RESUMO
Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of malignant lymphoma. The incidence of Epstein-Barr virus (EBV)-positive DLBCL in Asian and Latin American countries ranges from 8 to 10%. The prognosis of patients with EBV-positive DLBCL is controversial. To compare the clinical outcome of EBV-positive and EBV-negative patients with DLBCL in the rituximab era, we analyzed 239 patients with de novo DLBCL diagnosed between January 2007 and December 2011. The presence of EBV in lymphoma cells was detected using EBV-encoded RNA in situ hybridization, and it was found that 18 (6.9%) of 260 patients with diagnosed DLBCL tested positive. Among the 260 cases, 216 cases were treated with rituximab plus chemotherapy, as were 8 EBV-positive DLBCL patients. The median overall survival and progression-free survival times in patients with EBV-positive DLBCL were 8.7 months and 6.8 months, respectively. The median overall survival and progression-free survival could not be determined in EBV-negative DLBCL patients (P = 0.0002, P < 0.0001, respectively). The outcome of patients with EBV-positive DLBCL remains poor, even in the rituximab era.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Infecções por Vírus Epstein-Barr/complicações , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Idoso , Anticorpos Monoclonais Murinos/administração & dosagem , Anticorpos Monoclonais Murinos/uso terapêutico , Ciclofosfamida/uso terapêutico , Doxorrubicina/uso terapêutico , Infecções por Vírus Epstein-Barr/mortalidade , Feminino , Humanos , Estimativa de Kaplan-Meier , Linfoma Difuso de Grandes Células B/mortalidade , Linfoma Difuso de Grandes Células B/virologia , Masculino , Pessoa de Meia-Idade , Prednisona/uso terapêutico , Modelos de Riscos Proporcionais , Rituximab , Resultado do Tratamento , Vincristina/uso terapêuticoRESUMO
We report a case of HIV-negative Burkitt lymphoma (BL) that relapsed 9 years after complete remission. We performed a polymerase chain reaction analysis of three regions of the VDJ junction of the immunoglobulin heavy chain (IGH) gene and compared the clonality of the first and second BL lesions, which were found to be clonally distinct. The patient received the R-Hyper CVAD/R-MA regimen; however, leukoencephalopathy subsequently developed due to the effect of cytarabine, and the regimen was changed to R-IVAM. The patient achieved complete remission and received high-dose chemotherapy following autologous stem cell transplantation. He maintained the complete remission for 72 months after transplantation. Given this outcome, we suggest that clonally distinct relapse of HIV-negative BL may exhibit a good prognosis.
Assuntos
Linfoma de Burkitt/genética , Linfoma de Burkitt/terapia , Recidiva Local de Neoplasia/genética , Recidiva Local de Neoplasia/terapia , Recombinação V(D)J , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Sequência de Bases , Linfoma de Burkitt/patologia , Ciclofosfamida/uso terapêutico , Citarabina/uso terapêutico , Dexametasona/uso terapêutico , Doxorrubicina/uso terapêutico , Etoposídeo/uso terapêutico , Genes de Imunoglobulinas , Transplante de Células-Tronco Hematopoéticas , Humanos , Ifosfamida/uso terapêutico , Cadeias Pesadas de Imunoglobulinas/genética , Masculino , Metotrexato/uso terapêutico , Dados de Sequência Molecular , Recidiva Local de Neoplasia/patologia , Indução de Remissão , Vincristina/uso terapêuticoAssuntos
Linfoma de Células T Periférico , Transtornos Linfoproliferativos , Esclerose Múltipla , Cloridrato de Fingolimode/efeitos adversos , Humanos , Imunossupressores/efeitos adversos , Linfoma de Células T Periférico/diagnóstico , Linfoma de Células T Periférico/tratamento farmacológico , Transtornos Linfoproliferativos/induzido quimicamente , Transtornos Linfoproliferativos/diagnóstico , Esclerose Múltipla/diagnóstico , Esclerose Múltipla/tratamento farmacológicoRESUMO
A 64-year-old woman developed pure red cell aplasia (PRCA) 4 years after thymectomy for thymoma. During anti-thymocyte globulin treatment, the patient developed cytomegalovirus pneumonia and was thus unable to continue immunosuppressive therapy and became transfusion dependent. Deferasirox was started for treatment with iron overload when serum ferritin increased to >1000 ng/mL. Seven months after initiation of deferasirox treatment, serum ferritin level decreased the normal range and the patient has remained transfusion independent thereafter. Deferasirox was discontinued when serum ferritin level decreased below 500 ng/mL, and she has maintained in complete remission over the last 15 months. Hypotheses have been raised regarding the improvement of hematopoiesis by deferasirox treatment, but the mechanism whereby this might be achieved remains unclear. Deferasirox treatment may be clinically beneficial both by reducing iron overload and by improving hematopoiesis in patients with PRCA.
Assuntos
Benzoatos/uso terapêutico , Eritropoese/efeitos dos fármacos , Quelantes de Ferro/uso terapêutico , Aplasia Pura de Série Vermelha/tratamento farmacológico , Triazóis/uso terapêutico , Deferasirox , Transfusão de Eritrócitos , Feminino , Humanos , Pessoa de Meia-Idade , Aplasia Pura de Série Vermelha/diagnóstico , Aplasia Pura de Série Vermelha/terapia , Resultado do TratamentoRESUMO
In some patients with multiple myeloma, extramedullary masses may be present at diagnosis or may develop during treatment. Recently, multiple myeloma has been treated using newer therapeutic regimens based on thalidomide and bortezomib. Using these drugs, positive responses to treatment, not found with conventional antineoplastic agents, have been reported along with an improvement in patient outcome. In the present study, we report on three patients with extramedullary masses associated with multiple myeloma. Although all three patients were treated with bortezomib, it was ineffective against the extramedullary masses and the clinical course of the disease differed between the three patients. We propose that the effects of bortezomib on extramedullary masses may differ from case to case and may not be evident in cases of severe disease. Also, the effects of bortezomib may not be evident in the case of myeloma cells that have left the bone marrow microenvironment, similar to thalidomide. In addition, resistance to bortezomib may manifest as extramedullary masses. (160 words in the body of abstract).
Assuntos
Ácidos Borônicos/uso terapêutico , Resistencia a Medicamentos Antineoplásicos , Mieloma Múltiplo/tratamento farmacológico , Mieloma Múltiplo/patologia , Mieloma Múltiplo/fisiopatologia , Pirazinas/uso terapêutico , Bortezomib , Evolução Fatal , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Splenic marginal zone lymphoma (SP-MZL) is a rare low-grade B-cell neoplasm that often shows leukemic manifestation. Less than 20% of cases of SP-MZL express CD5. We analyzed 11 cases of CD5-positive SP-MZL with leukemic manifestation. The clinical characteristics of these cases did not differ from those of CD5-negative SP-MZL. Flow cytometry revealed positive results as follows : CD3, 0/9 ; CD5, 11/11 ; CD10, 0/11 ; CD11c, 4/10, CD13, 5/11 ; CD19, 11/11 ; CD20, 10/11 ; CD21, 4/4 ; CD22, 7/7 ; CD23, 5/10 ; CD25, 8/11 ; FMC7, 5/7 ; κ type 6/9, and λ type 2/9. All 3 cases with monoclonal γ-globulinemia expressed CD13. Resected spleen exhibited a proliferation of neoplastic cells in white pulp in all 8 splenectomy patients and a marginal pattern was detected in 5 patients. Only 2 cases showed involvement of red pulp. Immunohistochemistry showed that the lymphoma cells were positive for CD5, CD20, and BCL-2 and negative for CD3, CD10, cyclin D1, BCL-6, and MUM-1 in all 11 cases. These results suggest that CD5-positive SP-MZL differs from B-cell chronic lymphocytic leukemia, that CD13 expression is found in about half of CD5-positive SP-MZL cases, and that CD5-positive SP-MZL may be related to memory B-cell neoplasm or plasma cell differentiation.
Assuntos
Antígenos CD5/metabolismo , Linfoma de Zona Marginal Tipo Células B/metabolismo , Linfoma de Zona Marginal Tipo Células B/patologia , Neoplasias Esplênicas/metabolismo , Neoplasias Esplênicas/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/análise , Feminino , Citometria de Fluxo , Humanos , Imuno-Histoquímica , Imunofenotipagem , Masculino , Pessoa de Meia-IdadeRESUMO
Essential thrombocythemia (ET) is a chronic myeloproliferative disorder characterized by thrombo-hemorrhagic complications. But aortic thrombus formation is not so common in the patients with ET at the initial diagnosis. We describe a 65-year-old woman with ET found to have a thoraco-abdominal aortic thrombosis and have splenic infarction, which were successfully treated with medical therapy alone. To our knowledge, only two cases were published with presenting large aortic thrombosis at the onset.