RESUMO
Central nervous system (CNS) involvement is rare in primary mediastinal large B-cell lymphoma (PMLBCL). We aimed to evaluate the incidence of CNS relapse as first treatment failure event and the effect of the induction chemotherapy regimen, central nervous system - international prognostic index (CNS-IPI) and other clinical and laboratory variables on the risk of CNS relapse in 564 PMLBCL patients treated with immunochemotherapy. Only 17 patients (3.0%) received CNS prophylaxis. During a 55-month median follow-up only 8 patients experienced CNS relapse as first event, always isolated. The 2-year cumulative incidence of CNS relapse (CI-CNSR) was 1.47% and remained unchanged thereafter. The CI-CNSR was not affected by the chemotherapy regimen (R-CHOP or R-da-EPOCH). None of the established International Prognostic Index factors for aggressive lymphomas predicted CNS relapse in PMLBCL. The 2-year CI-CNSR in patients with versus without kidney involvement was 13.3% versus 0.96% (p < 0.001); 14.3% versus 1.13% with versus without adrenal involvement (p < 0.001); and 10.2% versus 0.97% with versus without either kidney or adrenal involvement. CNS-IPI was also predictive (2-year CI-CNSR in high-risk vs. intermediate/low-risk: 10.37% vs. 0.84%, p < 0.001). However, this association may be driven mainly by kidney and/or adrenal involvement. In conclusion, in PMLBCL, CNS relapse is rare and appears to be strongly associated with kidney and/or adrenal involvement.
Assuntos
Neoplasias do Sistema Nervoso Central , Linfoma de Células B , Linfoma Difuso de Grandes Células B , Humanos , Rituximab/uso terapêutico , Incidência , Neoplasias do Sistema Nervoso Central/tratamento farmacológico , Neoplasias do Sistema Nervoso Central/epidemiologia , Neoplasias do Sistema Nervoso Central/patologia , Recidiva Local de Neoplasia/patologia , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Fatores de Risco , Ciclofosfamida , Vincristina , Doxorrubicina , Doença Crônica , Sistema Nervoso Central/patologia , Linfoma de Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/patologiaRESUMO
OPINION STATEMENT: Nowadays, several novel agents have been introduced in the treatment of multiple myeloma, not only resulting in high response rates and prolonged survival but also offering good quality of life. However, the potential of cure, especially for patients with advanced or unfavorable disease features, remains elusive. Allogeneic hematopoietic stem cell transplantation, based mainly on the graft vs. myeloma effect, can offer prolonged disease control and probability of cure but unfortunately at the cost of considerable transplant-related toxicity rates. Therefore, the role of allogeneic hematopoietic stem cell transplantation in the treatment of multiple myeloma has been called into question. Recently, several studies, particularly those with long-term follow-up, demonstrated a trend of survival superiority for allografted patients with high-risk disease. These data fuel again the interest in allogeneic stem cell transplantation for selected patients with high-risk multiple myeloma, especially if the high remission rates which can be achieved with the currently used treatment protocols could be long-life sustained through the additional exploitation of the long-lasting anti-multiple myeloma effect, originating from the allograft.
Assuntos
Transplante de Células-Tronco Hematopoéticas , Mieloma Múltiplo/terapia , Humanos , Imunoterapia Adotiva , Transfusão de Linfócitos , Recidiva , Doadores de Tecidos , Condicionamento Pré-Transplante , Transplante HomólogoRESUMO
Follicular lymphoma is the most common indolent non-Hodgkin lymphoma. Survival has improved over the last several decades, mainly because of the incorporation of the anti-CD20 antibody rituximab into preexisting or rediscovered agents. The disease has a relapsing and remitting pattern, coupled with a risk of transformation into an aggressive lymphoma, and considered incurable for most patients. Next-generation sequencing technologies have increased our understanding of the biology and genetic landscape of the disease, identifying potential druggable targets for treatment. Current prognostic models cannot accurately identify patients at risk of early progression and despite the availability of treatment options for relapsed/refractory disease, rational treatment selection balancing disease control, efficacy with toxicity, and quality of life remain unmet needs. This review provides an overview of biology, prognostication, treatment options, and emerging therapies that provide valid grounds for optimism.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma Folicular/tratamento farmacológico , Terapia de Alvo Molecular/métodos , Gerenciamento Clínico , Humanos , Linfoma Folicular/imunologia , Linfoma Folicular/patologiaRESUMO
Bone marrow biopsy is an important tool for the evaluation of malignant and benign hematologic disorders. Performed blindly, usually with a Jamshidi biopsy needle penetrating the posterior iliac crest bone, the procedure is generally considered safe but rarely complications are observed; vascular complications associated with bleeding are the most serious and potentially life-threatening. We describe 3 cases of arterial injury following a bone marrow biopsy procedure, all treated successfully with minimal invasive endovascular management, and emphasize the need for clinical awareness and recognition of this rare complication in order to facilitate rapid diagnostic and minimal invasive therapeutic interventions, when appropriate, for successful outcomes.
RESUMO
BACKGROUND: Patients with refractory or relapsed lymphoma diagnosed with bulky disease at relapse or with residual disease after salvage treatment are considered to have a dismal outcome, even after autologous hematopoietic stem-cell transplantation, as a result of disease recurrence. To minimize the risk of relapse after receipt of a transplant, involved-field radiotherapy (IFRT) to sites of either bulky or localized residual disease has been utilized; however, the ideal timing for irradiation remains controversial. The aim of this study was to assess the safety and efficacy of IFRT in the early period after transplantation. PATIENTS AND METHODS: We retrospectively evaluated the outcome of 24 autografted patients with relapsed/refractory lymphoma who presented with bulky disease at relapse or who had a persistent localized residual mass after salvage treatment and consolidated with IFRT within 4 months after autografting. RESULTS: No significant toxicity was noticed during the early postradiotherapy period, while graft function was not impaired. After a median follow-up of 3 years for survivors, 21 patients were alive, 19 of whom were event free, while 2 patients died of disease recurrence and 1 died of treatment-related myelodysplastic syndrome. The 3-year overall, lymphoma relapse-free, and event-free survival rates were 86%, 86%, and 82%, respectively. CONCLUSION: Taking into consideration the poor-risk features of the study cohort, IFRT provided early after autologous hematopoietic stem-cell transplantation showed a safe and well-tolerated toxicity profile and demonstrated long-term effective tumor control, as reflected in the promising survival rates.
Assuntos
Transplante de Células-Tronco Hematopoéticas , Linfoma/terapia , Recidiva Local de Neoplasia/terapia , Radioterapia Adjuvante/métodos , Tempo para o Tratamento/estatística & dados numéricos , Adolescente , Adulto , Idoso , Feminino , Seguimentos , Humanos , Linfoma/mortalidade , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/mortalidade , Radioterapia Adjuvante/efeitos adversos , Radioterapia Adjuvante/estatística & dados numéricos , Estudos Retrospectivos , Taxa de Sobrevida , Transplante Autólogo , Adulto JovemRESUMO
Lymphoblastic lymphoma, seen primarily in children or young adults, is a type of non-Hodgkin lymphoma that originates from B or T lymphocyte precursors and rarely occurs in the oral cavity. A case of systemic precursor T-cell lymphoblastic lymphoma mimicking periodontitis of a lower second molar in a 20-year-old adult is currently presented. The case was initially misdiagnosed as periodontal disease and treated with tooth extraction by a dentist. Re-evaluation of the patient due to worsening of symptoms lead to cone beam computed tomography scanning that thoroughly revealed an extended osteolytic lesion of the right mandible. Afterward, a biopsy was performed, thus reaching the diagnosis of precursor T-cell lymphoblastic lymphoma. This report discusses differences in epidemiology of T-cell and B-cell lymphoblastic lymphomas, as well as their various intraoral manifestations that are mimicking a large family of oral pathology. It also focuses on conventional imaging findings that imply malignancy, which are often neglected during routine radiology interpretation.
RESUMO
Cancer cells escape immune recognition by exploiting the programmed cell-death protein 1 (PD-1)/programmed cell-death 1 ligand 1 (PD-L1) immune checkpoint axis. Immune checkpoint inhibitors that target PD-1/PD-L1 unleash the properties of effector T cells that are licensed to kill cancer cells. Immune checkpoint blockade has dramatically changed the treatment landscape of many cancers. Following the cancer paradigm, preliminary results of clinical trials in lymphoma have demonstrated that immune checkpoint inhibitors induce remarkable responses in specific subtypes, most notably classical Hodgkin lymphoma and primary mediastinal B-cell lymphoma, while in other subtypes, the results vary considerably, from promising to disappointing. Lymphomas that respond to immune checkpoint inhibitors tend to exhibit tumor cells that reside in a T-cell-rich immune microenvironment and display constitutive transcriptional upregulation of genes that facilitate innate immune resistance, such as structural variations of the PD-L1 locus, collectively referred to as T-cell-inflamed lymphomas, while those lacking such characteristics are referred to as noninflamed lymphomas. This distinction is not necessarily a sine qua non of response to immune checkpoint inhibitors, but rather a framework to move the field forward with a more rational approach. In this article, we provide insights on our current understanding of the biological mechanisms of immune checkpoint evasion in specific subtypes of B-cell and T-cell non-Hodgkin lymphomas and summarize the clinical experience of using inhibitors that target immune checkpoints in these subtypes. We also discuss the phenomenon of hyperprogression in T-cell lymphomas, related to the use of such inhibitors when T cells themselves are the target cells, and consider future approaches to refine clinical trials with immune checkpoint inhibitors in non-Hodgkin lymphomas.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Inibidores de Checkpoint Imunológico/uso terapêutico , Linfoma não Hodgkin/tratamento farmacológico , Terapia de Alvo Molecular , Animais , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Tomada de Decisão Clínica , Ensaios Clínicos como Assunto , Diagnóstico Diferencial , Gerenciamento Clínico , Progressão da Doença , Suscetibilidade a Doenças , Regulação Neoplásica da Expressão Gênica/efeitos dos fármacos , Humanos , Inibidores de Checkpoint Imunológico/administração & dosagem , Inibidores de Checkpoint Imunológico/efeitos adversos , Proteínas de Checkpoint Imunológico/genética , Proteínas de Checkpoint Imunológico/metabolismo , Linfoma não Hodgkin/diagnóstico , Linfoma não Hodgkin/etiologia , Linfoma não Hodgkin/mortalidade , Terapia de Alvo Molecular/efeitos adversos , Terapia de Alvo Molecular/métodos , Prognóstico , Resultado do TratamentoRESUMO
The Saudi Lymphoma Group had previously published recommendations on the management of the major subtypes of lymphoma. However, the effect the currently ongoing coronavirus disease 2019 (COVID-19) pandemic has on the management of patients with lymphoma has been paramount. Therefore, the Saudi Lymphoma Group has decided to provide clinical practice guidelines for the diagnosis, management and follow-up of patients with various types of lymphoma during the COVID-19 pandemic.
Assuntos
Doença das Cadeias Pesadas/diagnóstico , Plasmócitos/patologia , Idoso , Apoptose/fisiologia , Células da Medula Óssea/patologia , Tamanho Celular , Diagnóstico Diferencial , Feminino , Doença das Cadeias Pesadas/patologia , Humanos , Cadeias mu de Imunoglobulina/imunologia , Leucemia Linfocítica Crônica de Células B/diagnóstico , Leucemia Linfocítica Crônica de Células B/patologiaRESUMO
OBJECTIVE: To describe a patient with Bcr-abl(+) acute lymphoblastic leukemia who developed the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) while being treated with high-dose imatinib. CASE SUMMARY: A 29-year-old woman was diagnosed with Bcr-abl(+) acute lymphoblastic leukemia, and treatment was initiated with chemotherapy and imatinib 800 mg daily. Following imatinib initiation, a gradual decrease in serum sodium level was noticed. Prolonged aplasia and neutropenic fever prompted discontinuation of therapy for 4 weeks. Following the patient's recovery, complete remission was achieved and monotherapy with imatinib 800 mg daily was restarted; however, hyponatremia recurred a few days later. The clinical findings and laboratory workup were compatible with the diagnosis of SIADH, which was attributed to high-dose imatinib. Fluid restriction and imatinib dosage reduction (to 600 mg/day) restored sodium levels. According to the Naranjo probability scale, this adverse reaction was probably associated with imatinib. DISCUSSION: Imatinib emerged as the first tyrosine kinase inhibitor to enter everyday clinical practice for the treatment of Ph(+) leukemias. Due to its molecular specificity, imatinib lacks the broad cytotoxicity of conventional chemotherapy. Inhibition of kinases in normal tissues accounts for many of imatinib's adverse reactions. To our knowledge, this is the first reported case of imatinib-induced SIADH. CONCLUSIONS: We recommend monitoring for SIADH if a patient receiving high-dose imatinib develops hyponatremia.
Assuntos
Síndrome de Secreção Inadequada de HAD/induzido quimicamente , Piperazinas/efeitos adversos , Inibidores de Proteínas Quinases/efeitos adversos , Pirimidinas/efeitos adversos , Adulto , Antineoplásicos/administração & dosagem , Antineoplásicos/efeitos adversos , Benzamidas , Relação Dose-Resposta a Droga , Monitoramento de Medicamentos , Feminino , Humanos , Hiponatremia/induzido quimicamente , Mesilato de Imatinib , Piperazinas/administração & dosagem , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Inibidores de Proteínas Quinases/administração & dosagem , Pirimidinas/administração & dosagemAssuntos
Infecções por Vírus Epstein-Barr/diagnóstico , Linfo-Histiocitose Hemofagocítica/etiologia , Adulto , Antivirais/uso terapêutico , Células da Medula Óssea/ultraestrutura , Citofagocitose , Erros de Diagnóstico , Infecções por Vírus Epstein-Barr/tratamento farmacológico , Infecções por Vírus Epstein-Barr/patologia , Infecções por Vírus Epstein-Barr/fisiopatologia , Feminino , Herpesvirus Humano 4/isolamento & purificação , Humanos , Mononucleose Infecciosa/diagnóstico , Linfo-Histiocitose Hemofagocítica/virologia , Resultado do TratamentoAssuntos
Neoplasias do Sistema Nervoso Central , Doença de Hodgkin , Encéfalo , Sistema Nervoso Central , Neoplasias do Sistema Nervoso Central/tratamento farmacológico , Neoplasias do Sistema Nervoso Central/terapia , Doença de Hodgkin/tratamento farmacológico , Doença de Hodgkin/terapia , Humanos , Nivolumabe/uso terapêutico , RecidivaAssuntos
Hipergamaglobulinemia/patologia , Doenças Linfáticas/patologia , Linfocitose/patologia , Plasmócitos/patologia , Adulto , Humanos , Hipergamaglobulinemia/complicações , Hipergamaglobulinemia/imunologia , Linfonodos/patologia , Doenças Linfáticas/complicações , Doenças Linfáticas/imunologia , Linfocitose/complicações , Linfocitose/imunologia , MasculinoAssuntos
Antibacterianos/uso terapêutico , Antifúngicos/uso terapêutico , Fungemia/tratamento farmacológico , Fusarium , Leucemia Mieloide Aguda/complicações , Micoses/tratamento farmacológico , Peptídeos Cíclicos , Peptídeos , Idoso , Caspofungina , Equinocandinas , Fungemia/etiologia , Fungemia/microbiologia , Fusarium/efeitos dos fármacos , Humanos , Lipopeptídeos , Masculino , Micoses/etiologiaRESUMO
Since the introduction of combination antiretroviral therapy (cART), there has been a decrease in the incidence of non-Hodgkin lymphoma among the HIV-infected population and also significantly improved survival rates. We describe a remarkable case of a HIV-infected patient whose large B-cell lymphoma, most likely arising by transformation of a nodal marginal zone lymphoma, completely regressed with the use of cART alone. He remained disease-free for almost 3 years and he finally died from presumed flare up of his lymphoma. There are very few cases of spontaneous regression of lymphomas with cART alone in the HIV population. This is an extreme example of the significance of cART in improving survival in HIV-non-Hodgkin lymphoma and changing the face of the HIV epidemic in general.
Assuntos
Terapia Antirretroviral de Alta Atividade , Linfoma Relacionado a AIDS/tratamento farmacológico , Linfoma de Zona Marginal Tipo Células B/complicações , Linfoma de Zona Marginal Tipo Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/complicações , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Humanos , Linfoma Relacionado a AIDS/patologia , Linfoma de Zona Marginal Tipo Células B/patologia , Linfoma Difuso de Grandes Células B/patologia , Masculino , Pessoa de Meia-Idade , Indução de Remissão , Fatores de TempoAssuntos
Anemia/induzido quimicamente , Anticorpos Monoclonais Murinos/efeitos adversos , Antineoplásicos/efeitos adversos , Linfoma Folicular/tratamento farmacológico , Anticorpos Monoclonais Murinos/administração & dosagem , Antineoplásicos/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Humanos , Masculino , Pessoa de Meia-Idade , RituximabRESUMO
PURPOSE: The aim of this retrospective analysis was to determine the outcome of patients with follicular lymphoma who received myeloablative therapy supported by autologous bone marrow transplantation as consolidation of second or subsequent remission, with a minimum follow-up of 12 years. PATIENTS AND METHODS: One hundred twenty-one adults received cyclophosphamide (CY) and total-body irradiation (TBI) supported by autologous bone marrow transplantation, with the marrow mononuclear cell fraction having been treated with monoclonal antibodies and complement. Data from St Bartholomew's Hospital and Dana-Farber Cancer Institute were combined for the purpose of this analysis because the patients were treated in an identical manner. RESULTS: Fifty-seven patients are alive, 41 without progression between 9 and 19 years; 64 patients have died, 20 without progression. With a median follow-up of 13.5 years, 60 patients have developed recurrent lymphoma. There is an apparent plateau on the remission duration curve at 48% at 12 years. Survival of patients treated in second remission was significantly longer than the survival of patients treated later in the course of the illness. Both remission duration and overall survival were also significantly longer for patients treated in second remission compared with an age-matched, remission-matched group of patients treated at St Bartholomew's Hospital before the introduction of this treatment. However, use of CY+TBI was associated with a significant risk of secondary myelodysplasia and secondary acute myeloblastic leukemia, resulting in 15 patient deaths. CONCLUSION: These mature data confirm that prolonged freedom from recurrence may be achieved with myeloablative therapy and that a plateau on the curve seems to emerge with long follow-up.
Assuntos
Transplante de Medula Óssea , Linfoma Folicular/terapia , Adulto , Terapia Combinada , Ciclofosfamida/uso terapêutico , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva , Indução de Remissão , Estudos Retrospectivos , Taxa de Sobrevida , Transplante Autólogo , Resultado do Tratamento , Irradiação Corporal TotalRESUMO
The purpose of this study was to evaluate the use of combination anthracycline-based immunochemotherapy in intravascular lymphoma (IVL). This is an extremely rare, disseminated, and aggressive extranodal CD20(+) non-Hodgkin's lymphoma (NHL) with poor outcome following anthracycline-based chemotherapy. From a population of 700 newly diagnosed patients with NHL who were registered and followed up at our unit between 1990 and 2005, three cases (0.4%) have been classified as IVL. Among the patients, there were two men and one woman, with a median age of 52 years. We have assessed the clinicopathological characteristics, response to therapy, and outcome. All patients presented with systemic symptoms and disseminated disease. All patients received anthracycline-based chemotherapy in combination with the anti-CD20 monoclonal antibody rituximab (immunochemotherapy). Complete remission was achieved in all three patients, and currently all remain progression free with a follow-up of 24-45 months. In conclusion, anthracycline-based immunochemotherapy induces durable remissions in patients with IVL, an ultimately fatal disease, suggesting that the clinical course of this disease may be altered with immunochemotherapy.