell recipients. In this respect, the presence of an active fungal infection prior to transplantation may hinder subsequent hematopoietic stem cell transplantation (HSCT); which sometimes is the only curative treatment. We report here the results of 13 consecutive patients transplanted with active fungal infection. Thirteen patients (7 males and 6 females) with a median age of 34 years (range, 16-53 years) underwent 15 HSCT between September 2003 and April 2007. In this group of 15 patients, consisting of hematologic malignancies with high risk of relapse or severe aplastic anemia, 11 (73%) transplants performed in subjects with active invasive fungal infection (IFI) patients survived 30 days after transplantation. Three patients (1 patient with primary disease relapse, 1 patient with graft versus host disease [GVHD] complicated with fungal pneumonia, and 1 patient with severe sinusoidal obstruction syndrome and GVHD complicated with aspiration pneumonia) died on days +66, +74, and +62 posttransplantation, respectively, within the first 100 days of HSCT. After a median follow-up time of 306 days (range, 145-680 days), four of 13 (31%) patients with active IFI were alive and disease free. Among a population of HSCT recipients with a dismal prognosis without transplantation, performing the procedure despite active IFI saved a considerable proportion of the patients. The presence of active IFI did not seem to be an absolute contraindication for HSCT, particularly among high-risk patients in whom a treatment delay could be fatal.
Assuntos
Transplante de Células-Tronco Hematopoéticas/métodos , Micoses/cirurgia , Transplante , Adolescente , Adulto , Contraindicações , Feminino , Neoplasias Hematológicas/complicações , Neoplasias Hematológicas/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Micoses/epidemiologia , Estudos Retrospectivos , Transplante Autólogo , Transplante Homólogo , Resultado do TratamentoRESUMO
One hundred eleven patients who received 125 hematopoetic stem cell transplantations (HSCT) with myeloablative conditioning regimens were retrospectively evaluated for the development of cardiac toxicity (CT). The aims of this study were to assess the frequency of cardiac complications in patients receiving HSCT and to investigate the value of pretransplantation variables to predict posttransplantation CT. Severe grade III-IV CT was not observed in this cohort, in whom pretransplantation eligibility criteria excluded the patients with a left ventricular ejection fraction (LVEF) of 50% or less. Grade I-II CT was seen in 13.4% patients. Patients with a history of previous mediastinal radiotherapy, high doses of anthracycyclines, and a longer interval between diagnosis and treatment were found to have higher risk of developing CT. Pretransplantation ferritin levels and the type of HSCT did not seem to have an effect on posttransplantation cardiac complications. Our results indicated that CT was managable in patients with a LVEF of at least 50%.
Assuntos
Cardiopatias/etiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Sístole , Disfunção Ventricular Esquerda/etiologia , Adolescente , Adulto , Idoso , Feminino , Ferritinas/sangue , Humanos , Leucemia Mieloide Aguda/mortalidade , Leucemia Mieloide Aguda/cirurgia , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/mortalidade , Mieloma Múltiplo/cirurgia , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidade , Leucemia-Linfoma Linfoblástico de Células Precursoras/cirurgia , Estudos Retrospectivos , Fatores de Risco , Taxa de SobrevidaRESUMO
Sinusoidal obstruction syndrome (SOS) is a frequent, troubling, and potentially fatal complication of hematopoietic stem cell transplantation. Despite promising results with defibrotide (DF), no treatment has been established as standard. DF is a single-stranded polydeoxyribonucleotide, obtained from controlled depolymerization of porcine intestinal mucosal cells. It has antithrombotic, antiischemic, antiinflammatory, and thrombolytic properties without significant side effects. We retrospectively evaluated the charts of 80 consecutive patients, with 89 hematopoietic stem cell transplants for hematologic malignancies. The results of early initiation of DF treatment in 14 patients with SOS are presented in this study. Fourteen patients, 8 males and 6 females % median age 40.5 years (range, 16-46 years) were diagnosed to have SOS. Disease severity was classified as severe in 6 (42.85%), moderate in 4 (28.57%), and mild in 4 (28.57%) patients. We treated 14 patients with DF for a median of 21.5 days (range, 4-39 days). All 14 patients received DF after the diagnosis of SOS. Three patients with severe and all of the patients with mild to moderate SOS responded to treatment with complete resolution of SOS-related signs and symptoms. All patients responding to DF were alive at 100 days posttransplantation. There was no significant drug-related side effect among patients treated with DF. With an overall response rate of 78.56% and a 50% complete response rate in severe SOS cases and minimal side effects, we suggest that DF is the best available agent to treat SOS.
Assuntos
Fibrinolíticos/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Hepatopatia Veno-Oclusiva/tratamento farmacológico , Hepatopatia Veno-Oclusiva/etiologia , Polidesoxirribonucleotídeos/uso terapêutico , Adolescente , Adulto , Idoso , Incompatibilidade de Grupos Sanguíneos , Feminino , Heparina/uso terapêutico , Humanos , Leucemia/terapia , Linfoma/terapia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Transplante HomólogoRESUMO
PURPOSE: The aim of the present study was to examine the relationship between peripheral CD34+ and bone marrow CD34+ levels and the clinicopathologic characteristics and laboratory parameters of myeloproliferative disease (MPD) patients. PATIENTS AND METHODS: A total of 103 MPD patients were enrolled in this study. We examined the relationship between bone marrow CD34+ and peripheral CD34+ levels and the patients' clinicopathologic and laboratory parameters. RESULTS: There were no significant correlations between the peripheral CD34+ levels and the JAK-2 V617F mutation, thrombosis, white blood cells (WBC), lactate dehydrogenase (LDH), transferrin saturation (TS), ferritin, or bone marrow cellularity. In addition, there were no significant correlations between bone marrow CD34+ levels and the JAK-2 V617F mutation, thrombosis, WBC, LDH, TS, ferritin, or bone marrow cellularity (P > 0.05). We did not identify any significant relationship between peripheral CD34+ and bone marrow CD34+ levels (P > 0.05). However, there were significant correlations between peripheral CD34+ levels and bone marrow fibrosis (P < 0.001), between bone marrow CD34+ levels and constitutional symptoms (P < 0.05), and between bone marrow CD34+ levels and bone marrow fibrosis (P < 0.001). CONCLUSION: We did not find any significant relationship between the clinicopathologic and laboratory characteristics and peripheral and bone marrow CD34+ cells from bone marrow fibrosis patients. There was also no significant relationship between bone marrow CD34+ cells and peripheral CD34+ cells. Some peripheral CD34+ cells may originate from the spleen rather than the bone marrow, which may given us different result of some parameters.
Assuntos
Antígenos CD34/metabolismo , Transtornos Mieloproliferativos/metabolismo , Antígenos CD34/sangue , Doença Crônica , Feminino , Citometria de Fluxo , Humanos , Imuno-Histoquímica , Masculino , Transtornos Mieloproliferativos/sangueRESUMO
We report a retrospective analysis of 246 myelodysplastic syndrome (MDS) patients in the EBMT (The European Society for Blood and Marrow Transplantation) database who were transplanted for International Prognostic Scoring System (IPSS) low or intermediate-1 disease. The majority of these patients (76%) were reclassified as intermediate or higher risk according to R-IPSS. The 3-year overall survival (OS) and PFS were 58% and 54%, respectively. In a multivariate analysis, adverse risk factors for PFS were marrow blast percentage (hazard ratio (HR): 1.77, P=0.037), donor/recipient CMV serostatus (donor-/recipient+: HR: 2.02, P=0.011) and source of stem cells (marrow and non-CR: HR: 5.72, P<0.0001, marrow and CR: HR: 3.17, P=0.027). Independent risk factors for OS were disease status at time of transplant and the use of in vivo T-cell depletion (TCD). Patients who did not receive TCD and were transplanted from an unrelated donor had worse OS (HR: 4.08, P<0.0001). In conclusion, 'lower' risk MDS patients have better outcome than those with 'higher risk' after haematopoietic stem cell transplant (HSCT). Selecting the right source of stem cells, a CMV-positive donor for CMV-positive patients and using in vivo TCD results in the best outcome in these patients. More studies are needed to evaluate the role of HSCT in these patients as compared with conventional treatment.
Assuntos
Anemia Refratária com Excesso de Blastos/mortalidade , Anemia Refratária com Excesso de Blastos/terapia , Sistema de Registros , Aloenxertos , Intervalo Livre de Doença , Feminino , Seguimentos , Transplante de Células-Tronco Hematopoéticas , Humanos , Masculino , Fatores de Risco , Taxa de SobrevidaRESUMO
This corrects the article DOI: 10.1038/bmt.2016.266.
RESUMO
Allogeneic hematopoietic stem cell transplantation (allo-SCT) is a therapeutic option for adult patients with T-cell ALL (T-ALL). Meanwhile, few allo-SCT data specific to adult T-ALL have been described thus far. Specifically, the optimal myeloablative conditioning regimen is unknown. In this retrospective study, 601 patients were included. Patients received allo-SCT in CR1, CR2, CR >2 or in advanced disease in 69%, 15%, 2% and 14% of cases, respectively. With an overall follow-up of 58 months, 523 patients received a TBI-based regimen, whereas 78 patients received a chemotherapy-based regimen including IV busulfan-cyclophosphamide (IV Bu-Cy) (n=46). Unlike patients aged ⩾35 years, patients aged <35 years who received a TBI-based regimen displayed an improved outcome compared with patients who received a chemotherapy-based regimen (5-year leukemia-free survival (LFS) of 50% for TBI versus 18% for chemo-only regimen or IV Bu-Cy regimens, P=10(-5) and 10(-4), respectively). In multivariate analysis, use of TBI was associated with an improved LFS (hazard ratio (HR)=0.55 (0.34-0.86), P=0.01) and overall survival (HR=0.54 (0.34-0.87), P=0.01) in patients aged <35 years. In conclusion, younger adult patients with T-ALL entitled to receive a myeloablative allo-SCT may benefit from TBI-based regimens.
Assuntos
Bussulfano/administração & dosagem , Ciclofosfamida/administração & dosagem , Transplante de Células-Tronco Hematopoéticas , Sistema de Registros , Condicionamento Pré-Transplante/métodos , Irradiação Corporal Total , Adulto , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Masculino , Leucemia-Linfoma Linfoblástico de Células T Precursoras/mortalidade , Leucemia-Linfoma Linfoblástico de Células T Precursoras/terapia , Taxa de SobrevidaRESUMO
Little is known about the prognostic impact of prior paclitaxel therapy and response to induction chemotherapy defined as the regimen preceding high-dose chemotherapy (HDCT) for the salvage therapy of advanced germ cell tumors. Twenty European Society for Blood and Marrow Transplantation centers contributed data on patients treated between 2002 and 2012. Paclitaxel used in either prior lines of therapy or in induction-mobilization regimens was considered. Multivariable Cox analyses of prespecified factors were undertaken on PFS and overall survival (OS). As of October 2013, data for 324 patients had been contributed to this study. One hundred and ninety-two patients (59.3%) had received paclitaxel. Sixty-one patients (19%) had a progression to induction chemotherapy, 234 (72%) a response (29 (9%) missing or granulocyte colony-stimulating factor without chemotherapy). Both progression to induction chemotherapy and prior paclitaxel were significantly associated with shorter OS univariably (P<0.001 and P=0.032). On multivariable analysis from the model with fully available data (N=216) progression to induction was significantly prognostic for PFS and OS (P=0.003), but prior paclitaxel was not (P=0.674 and P=0.739). These results were confirmed after multiple imputation of missing data. Progression to induction chemotherapy could be demonstrated as an independent prognostic factor, in contrast to prior paclitaxel.
Assuntos
Quimioterapia de Indução , Neoplasias Embrionárias de Células Germinativas/mortalidade , Neoplasias Embrionárias de Células Germinativas/terapia , Paclitaxel/administração & dosagem , Terapia de Salvação , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Taxa de Sobrevida , Adulto JovemRESUMO
The objective of this registry study was to analyse the outcome of patients who underwent allogeneic or autologous haematopoietic stem cell transplantation (HSCT) for hepatosplenic T-cell lymphoma (HSTL), a rare and extremely aggressive peripheral T-cell lymphoma subtype. Patients were eligible if they had histologically verified HSTL and underwent HSCT between 2003 and 2011. Seventy-six patients were identified in the European Society for Bone and Marrow Transplantation database. Additional baseline and follow-up information could be obtained from the referring centres for 36 patients. Eleven of these were excluded following histopathology review, leaving 25 patients in the final study cohort (alloHSCT 18, autoHSCT 7). With a median follow-up of 36 months, 2 patients relapsed after alloHSCT, resulting in a 3-year progression-free survival of 48%. After autoHSCT, 5 patients relapsed and subsequently died. This study indicates that graft-versus-lymphoma activity conferred by alloHSCT can result in long-term survival for a substantial proportion of patients with HSTL.
Assuntos
Transplante de Células-Tronco Hematopoéticas , Neoplasias Hepáticas/terapia , Linfoma de Células T Periférico/terapia , Sistema de Registros , Neoplasias Esplênicas/terapia , Adolescente , Adulto , Idoso , Comportamento Cooperativo , Bases de Dados Factuais , Europa (Continente) , Feminino , Seguimentos , Humanos , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/patologia , Linfoma de Células T Periférico/mortalidade , Linfoma de Células T Periférico/patologia , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/patologia , Análise de Sobrevida , Transplante Autólogo , Transplante Homólogo , Resultado do TratamentoRESUMO
Therapy-related myelodysplastic syndrome/acute myelogenous leukemia (t-MDS/AML) is extremely rare in chronic lymphocytic leukemia (CLL) despite extensive use of alkylating agents. We present a case of heavily treated CLL with resultant therapy-related refractory anemia with ringed sideroblasts (RARS). A complex cytogenetic abnormality including involvement of 3q21 was detected and to our knowledge, is the first report of a RARS case with a 3q21 abnormality.
Assuntos
Anemia Refratária/genética , Aberrações Cromossômicas , Cromossomos Humanos Par 3 , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Leucemia Mieloide Aguda/genética , Segunda Neoplasia Primária/genética , Feminino , Humanos , Leucemia Linfocítica Crônica de Células B/genética , Pessoa de Meia-IdadeRESUMO
Clonal chromosomal aberrations are reported in about 25% of the patients with hairy cell leukemia (HCL). No consistent cytogenetic abnormality has been described in HCL; most of the chromosomal changes found have been deletions and inversions, with the rare occurrence of translocations. While most of the chromosomal aberrations in HCL are common to the ones found in B cell chronic lymphocytic leukemia and other B cell lymphoproliferative disorders, there are also certain chromosomal changes that are not found in other B cell lymphoproliferative disorders. We present here a 63-year-old male patient with hairy cell leukemia with the clonal del(17)(q25), which has not previously been reported in HCL.
Assuntos
Aberrações Cromossômicas , Deleção Cromossômica , Cromossomos Humanos Par 17 , Leucemia de Células Pilosas/genética , Bandeamento Cromossômico , Humanos , Cariotipagem , Masculino , Pessoa de Meia-IdadeRESUMO
Idiopathic hypereosinophilic syndrome (HES) is a disorder of the hematopoietic system, characterized by persistent elevation in the total eosinophil count (> 1500/microliter) for over 6 months, associated with organ damage and no detectable underlying cause. Treatment is centered on the reduction of total circulating eosinophils, which generally leads to remission of symptoms. We report a 68-year-old female patient with HES and peripheral neuropathy, presenting with cutaneous lesions, mental changes, cardiac and pulmonary symptoms, followed by right foot drop and eventually paraparesis, which caused an inability to ambulate. Weakness progressed to include the upper extremities despite adequate control of eosinophilia by steroids. Worsening of the peripheral neuropathy can occur despite lowering of the eosinophil levels.