Реферат
The term autoimmune cytopenias is referred to a heterogeneous group of diseases characterized by a reduced peripheral blood cell counts in one or more cellular series, because an immunological disorder. The first line therapy is steroids, followed by splenectomy or immunesupressant therapy in non-responders. Rituximab is an anti CD20 monoclonal antibody used as a third line in refractory patients or as an alternative to splenectomy. We present a retrospective study of nine patients with autoimmune cytopenias treated in a public hospital setting with rituximab. Five patients with the diagnosis of inmune thrombocytopenic purpura received it, all of them achieved hematological response (4 complete and one partial). The median time to the best response was 6 weeks, staying in this category after 6 months of follow up. Four patients with autoimmune hemolytic anemia received rituximab, three of them achieving partial response and one was lost from follow up. No severe adverse effects related to rituximab were registered.
Тема - темы
Humans , Male , Female , Adult , Middle Aged , Aged , Autoimmune Diseases/drug therapy , Thrombocytopenia/drug therapy , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Immunologic Factors/therapeutic use , Anemia, Hemolytic, Autoimmune/drug therapy , Neutropenia/drug therapy , Retrospective Studies , Purpura, Thrombocytopenic, Idiopathic/immunology , Rituximab/administration & dosageРеферат
BACKGROUND/AIMS: Several studies have demonstrated the effect of autologous hematopoietic stem cell transplantation (auto-HSCT) as a salvage treatment for patients with relapsed diffuse large B-cell lymphoma (DLBCL). However, the role of auto-HSCT as a frontline treatment has not been fully investigated in the rituximab era. We validated the age-adjusted International Prognostic Index (aaIPI) score for high-risk DLBCL patients and identified a possible role for frontline auto-HSCT. METHODS: We recommended frontline auto-HSCT for high-risk DLBCL patients who satisfied the criteria of both a higher Ann-Arbor stage (III to IV) and an elevated lactate dehydrogenase (LDH) level at diagnosis with an aaIPI score > or = 2. From 2006 to 2011, among the 150 DLBCL patients aged or = 2 showed inferior overall survival (OS; p = 0.040) and progression-free survival (PFS; p = 0.007) compared to the aaIPI score 0 to 1. Between the two treatment arms among the high-risk DLBCL patients, the clinical parameters were not different. The high-risk group treated with frontline auto-HSCT showed similar OS (p = 0.392) and PFS (p = 0.670) to those in the low-risk group. Thus, frontline auto-HSCT showed superior PFS (p = 0.004), but only a trend towards favorable OS (p = 0.091) compared to R-CHOP alone. CONCLUSIONS: We identified the possible role of frontline auto-HSCT for high-risk DLBCL with a higher stage (III to IV) and elevated LDH level.
Тема - темы
Adolescent , Adult , Female , Humans , Male , Middle Aged , Young Adult , Age Factors , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biomarkers, Tumor/blood , Chemotherapy, Adjuvant , Cyclophosphamide/therapeutic use , Disease Progression , Disease-Free Survival , Doxorubicin/therapeutic use , Kaplan-Meier Estimate , L-Lactate Dehydrogenase/blood , Lymphoma, Large B-Cell, Diffuse/blood , Neoadjuvant Therapy , Neoplasm Staging , Predictive Value of Tests , Prednisone/therapeutic use , Proportional Hazards Models , Reproducibility of Results , Risk Assessment , Risk Factors , Stem Cell Transplantation , Time Factors , Transplantation, Autologous , Treatment Outcome , Up-Regulation , Vincristine/therapeutic useРеферат
BACKGROUND/AIMS: Hepatitis-B-related acute-on-chronic liver failure has a poor prognosis. However, the advent of potent oral antiviral agents means that some patients can now recover with medical treatment. We aimed to identify the prognostic factors for hepatitis-B-related acute-on-chronic liver failure including the initial as well as the dynamically changing clinical parameters during admission. METHODS: Sixty-seven patients were retrospectively enrolled from 2003 to 2012 at Samsung Medical Center. The patients were classified into three categories: Recovery group (n=23), Liver transplantation group (n=28), and Death group (n=16). The Liver transplantation and Death groups were combined into an Unfavorable prognosis group. We analyzed the prognostic factors including the Model for End-Stage Liver Disease (MELD) scores determined at 3-day intervals. RESULTS: A multivariable analysis showed that the unfavorable prognostic factors were a high initial MELD score (> or =28) (odds ratio [OR] =6.64, p=0.015), moderate-to-severe ascites at admission (OR=6.71, P=0.012), and the aggravation of hepatic encephalopathy during hospitalization (> or =grade III) (OR=15.41, P=0.013). Compared with the baseline level, significant reductions in the MELD scores were observed on the 7th day after admission in the Recovery group (P=0.016). CONCLUSIONS: Dynamic changes in clinical parameters during admission are useful prognostic factors for hepatitis-B-related acute-on-chronic liver failure.
Тема - темы
Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Acute-On-Chronic Liver Failure/diagnosis , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Antiviral Agents/therapeutic use , Cyclophosphamide/therapeutic use , DNA, Viral/analysis , Doxorubicin/therapeutic use , Hepatitis B virus/genetics , Hepatitis B, Chronic/complications , Hospitalization , Liver Transplantation , Multivariate Analysis , Odds Ratio , Prednisone/therapeutic use , Prognosis , Retrospective Studies , Severity of Illness Index , Vincristine/therapeutic useРеферат
No abstract available.
Тема - темы
Female , Humans , Middle Aged , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , PAX5 Transcription Factor/metabolism , Bone Marrow/metabolism , Cyclophosphamide/therapeutic use , Doxorubicin/therapeutic use , Endoscopy, Digestive System , Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor , Genetic Loci , Liver/metabolism , Lymphocytes/cytology , Lymphoma, Large B-Cell, Diffuse/complications , Lymphoma, T-Cell, Peripheral/complications , Prednisone/therapeutic use , Receptors, Antigen, T-Cell, gamma-delta/genetics , Tomography, X-Ray Computed , Vincristine/therapeutic useРеферат
The main treatment for pemphigus vulgaris are systemic corticosteroids and immunosuppressive agents, but due to adverse reactions and therapeutic failure, new drugs such as rituximab and mycophenolate mofetil have been used. In this case report are described two cases of severe pemphigus vulgaris refractory to various treatments, with resolution after use of rituximab and mycophenolate mofetil, associated with corticosteroids. A higher-than-usual dose of rituximab was employed, without the occurrence of serious adverse reactions. Mycophenolate mofetil was added as adjunctive therapy due to lack of response to azathioprine.
Тема - темы
Female , Humans , Male , Middle Aged , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Immunologic Factors/therapeutic use , Immunosuppressive Agents/therapeutic use , Mycophenolic Acid/analogs & derivatives , Pemphigus/drug therapy , Adrenal Cortex Hormones/therapeutic use , Azathioprine/therapeutic use , Mycophenolic Acid/therapeutic use , Time Factors , Treatment OutcomeРеферат
Use of rituximab in patients with chronic viral hepatitis can worsen pre-existing hepatitis or reactivate occult infection. There are no reports of use of rituximab in pemphigus patients with co-existing viral hepatitis. Herein, we report three pemphigus patients with co-existing chronic viral hepatitis (hepatitis C (n = 2), hepatitis B (n = 1)), who were treated successfully with rituximab under close supervision and concurrent antiviral drug administration. There was no derangement of the liver function tests or increase in viral load in any of the patients. By incorporating good collaboration with a hepatologist and close follow-up, such patients can be managed successfully with biologic therapies when the conventional treatment modalities have failed.
Тема - темы
Adult , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Antiviral Agents/therapeutic use , Dermatologic Agents/therapeutic use , Female , Hepatitis B, Chronic/complications , Hepatitis B, Chronic/drug therapy , Hepatitis C, Chronic/complications , Hepatitis C, Chronic/drug therapy , Humans , Male , Middle Aged , Pemphigus/complications , Pemphigus/drug therapyРеферат
Objective : To report the experience in three Brazilian institutions with the use of rituximab in patients with different clinical forms of lupus erythematosus systemic in activity. Methods : The study consisted of a sample of 17 patients with LES, who were already being treated, but that at some stage of the disease showed refractory symptoms. The patients were subdivided into groups according to the clinical manifestation, and the responses for the use of rituximab were rated as complete, partial or no response. Data were collected through a spreadsheet, and used specific parameters for each group. The treatment was carried on by using therapeutic dose of 1g, and repeating the infusion within an interval of 15 days. Results : The clinical responses to rituximab of the group only hematological and of the group only osteoarticular were complete in all cases. In the renal group there was a clinical complete response, two partial and one absent. In the renal and hematological group complete response, there was one death and a missing response. The pulmonary group presented a complete response and two partial. Conclusion : The present study demonstrated that rituximab can bring benefits to patients with lupus erythematosus systemic, with good tolerability and mild side effects; it presented, however, variable response according to the system affected. .
Objetivo : Relatar a experiência obtida em três instituições brasileiras com o uso do rituximabe em pacientes com diferentes formas clínicas de lúpus eritematoso sistêmico em atividade. Métodos : Estudo composto por amostra de 17 pacientes portadores de lúpus, que já faziam tratamento, mas que, em algum momento da evolução da doença, apresentaram sintomas refratários. Os pacientes foram subdivididos em grupos de acordo com o acometimento clínico que motivou o uso do imunobiológico, e a resposta ao uso do rituximabe foi classificada como completa, parcial ou ausente. Os dados foram coletados por meio de uma planilha padronizada, sendo utilizados parâmetros específicos para cada grupo. O tratamento foi padronizado com dose terapêutica de 1g, com repetição da infusão em um intervalo de 15 dias. Resultados : As respostas clínicas ao rituximabe dos grupos apenas hematológico e do apenas osteoarticular foi completa em todos os casos. No grupo renal, houve uma resposta clínica completa, duas parciais e uma ausente. No grupo renal e hematológico, houve uma resposta completa, um óbito e uma resposta ausente. O grupo pulmonar apresentou um caso de resposta clínica completa e dois parciais. Conclusão : O presente estudo demonstrou que rituximabe pode trazer benefícios aos pacientes com lúpus eritematoso sistêmico, com tolerabilidade boa e efeitos colaterais brandos, apresentando, contudo, resposta variável, de acordo com o sistema acometido. .
Тема - темы
Adult , Female , Humans , Male , Middle Aged , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Immunologic Factors/therapeutic use , Lupus Erythematosus, Systemic/drug therapy , Brazil , Dose-Response Relationship, Drug , Lung Diseases/drug therapy , Lung Diseases/etiology , Lupus Erythematosus, Systemic/complications , Retrospective Studies , Time Factors , Treatment OutcomeРеферат
Introducción: rituximab es un anticuerpo monoclonal que se une específicamente al antígeno CD20 expresado en los linfocitos B. El uso de rituximab en el tratamiento de la trombocitopenia inmune refractaria no se encuentra aprobado en su ficha técnica. Objetivo: describir las características clínicas, la respuesta terapéutica y los aspectos vinculados a la seguridad con el uso de rituximab en los pacientes con trombocitopenia inmune refractaria asistidos en la Cátedra de Hematología del Hospital de Clínicas y revisar la evidencia sobre el beneficio clínico esperado en este grupo de pacientes. Material y método: se realizó un estudio descriptivo de los pacientes con trombocitopenia inmune refractaria asistidos en la Cátedra de Hematología del Hospital de Clínicas a quienes se les prescribió rituximab. Se realizó una búsqueda bibliográfica en PubMed sobre el uso de rituximab en este tipo de patología. Resultados: se trataron cuatro pacientes con trombocitopenia inmune refractaria con rituximab. Se obtuvo respuesta en tres de cuatro pacientes. La media de tiempo de respuesta fue 9,25 semanas. La respuesta se ha mantenido en los tres pacientes. No se registraron efectos adversos durante la perfusión de rituximab. La evidencia publicada se limita a estudios observacionales, en adultos, con pocos pacientes, habiendo mostrado respuestas favorables. Conclusiones: existen limitaciones en la evidencia sobre el tratamiento de la trombocitopenia inmune refractaria, pero rituximab constituye una alternativa efectiva. Es indispensable la integración clínica para monitorizar la efectividad y seguridad del uso de anticuerpos monoclonales, especialmente en indicaciones no aprobadas...
Тема - темы
Humans , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Thrombocytopenia/therapyРеферат
Granulomatosis with polyangiitis (GPA) or Wegener's disease is characterized by a granulomatous vasculitis of the upper and lower airways and kidney. It involves the lower respiratory tract causing subglottic tracheal stenosis, which occurs in approximately 22% of patients. We report two females aged 40 and 52 years, admitted to the hospital with a subglottic tracheal stenosis. Their symptoms and management are reviewed. The frst patient responded to rituximab. The second patient required a tracheostomy.
Тема - темы
Adult , Female , Humans , Middle Aged , Granulomatosis with Polyangiitis/complications , Tracheal Stenosis/etiology , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Tracheal Stenosis/therapy , TracheostomyРеферат
PURPOSE: The modified Glasgow Prognostic Score (mGPS) consisting of serum C-reactive protein and albumin levels, shows significant prognostic value in several types of tumors. We evaluated the prognostic significance of mGPS in 285 patients with diffuse large B cell lymphoma (DLBCL), retrospectively. MATERIALS AND METHODS: According to mGPS classification, 204 patients (71.5%) had an mGPS of 0, 57 (20%) had an mGPS of 1, and 24 (8.5%) had an mGPS of 2. RESULTS: Our study found that high mGPS were associated with poor prognostic factors including older age, extranodal involvement, advanced disease stage, unfavorable International Prognostic Index scores, and the presence of B symptoms. The complete response (CR) rate after 3 cycles of R-CHOP chemotherapy was higher in patients with mGPS of 0 (53.8%) compared to those with mGPS of 1 (33.3%) or 2 (25.0%) (p=0.001). Patients with mGPS of 0 had significantly better overall survival (OS) than those with mGPS=1 and those with mGPS=2 (p=0.036). Multivariate analyses revealed that the GPS score was a prognostic factor for the CR rate of 3 cycle R-CHOP therapy (p=0.044) as well as OS (p=0.037). CONCLUSION: mGPS can be considered a potential prognostic factor that may predict early responses to R-CHOP therapy in DLBCL patients.
Тема - темы
Adult , Aged , Female , Humans , Male , Middle Aged , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , C-Reactive Protein/metabolism , Cyclophosphamide/therapeutic use , Doxorubicin/therapeutic use , Glasgow Outcome Scale , Lymphoma, Large B-Cell, Diffuse/blood , Multivariate Analysis , Prednisone/therapeutic use , Prognosis , Remission Induction , Retrospective Studies , Serum Albumin/metabolism , Survival Rate , Treatment Outcome , Vincristine/therapeutic useРеферат
The gastrointestinal tract is the most common primary extranodal site for diffuse large B-cell lymphoma (DLBCL). However, there is no consensus on the most appropriate staging system for intestinal DLBCL. We evaluated the utility of the modified Ann Arbor system, the Lugano system, and the Paris staging system (a modification of the Tumor, Node, Metastases [TNM] staging for epithelial tumors) in 66 cases of resected intestinal DLBCL. The cases were treated with surgery, plus either cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) chemotherapy alone (n=26) or with the addition of rituximab immunotherapy (n=40). Median follow-up time was 40.4 months (range, 2.1-171.6 months). Fifty-six patients (84.8%) achieved complete remission. The overall 5-yr survival rate was 86.4% (57/66). Of the stage categories defined for each staging system, only the T stage of the Paris classification showed prognostic significance for overall survival by univariate analysis. However, none of the stage parameters was significantly correlated with patient survival on multivariate analysis. In conclusion, the results suggest that the T stage of the Paris classification system may be a prognostic indicator in intestinal DLBCL. The results also imply that in surgically resected intestinal DLBCL, the addition of rituximab to the CHOP regimen does not confer significant survival advantage.
Тема - темы
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Cyclophosphamide/therapeutic use , Doxorubicin/therapeutic use , Immunologic Factors/therapeutic use , Intestinal Neoplasms/classification , Lymphoma, Large B-Cell, Diffuse/classification , Neoplasm Staging/methods , Prednisone/therapeutic use , Retrospective Studies , Survival , Survival Rate , Treatment Outcome , Vincristine/therapeutic useРеферат
Several studies have suggested that a positive lymphocyte cross-matching (XM) is associated with low graft survival rates and a high prevalence of acute rejection after adult living donor liver transplantations (ALDLTs) using a small-for-size graft. However, there is still no consensus on preoperative desensitization. We adopted the desensitization protocol from ABO-incompatible LDLT. We performed desensitization for the selected patients according to the degree of T lymphocyte cross-match titer, model for end-stage liver disease (MELD) score, and graft liver volume. We retrospectively evaluated 230 consecutive ALDLT recipients for 5 yr. Eleven recipients (4.8%) showed a positive XM. Among them, five patients with the high titer (> 1:16) by antihuman globulin-augmented method (T-AHG) and one with a low titer but a high MELD score of 36 were selected for desensitization: rituximab injection and plasmapheresis before the transplantation. There were no major side effects of desensitization. Four of the patients showed successful depletion of the T-AHG titer. There was no mortality and hyperacute rejection in lymphocyte XM-positive patients, showing no significant difference in survival outcome between two groups (P=1.000). In conclusion, this desensitization protocol for the selected recipients considering the degree of T lymphocyte cross-match titer, MELD score, and graft liver volume is feasible and safe.
Тема - темы
Adult , Female , Humans , Male , Middle Aged , ABO Blood-Group System/immunology , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Desensitization, Immunologic/methods , End Stage Liver Disease/surgery , Graft Rejection/immunology , Graft Survival/immunology , Histocompatibility Testing , Liver/surgery , Liver Transplantation , Living Donors , Plasmapheresis , Preoperative Care , Retrospective Studies , Severity of Illness Index , Survival Rate , T-Lymphocytes/immunology , Transplant RecipientsРеферат
For years the mainstay of antiphospholipid syndrome treatment has been anticoagulation and antiplatelet therapy, but the autoimmune nature of the disease, and complications of these therapies, created the need to develop new therapeutic strategies. New therapeutic alternatives inhibit at different levels, the cascade of events leading to the pro-thrombotic state characteristic of the antiphospholipid syndrome. We conducted a literature review of these new treatments, focusing on the pathophysiological bases that support them and their possible clinical applications.
Тема - темы
Humans , Antiphospholipid Syndrome/drug therapy , Agmatine/analogs & derivatives , Agmatine/therapeutic use , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Antiphospholipid Syndrome/physiopathology , Dipeptides/therapeutic use , Glucocorticoids/therapeutic use , Hydroxychloroquine/therapeutic use , Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use , Thrombosis/drug therapyРеферат
El linfoma B difuso de células grandes es un linfoma no Hodgkin de fenotipo B y gran agresividad, con una diseminación a ovario del 7 por ciento. Su presentación de forma primaria en ovario es muy poco común, representando el 0,5 por ciento de todos los linfomas no Hodgkin y el 1,5 por ciento de todos los tumores de ovario. En este caso se presenta una paciente con clínica de dolor y distensión abdominal con una masa pélvica palpable, estableciéndose posteriormente el diagnóstico de linfoma B difuso de células grandes bilateral de ovario. En determinadas situaciones resulta complicado diferenciar el origen primario o secundario de la neoplasia. Los linfomas primarios localizados en ovario tienen un mejor pronóstico que los secundarios diseminados a ovario cuyo pronóstico es más sombrío. Sin embargo la mayoría de los casos publicados están basados en tratamiento quimioterápico previo a la era de rituximab.
The diffuse large B-cell lymphoma is a highly aggressive phenotype B non-Hodgkin lymphoma which is characterized by 7 percent dissemination in the ovary. Its presentation in primary form in the ovary is very uncommon and accounts for 0.5 percent of all lymphomas. In this case, the patient presents symptoms of pain and abdominal strain with a palpable pelvic mass and a post-diagnosis of bilateral diffuse large B-cell lymphoma in the ovary. In certain situations, it is complicated to differentiate between the primary and secondary origin of the neoplasia. The primary lymphomas located in the ovary have a better prognosis than secondary lymphomas whose prognosis is more uncertain. However, the majority of published cases are subjected to chemothe-rapeutic treatment prior to rituximab.
Тема - темы
Humans , Female , Middle Aged , Lymphoma, Large B-Cell, Diffuse/diagnosis , Lymphoma, Large B-Cell, Diffuse/therapy , Ovarian Neoplasms/diagnosis , Ovarian Neoplasms/therapy , Appendectomy , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Hysterectomy , Lymph Node Excision , Antineoplastic Combined Chemotherapy Protocols/therapeutic useРеферат
El linfoma primario de hueso es una enfermedad infrecuente, que tiene una presentación y evolución diferente a los linfomas de otras localizaciones. Se presenta un caso de linfoma primario de hueso de localización craneana y esternal de rápido crecimiento. En su evolución, realizada la exéresis de la lesión primaria de calota, presentó aparición de nuevas lesiones de rápido crecimiento a nivel craneano y fémur y progresión de lesión preesternal que, con anatomía patológica de linfoma no Hodgkin difuso de células grandes B, inició R-CHOPP (ciclofosfamida, doxorrubicina, vincristina, prednisona y rituximab) con rápida disminución de todas las lesiones sin evidencia de progresión al cabo de los seis ciclos.
Primary bone lymphoma is a rare disease, which usually has a different presentation and evolution than lymphomas of other locations. We are presenting a case of primary bone lymphoma of rapid growth, in cranial and sternal locations. In its evolution, once the excision of the primary lesion of the skull was performed, the patient presented new lesions of rapid growth at the skull and femur level, and progression of pre-sternal lesion. With large B-cell diffuse non-Hodgkin lymphoma pathology, the patient initiated R-CHOPP (cyclophosphamide, doxorubicin, vincristine, prednisone and rituximab) with rapid reduction of all lesions without evidence of progression after the six cycles.
Тема - темы
Female , Humans , Middle Aged , Bone Neoplasms/pathology , Lymphoma, Large B-Cell, Diffuse/pathology , Neoplasms, Multiple Primary/pathology , Skull Neoplasms/pathology , Sternum/pathology , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Bone Neoplasms/drug therapy , Cyclophosphamide/therapeutic use , Doxorubicin/therapeutic use , Femoral Neoplasms/drug therapy , Femoral Neoplasms/pathology , Lymphoma, Large B-Cell, Diffuse/drug therapy , Neoplasms, Multiple Primary/drug therapy , Prednisone/therapeutic use , Rare Diseases/drug therapy , Rare Diseases/pathology , Skull Neoplasms/drug therapy , Vincristine/therapeutic useРеферат
Massive splenomegaly is in the which the growth of the spleen has spread to other quadrants of the abdomen. It is produced by a limited number of pathologies, both benign and malignant. It is presented a case of a 62 year-old woman who is consulting for four years of progressive increase in her abdominal volume, associated to the feeling of abdominal fullness, dyspnea on moderate exertion and lower extremities edema. At the physical examination was observed massive splenomegaly and jaundice. The hemogram showed pancytopenia and a lymphocyte count of80 percent. The myelogram revealed marrow infiltration by lymphocytes of mature appearance. Flow cytometry of peripheral blood showed 70 percent of lymphocytes, which expressed B cells markers CD19, CD20, CD23and FMC7 in addition to Kappa light chain restriction, suggesting marginal splenic zone lymphoma. The bone marrow biopsy showed lymphoid small cells infiltrate with positive markers CD20, CD5,CD23 and negative cyclin D1 study. BCL-2 was also positive. It was considered unfit to receive chemotherapy and was treated with 4 cycles of rituximab, with significant decrease of splenic size.
Тема - темы
Humans , Female , Middle Aged , Splenomegaly/pathology , Lymphoma, B-Cell, Marginal Zone/pathology , Splenic Neoplasms/pathology , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Antineoplastic Agents/therapeutic use , Splenomegaly/etiology , Lymphoma, B-Cell, Marginal Zone/complications , Lymphoma, B-Cell, Marginal Zone/drug therapy , Splenic Neoplasms/drug therapyРеферат
A anemia hemolítica autoimune (AHAI) é uma doença na qual são produzidos anticorpos diretamente contra as glicoproteínas adsorvidas na superfície da membrana dos eritrócitos. Algumas medicações e outras associações têm sido implicadas. Descrevemos e discutimos um caso de livedo reticular associado à AHAI tratado com transplante de células-tronco de sangue periférico (TCTSP) e que entrou em total remissão por 10 anos. Após esse período, a paciente apresentou recaída, foi tratada com anticorpo anti-CD20 (rituximabe), e atualmente encontra-se em total remissão. O papel do TCTSP e o uso de rituximabe no tratamento de AHAI serão discutidos neste relato de caso.
Autoimmune hemolytic anemia (AIHA) is a disease where patients produce antibodies against erythrocytes directed towards membrane glycoproteins adsorbed onto the erythrocyte surface. Drugs and other associations have been implicated. It is described and discussed a case of livedo reticularis associated with AIHA treated with peripheral blood stem cell transplantation (PBSCT) that went into full remission for 10 years. After that period the patient relapsed and was treated with antibody anti-CD20, rituximab, and is now in full remission. The role of PBSCT and rituximab in the treatment of AIHA will be discussed.
Тема - темы
Aged , Female , Humans , Anemia, Hemolytic, Autoimmune/drug therapy , Anemia, Hemolytic, Autoimmune/surgery , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Immunologic Factors/therapeutic use , Livedo Reticularis/drug therapy , Livedo Reticularis/surgery , Peripheral Blood Stem Cell Transplantation , Anemia, Hemolytic, Autoimmune/complications , Livedo Reticularis/complications , Recurrence , Remission Induction , Time FactorsРеферат
Neuromyelitis optica (NMO) is an inflammatory disease of the central nervous system characterized by severe optic neuritis and transverse myelitis, usually with a relapsing course. Aquaporin-4 antibody is positive in a high percentage of NMO patients and it is directed against this water channel richly expressed on foot processes of astrocytes. Due to the severity of NMO attacks and the high risk for disability, treatment should be instituted as soon as the diagnosis is confirmed. There is increasing evidence that NMO patients respond differently from patients with multiple sclerosis (MS), and, therefore, treatments for MS may not be suitable for NMO. Acute NMO attacks usually are treated with high dose intravenous corticosteroid pulse and plasmapheresis. Maintenance therapy is also required to avoid further attacks and it is based on low-dose oral corticosteroids and non-specific immunosuppressant drugs, like azathioprine and mycophenolate mofetil. New therapy strategies using monoclonal antibodies like rituximab have been tested in NMO, with positive results in open label studies. However, there is no controlled randomized trial to confirm the safety and efficacy for the drugs currently used in NMO.
Neuromielite óptica (NMO) é uma doença inflamatória do sistema nervoso central caracterizada por grave neurite óptica e mielite transversa, com um curso usualmente recorrente. O anticorpo contra aquaporina-4 é positivo em grande porcentagem dos pacientes com NMO e se liga a este canal de água altamente expresso nos processos pediosos dos astrócitos. Devido à gravidade dos ataques de NMO e ao elevado risco de incapacidade, o tratamento deve ser instituído tão logo o diagnostico seja confirmado. Existem evidências crescentes de que pacientes com NMO respondem de forma diferente dos pacientes com esclerose múltipla (EM) e, portanto, os tratamentos utilizados na EM podem não ser adequados para NMO. Os quadros agudos de NMO são tratados com pulsos de corticosteroides em altas doses e plasmaférese. O tratamento de manutenção também deve ser instituído para evitar ataques subsequentes e é baseado em corticosteroides orais em baixas doses ou imunossupressores, como a azatioprina e o micofenolato mofetil. Novas estratégias de tratamento utilizando anticorpos monoclonais como rituximab têm sido avaliadas para NMO, com resultados positivos em estudos abertos. Entretanto, não existem estudos clínicos controlados, randomizados, para confirmar a segurança e eficácia dos tratamentos atualmente utilizados na NMO.
Тема - темы
Humans , Immunosuppressive Agents/therapeutic use , Neuromyelitis Optica/drug therapy , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Antibodies, Monoclonal/therapeutic use , /therapeutic use , Autoantibodies/therapeutic use , Azathioprine/therapeutic use , Evidence-Based Medicine , Mycophenolic Acid/analogs & derivatives , Mycophenolic Acid/therapeutic useРеферат
El Síndrome Púrpura Trombótico Trombocitopénico/Síndrome Hemolítico Urémico (PTT/SHU) es la principal causa de Microangiopatía Trombótica (MAT) en pacientes con Lupus Eritematoso Sistémico (LES). Entre sus manifestaciones destacan la presencia de anemia hemolítica autoinmune, con trombocitopenia y falla renal en grados variables. No existe correlación entre los niveles de actividad de ADAMTS 13 y MAT. Presentamos un caso clínico de MAT asociado a LES. Se debe tener una alta sospecha diagnóstica por la sobreposición de las manifestaciones clínicas de PTT/SHU y LES. El tratamiento con plasmaféresis ha disminuido la mortalidad de 90 por ciento a 15 por ciento. En casos refractarios se ha reportado el uso de Rituximab, aunque aún falta evidencia que lo avale.
The thrombotic Thrombocytopenic Purpura Syndrome / Hemolytic Uremic Syndrome (TTP/HUS) is the main cause behind Thrombotic Microangiopathy (TMA) in patients with Systemic Lupus Erythematosus (SLE). Among the ways in which it manifests itself is the presence of autoimmune hemolytic anemia (AIHA), with thrombocytopenia and kidney failure in various degrees. There is no co-relation between the levels of activity of ADAMTS13 and TMA. We present a clinical case of TMA associated to SLE. A high suspicion is paramount for diagnose due to the overlapping of clinical manifestations of TTP/HUS and SLE. Treatment with plasmapheresis has decreased mortality from 90 percent to 15 percent. Use of Rituximab in refractory cases has been reported, albeit a lack of supporting evidence.
Тема - темы
Humans , Female , Lupus Erythematosus, Systemic/complications , Thrombotic Microangiopathies/diagnosis , Thrombotic Microangiopathies/therapy , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Lupus Erythematosus, Systemic/therapy , Plasmapheresis , PrognosisРеферат
Diffuse large B cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin lymphoma in all countries and all age groups. DLBCL is potentially curable, and the outcome of patients with DLBCL has completely changed with the introduction of therapy involving the monoclonal antibody rituximab in combination with chemotherapy. Nonetheless, relapse is detected after treatment with rituximab, cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisolone in approximately 30% of patients. It has recently become clear that DLBCL represents a heterogeneous admixture of quite different entities. Gene expression profiling has uncovered DLBCL subtypes that have distinct clinical behaviors and prognoses; however, incorporation of this information into treatment algorithms awaits further investigation. Future approaches to DLBCL treatment will use this new genetic information to identify potential biomarkers for prognosis and targets for treatment.