Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 14.213
Filtrar
2.
Rinsho Ketsueki ; 61(1): 39-43, 2020.
Artigo em Japonês | MEDLINE | ID: mdl-32023601

RESUMO

There are few reports of a patient presenting with combined follicular lymphoma (FL) and classical Hodgkin lymphoma (cHL). A 37-year-old Japanese man was diagnosed with FL with generalized lymphadenopathy and treated with R-CHOP. Four months later, he presented with fever, elevated lactic acid dehydrogenase (LDH), and pancytopenia. Consequently, he was diagnosed with cHL in the bone marrow. Identical clonality of FL and cHL tumor cells suggested identical immunoglobulin heavy chain gene rearrangements. He was treated with salvage chemotherapy and high-dose chemotherapy with autologous hematopoietic stem cell transformation (HDT-ASCT), which has led to complete remission and no recurrence for more than two years after transplantation. Our case suggests that HDT-ASCT may be an effective treatment for this rare clinical condition.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Doença de Hodgkin , Linfoma Folicular , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica , Humanos , Masculino , Recidiva Local de Neoplasia , Rituximab , Transplante Autólogo
3.
Medicine (Baltimore) ; 99(6): e18590, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32028388

RESUMO

RATIONALE: The specific pathogenesis of the diffuse large B-cell lymphoma(DLBCL)is still indefinite and argumentative. It is known that DLBCL is the most common type of non-Hodgkin's lymphomas (NHL). A lot of cases of DLBCL such as primary gastric diffuse large B-cell lymphoma(PG-DLBCL) are reported. However, primary intestinal diffuse large B-cell lymphoma(PI-DLBCL) is unusual. PATIENT CONCERNS: We present a case of a 57-year-old male diagnosed in the Gastroenterology Department, which presented a bleeding duodenal ulcer with irregular borders. DIAGNOSES: The immunohistochemical staining showed: CD20(+++), CD10(+) and Ki-67>40%. INTERVENTIONS: The patient was successfully treated by Poly-chemotherapy with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vindesine and prednisolone). OUTCOMES: After 6 courses of chemotherapy treatment, the duodenal ulcer was completely healed by reviewing the UGIE. LESSONS: Our report might give further strength to avoiding the erroneous and missed diagnosis for PI-DLBCL which is different from common duodenal ulcer.


Assuntos
Úlcera Duodenal/etiologia , Neoplasias Intestinais/diagnóstico , Linfoma Difuso de Grandes Células B/diagnóstico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ciclofosfamida/uso terapêutico , Diagnóstico Diferencial , Doxorrubicina/uso terapêutico , Humanos , Imuno-Histoquímica , Neoplasias Intestinais/complicações , Neoplasias Intestinais/diagnóstico por imagem , Neoplasias Intestinais/tratamento farmacológico , Linfoma Difuso de Grandes Células B/complicações , Linfoma Difuso de Grandes Células B/diagnóstico por imagem , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Prednisona/uso terapêutico , Rituximab/uso terapêutico , Vincristina/uso terapêutico
4.
Harefuah ; 159(1): 31-33, 2020 Jan.
Artigo em Hebraico | MEDLINE | ID: mdl-31930805

RESUMO

INTRODUCTION: Pemphigus is a chronic autoimmune bullous disease. To date there is no curative treatment for the disease. The standard treatment has been based on systemic corticosteroids and immune-suppressants. Rituximab is a monoclonal antibody against CD20 cells which leads to B cell depletion, resulting in decreased antibody production. In recent years increasing evidence on promising efficacy and safety of rituximab in the treatment of pemphigus has emerged. This review presents the key publications and the Israeli experience with rituximab treatment at the Rabin and the Sheba Medical Centers. Disclosure: Prof. Daniel Mimouni participated in an advisory board of Roche.


Assuntos
Doenças Autoimunes , Fatores Imunológicos/uso terapêutico , Pênfigo/tratamento farmacológico , Rituximab/uso terapêutico , Anticorpos Monoclonais Murinos , Humanos , Imunossupressores
5.
Ann Hematol ; 99(1): 105-112, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31776726

RESUMO

Outcome of patients with relapsed/refractory (r/r) diffuse large B-cell lymphoma (DLBCL) remains poor, highlighting the need for novel treatment approaches. The multicentre randomised phase II LEGEND trial evaluated lenalidomide in combination with rituximab, methylprednisolone and gemcitabine (R-GEM-L) vs. standard R-GEM-P as second-line treatment of DLBCL. The study closed early to recruitment after the planned interim analysis failed to demonstrate a complete response (CR) rate of ≥ 40% in either arm. Among 34 evaluable patients, 7/18 (38.9%) achieved CR with R-GEM-L and 3/16 (18.8%) with R-GEM-P. Median event-free and overall survival was 3.5/3.8 months and 10.8/8.3 months for R-GEM-L and R-GEM-P, respectively. The incidence of grade ≥ 3 toxicities was 52% in R-GEM-L and 83% in R-GEM-P. Efficacy and tolerability of R-GEM-L seem comparable with R-GEM-P and other standard salvage therapies, but a stringent design led to early trial closure. Combination of lenalidomide with gemcitabine-based regimens should be further evaluated in r/r DLBCL.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/mortalidade , Adulto , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Desoxicitidina/administração & dosagem , Desoxicitidina/efeitos adversos , Desoxicitidina/análogos & derivados , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Metilprednisolona/administração & dosagem , Metilprednisolona/efeitos adversos , Pessoa de Meia-Idade , Rituximab/administração & dosagem , Rituximab/efeitos adversos , Taxa de Sobrevida
6.
J Oral Pathol Med ; 49(1): 91-95, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31420993

RESUMO

BACKGROUND: Pemphigus vulgaris patients with exclusive oral involvement (OPV) treated with conventional immunosuppressive therapy may be non-responders or experience severe side effects and/or relapses. In such cases, rituximab could be used as an adjuvant in recalcitrant OPV patients. METHODS: A retrospective single-center study on patients with oral pemphigus vulgaris treated with RTX at a dose of 375 mg/m2 was performed, evaluating the complete clinical and immunological remission, side effects of RTX, and possible correlation between anti-desmoglein (Dsg) 3 antibodies and clinical remission. RESULTS: We treated 10 OPV patients, of which 60% had a moderate and 40% mild disease severity before therapy with RTX. Complete clinical remission (CCR) was achieved in 100% of OPV patients, of which 20% developed side effects and 20% experienced a relapse in a mean time of 15.2 ± 10.2 weeks. The mean time for CCR was achieved in 19.8 ± 10.3 weeks, whereas the duration of the CCR consisted in 37.4 ± 33.5 weeks. OPV patients underwent a mean follow-up of 57.2 ± 37.7 weeks. In all patients, the mean of pemphigus disease area index (PDAI) decreased from 20.3 ± 14.1 to 0.4 ± 0.0, whereas the mean Dsg3 value dropped from 157.1 ± 40.6 to 67.0 ± 26.6 after therapy with RTX. However, no correlation was found between PDAI and anti-Dsg3 antibodies before and after therapy with RTX (P > .05). CONCLUSIONS: RTX represents a valid and safe alternative as an adjuvant in OPV patients with low rate of relapses and side effects.


Assuntos
Pênfigo , Rituximab/uso terapêutico , Desmogleína 3 , Humanos , Imunossupressores , Pênfigo/tratamento farmacológico , Estudos Retrospectivos
8.
Ann Hematol ; 99(2): 223-228, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31853704

RESUMO

Limited-stage (Ann Arbor stage I or II) mantle cell lymphoma (MCL) is an extremely rare disease. Thus, there is little data on the clinical features and treatment outcomes of patients with early-stage MCL. We examined consecutive stage I or II MCL 41 cases diagnosed between 2000 and 2016 in 16 institutions of the Consortium for Improving Survival of Lymphoma group. All cases were pathologically confirmed and systemic evaluation was performed for staging. The clinical features were reviewed, and the treatment outcomes were analyzed. The median age of patients was 66 years (range 19-85 years); there were more men (n = 31, 75.6%) than women. Most patients (n = 28, 68.3%) had stage 2 disease, and 29 (70.7%) were symptomatic. The elevation of lactate dehydrogenase (n = 2, 4.9%) was not common; thus, 39 patients (95.1%) had a low-risk score (0 or 1) for the International Prognostic Index, and 28 (68.3%) had a low-risk score (1-3) for the MCL International Prognostic Index. Most patients (n = 37, 90.1%) received chemotherapy as the first therapeutic strategy, while some received radiotherapy (n = 2), surgical resection (n = 1), or no treatment (n = 1). Of the patients who received chemotherapy, 23 (56.9%) received a rituximab-containing regimen, and R-CHOP (n = 17) and R-bendamustine (n = 5) were commonly used. The best response was noted in 97.4% (n = 38) of patients, including 32 who showed a complete response (78%). With a median follow-up duration of 40.6 months, the 42 months relapse-free survival was 59.1%, and the 5-year overall survival rate was 80.4%. Limited-state MCL showed indolent clinical and low-risk prognostic features. Chemotherapy could be effective for controlling localized MCL lesions, with high complete response rates.


Assuntos
Linfoma de Célula do Manto , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Cloridrato de Bendamustina/administração & dosagem , Ciclofosfamida/administração & dosagem , Intervalo Livre de Doença , Doxorrubicina/administração & dosagem , Feminino , Seguimentos , Humanos , Linfoma de Célula do Manto/diagnóstico , Linfoma de Célula do Manto/mortalidade , Linfoma de Célula do Manto/patologia , Linfoma de Célula do Manto/terapia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prednisona/administração & dosagem , Radioterapia , Estudos Retrospectivos , Fatores de Risco , Rituximab/administração & dosagem , Taxa de Sobrevida , Fatores de Tempo , Vincristina/administração & dosagem
9.
Lancet ; 394(10216): 2271-2281, 2020 12 21.
Artigo em Inglês | MEDLINE | ID: mdl-31868632

RESUMO

BACKGROUND: Six cycles of R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone) are the standard treatment for aggressive B-cell non-Hodgkin lymphoma. In the FLYER trial, we assessed whether four cycles of CHOP plus six applications of rituximab are non-inferior to six cycles of R-CHOP in a population of patients with B-cell non-Hodgkin lymphoma with favourable prognosis. METHODS: This two-arm, open-label, international, multicentre, prospective, randomised phase 3 non-inferiority trial was done at 138 clinical sites in Denmark, Israel, Italy, Norway, and Germany. We enrolled patients aged 18-60 years, with stage I-II disease, normal serum lactate dehydrogenase concentration, ECOG performance status 0-1, and without bulky disease (maximal tumour diameter <7·5 cm). Randomisation was computer-based and done centrally in a 1:1 ratio using the Pocock minimisation algorithm after stratification for centres, stage (I vs II), and extralymphatic sites (no vs yes). Patients were assigned to receive either six cycles of R-CHOP or four cycles of R-CHOP plus two doses of rituximab. CHOP comprised cyclophosphamide (750 mg/m2), doxorubicin (50 mg/m2), and vincristine (1·4 mg/m2, with a maximum total dose of 2 mg), all administered intravenously on day 1, plus oral prednisone or prednisolone at the discretion of the investigator (100 mg) administered on days 1-5. Rituximab was given at a dose of 375 mg/m2 of body surface area. Cycles were repeated every 21 days. No radiotherapy was planned except for testicular lymphoma treatment. The primary endpoint was progression-free survival after 3 years. The primary analysis was done in the intention-to-treat population. Safety was assessed in all patients who received at least one dose of assigned treatment. A non-inferiority margin of -5·5% was chosen. The trial, which is completed, was prospectively registered at ClinicalTrials.gov, NCT00278421. FINDINGS: Between Dec 2, 2005, and Oct 7, 2016, 592 patients were enrolled, of whom 295 patients were randomly assigned to receive six cycles of R-CHOP and 297 were assigned to receive four cycles of R-CHOP plus two doses of rituximab. Four patients in the four-cycles group withdrew informed consent before the start of treatment, so 588 patients were included in the intention-to-treat analysis. After a median follow-up of 66 months (IQR 42-100), 3-year progression-free survival of patients who had four cycles of R-CHOP plus two doses of rituximab was 96% (95% CI 94-99), which was 3% better (lower limit of the one-sided 95% CI for the difference was 0%) than six cycles of R-CHOP, demonstrating the non-inferiority of the four-cycles regimen. 294 haematological and 1036 non-haematological adverse events were documented in the four-cycles group compared with 426 haematological and 1280 non-haematological adverse events in the six-cycles group. Two patients, both in the six-cycles group, died during study therapy. INTERPRETATION: In young patients with aggressive B-cell non-Hodgkin lymphoma and favourable prognosis, four cycles of R-CHOP is non-inferior to six cycles of R-CHOP, with relevant reduction of toxic effects. Thus, chemotherapy can be reduced without compromising outcomes in this population. FUNDING: Deutsche Krebshilfe.


Assuntos
Antineoplásicos Imunológicos/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Rituximab/administração & dosagem , Administração Intravenosa , Administração Oral , Adulto , Antineoplásicos Imunológicos/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ciclofosfamida/administração & dosagem , Ciclofosfamida/uso terapêutico , Dinamarca , Doxorrubicina/administração & dosagem , Doxorrubicina/uso terapêutico , Esquema de Medicação , Feminino , Alemanha , Humanos , Cooperação Internacional , Israel , Itália , Linfoma Difuso de Grandes Células B/patologia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Noruega , Prednisona/administração & dosagem , Prednisona/uso terapêutico , Estudos Prospectivos , Rituximab/uso terapêutico , Análise de Sobrevida , Resultado do Tratamento , Vincristina/administração & dosagem , Vincristina/uso terapêutico , Adulto Jovem
11.
Ann Hematol ; 99(2): 391-393, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31858188
12.
J Oncol Pharm Pract ; 26(1): 220-223, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30854926

RESUMO

Kaposi sarcoma (KS) is a low-grade mesenchymal angioproliferative disease, mostly observed in immune compromised patients. KS is mostly encountered in HIV-positive or organ transplant patients. The drugs causing immunosuppression have also been associated with KS. Here, we present a KS experience associated with rituximab-based therapy.


Assuntos
Antineoplásicos Imunológicos/efeitos adversos , Rituximab/efeitos adversos , Sarcoma de Kaposi/induzido quimicamente , Idoso , Humanos , Masculino
13.
Anal Bioanal Chem ; 412(3): 763-775, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31853605

RESUMO

Biosimilars are highly similar to, but not identical with, their originator products. As a result, structural differences between originators and biosimilars can be difficult to detect and characterize without the appropriate analytical tools. Therefore, we first focus on identifying initial structural differences between rituximab, bevacizumab, and trastuzumab originator and biosimilar pairs and later address how these differences change after applying thermal stress at 40 °C with orbital shaking for 4 weeks. Prior to incubation, we detected comparable secondary and tertiary structures for each pair and identified different levels of soluble aggregates, charge variants, and molecular weight variants due to differences in glycoforms and the number of C-terminal lysine groups. Over the course of incubation, we compared differences in charge variants and unfolding patterns. Taken together, our study provides a comparability exercise, providing information on the minor differences present between originator and biosimilar products and how those differences are impacted by stress.


Assuntos
Bevacizumab/química , Medicamentos Biossimilares/química , Temperatura Alta , Rituximab/química , Trastuzumab/química , Peso Molecular , Análise Espectral/métodos
14.
Zhonghua Bing Li Xue Za Zhi ; 48(12): 951-954, 2019 Dec 08.
Artigo em Chinês | MEDLINE | ID: mdl-31818069

RESUMO

Objectives: To investigate the clinicopathological features, therapy and prognosis of primary cardiac CD5-positive diffuse large B-cell lymphoma with C-MYC and bcl-2 double expression. Methods: Two cases diagnosed at Guangdong Provincial People's Hospital were included, the clinical data were collected; the tumor morphology, immunophenotypic profiles, therapy and prognosis were analyzed. Results: Case 1 was a 55-year-old man and case 2 was a 61-year-old women. Intraoperatively, both cases showed large masses in the right atrium or ventricle, involving adjacent tissue. Pathologically, the tumors were composed of diffusely infiltrating large lymphoid cells with high mitotic activity and apoptosis. The tumor cells were positive for CD20, CD5, bcl-6, MUM1, C-MYC and bcl-2, and the Ki-67 index was equal or greater than 90%. Case 1 had bcl-6, but not bcl-2 or MYC gene rearrangements. No MYC, bcl-2 or bcl-6 gene rearrangements were detected in case 2. Case 1 defaulted chemotherapy after operation and died 1 month after diagnosis. Case 2 was treated with 4 cycles of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) therapy after surgery and attained partial remission, and was then treated with apatinib and ibrutinib, and remained stable 18 months after initial diagnosis. Conclusion: Primary cardiac CD5-positive diffuse large B-cell lymphoma with C-MYC and bcl-2 double expression usually shows large infiltrative mass in the right atrium or ventricle, non-germinal center like immunophenotype and high proliferation index, and this may contribute to the aggressiveness of primary cardiac lymphoma.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/patologia , Ciclofosfamida , Doxorrubicina , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prednisona , Proteínas Proto-Oncogênicas c-bcl-2/genética , Proteínas Proto-Oncogênicas c-myc/genética , Rituximab , Vincristina
15.
Medicine (Baltimore) ; 98(50): e18384, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31852153

RESUMO

RATIONAL: Intravascular large B-cell lymphoma (IVLBCL) is a rare condition with a poor prognosis. The clinical presentation of primary lymphoma of the prostate is non-specific and it is difficult to distinguish from other prostatic diseases. The primary prostate IVLBCL is very rare, the diagnosis and treatment of which remains unclear. We reported a rare case to explore the diagnosis and treatment for the primary prostate IVLBCL. PATIENTS CONCERNS: This report described a case of a 71-year-old male diagnosed as primary prostate IVLBCL who presented with prostatic hyperplasia. DIAGNOSIS: The patient first visited an outpatient clinic of urinary surgery because of urinary urgency and frequency and was diagnosed as benign prostatic hyperplasia in about January 2010. Four years later, the symptoms worsened quickly within two months. The diagnosis was still prostatic hyperplasia according to the physical examination and imaging. However, histopathology showed IVLBCL of prostate after transurethral resection of the prostate. INTERVENTIONS: With the clear diagnosis of primary prostate stage I IVLBCL, the patient received immunochemotherapy of R-CHOP (rituximab, cyclophosphamide, adriamycin, vincristine, and prednisolone) for 4 cycles and intensity-modulated radiation therapy (IMRT) including the region of prostate with the dose of 45Gy/25f. OUTCOMES: The response was complete remission after all treatment. The last follow-up time of the patient was June 20th, 2019, and no evidence of disease progression was observed. The progression-free survival of the patient was about 49 months until now. LESSONS: The biopsy of prostate by prostatectomy plays an important role in the diagnosis and removal of the original lesion of primary prostate lymphoma. There is no consensus on therapeutic modalities for the treatment of primary prostate IVLBCL till now. Individual treatments include immunochemotherapy and/or radiotherapy according to the National Comprehensive Cancer Network (NCCN) practice guideline of diffuse large B cell lymphoma (DLBCL) based on the performance status and tumor staging of the patient. Timely and accurate diagnosis as well as the appropriate treatment may improve the clinical outcome.


Assuntos
Linfoma Difuso de Grandes Células B/patologia , Neoplasias da Próstata/patologia , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica , Ciclofosfamida , Erros de Diagnóstico , Doxorrubicina , Humanos , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/terapia , Imagem por Ressonância Magnética , Masculino , Prednisona , Hiperplasia Prostática/diagnóstico , Neoplasias da Próstata/diagnóstico , Neoplasias da Próstata/terapia , Rituximab , Vincristina
16.
Medicine (Baltimore) ; 98(50): e18393, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31852157

RESUMO

RATIONALE: Pyothorax-associated lymphoma (PAL) is a rare type of malignant pleural lymphoma. Most lymphomas are normally discovered around 20 to 50 years after tuberculosis infection. In China, there have been few reports about PAL cases so far. We report a case of a patient, whose tuberculosis and lymphoma were diagnosed concurrently. PATIENT CONCERNS: The patient, a 76-year-old male, was reported to our hospital on March 13, 2015. He had recurrent shortness of breath during the previous 2 years of routine activities solely. His symptoms became more serious which was manifested by edema of lower limbs 1 day before his admission to our hospital. DIAGNOSES: Doctors reached the diagnosis of PAL based on the patient's pathologic cell morphology and immunohistochemistry. The chest computed tomography examination revealed that there were pleural effusions on both sides, and some extent of compressive atelectasis in the lower parts of the inflamed lungs yet without space-occupying lesions. There were multiple small nodules which may be benign in the right upper lung. INTERVENTIONS: The current first-line treatment for diffuse large B-cell lymphoma is the cyclophosphamide, adriamycin, vincristine, prednisone (CHOP) protocol. Given that the patient had cardiac diseases and cardiotoxicity of anthracyclines, doctors decided to adopt rituximab with cyclophosphamide, vincristine, and prednisone chemotherapy without anthracyclines. OUTCOMES: The treatment effect was obvious after one cycle of chemotherapy. The patient's pleural and pericardial effusions were significantly reduced. With the chemotherapy protocol above continuously adopted, pleural and pericardial effusions did not increase in multiple reexaminations on October 25, 2015, February 15, 2016, and August 10, 2016. LESSONS: Analytical research revealed that chemotherapy with rituximab can increase the complete remission rate of non-Hodgkin lymphoma, reduce the possibility of failure and relapse, and prolong disease-free and overall survival. Moreover, there is no significant increase in adverse drug reactions compared with the effect of chemotherapy with CHOP alone. In the case of this patient, chemotherapy with rituximab was safe and efficacious.


Assuntos
Antineoplásicos Imunológicos/uso terapêutico , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Neoplasias Pleurais/tratamento farmacológico , Rituximab/uso terapêutico , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Empiema Pleural/etiologia , Humanos , Linfoma Difuso de Grandes Células B/diagnóstico , Masculino , Neoplasias Pleurais/diagnóstico
17.
Rev Med Chil ; 147(7): 836-841, 2019 Jul.
Artigo em Espanhol | MEDLINE | ID: mdl-31859981

RESUMO

BACKGROUND: Autoimmune hemolytic anemia (AIHA) is an uncommon disease. In its presentation, it can be severe and even lethal. There is only one clinical report concerning this pathology in Chile. OBJECTIVE: To describe the clinical characteristics and evolution of adult AIHA inpatients. MATERIALS AND METHODS: Retrospective review of clinical records of adult AIHA inpatients between January 2010 and June 2018 was done. Demographic, clinical, laboratory and therapeutic information was analyzed. A descriptive, analytical and survival analysis was performed. RESULTS: Forty-three adult patients diagnosed with AHIA were hospitalized in a period of 8 years. Median age was 63 years (range 22-86 years), mostly women (72%). Warm antibodies were detected in 36 cases (84%) and cold antibodies in seven. Seventy two percent of the patients had an underlying cause, and 58% were secondary to lymphoproliferative neoplasms. All patients except two, received steroids as initial treatment, with response in 37 (90%) of them. Three refractory patients received rituximab, with response in all of them, and relapse in one. Median follow-up was 38 months (range 2-98 months). Five year overall survival was 72%. CONCLUSION: AHIA in adults inpatients is a heterogeneous disease, mainly due to warm antibodies, and to secondary etiology.


Assuntos
Anemia Hemolítica Autoimune/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Anemia Hemolítica Autoimune/mortalidade , Anemia Hemolítica Autoimune/terapia , Azatioprina/administração & dosagem , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Rituximab/administração & dosagem , Esplenectomia , Análise de Sobrevida , Adulto Jovem
18.
Medicine (Baltimore) ; 98(51): e18139, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31860960

RESUMO

RATIONALE: Rituximab is recommended to induce remission of severe granulomatosis with polyangiitis (GPA). Plasma exchange (PE) may be considered in the setting of rapidly progressive glomerulonephritis (RPGN) with a serum creatinine increase of more than 5.6 mg/dl or diffuse alveolar hemorrhage (DAH). However, there are no sufficient studies on combination therapy with rituximab and PE in GPA. PATIENT CONCERNS: A 23-year-old woman was admitted with fever, abdominal pain, and diarrhea on suspicion of infectious colitis. Colonoscopy showed hemorrhagic colitis and antibiotic treatment was ineffective. Physical examination revealed episcleritis and skin lesions similar to Janeway lesions or Osler nodes on her palms and soles. Transesophageal echocardiogram (TEE) revealed mitral valve vegetation mimicking infective endocarditis. However, no pathogen was grown in the blood culture. Ten days after admission, blood-tinged sputum and respiratory distress developed. Imaging studies of lung, bronchoscopy, and bronchoalveolar lavage indicated DAH. Moreover, serum creatinine levels rapidly increased from 0.8 mg/dl to 6.1 mg/dl with proteinuria. DIAGNOSIS: The patient was diagnosed with GPA and non-infectious endocarditis, DAH, and RPGN, based on a biopsy which revealed pauci-immune crescentic glomerulonephritis with granuloma and leukocytoclastic vasculitis and antineutrophil cytoplasmic antibodies against proteinase 3- positivity. INTERVENTIONS: Initial methylprednisolone pulse therapy (1 g daily for 3 days) proved unsuccessful. After initiating PE, creatinine levels began to slowly decline, but DAH continued to deteriorate. Rituximab combined with PE therapy was considered. We performed PE every 2 to 3 days for 5 total treatments combined with rituximab (375 mg/m, once weekly for 4 weeks). OUTCOMES: After the combination treatment of rituximab and PE, alveolar hemorrhage stopped. Chest X-ray and laboratory data, including serum creatinine and hemoglobin, notably improved. Mitral valve vegetation was no longer observed in follow-up TEE. GPA remained stable with low dose prednisolone and immunosuppressants over a follow-up period of 5 years. LESSONS: This case suggests that the use of rituximab and concurrent PE may represent a promising combination for severe and refractory GPA.


Assuntos
Granulomatose com Poliangiite/patologia , Granulomatose com Poliangiite/terapia , Troca Plasmática/métodos , Rituximab/uso terapêutico , Anticorpos Monoclonais/uso terapêutico , Biópsia por Agulha , Colite/diagnóstico , Colite/etiologia , Colonoscopia/métodos , Terapia Combinada , Esquema de Medicação , Serviço Hospitalar de Emergência , Feminino , Seguimentos , Granulomatose com Poliangiite/diagnóstico , Humanos , Imuno-Histoquímica , Medição de Risco , Índice de Gravidade de Doença , Fatores de Tempo , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Adulto Jovem
19.
Presse Med ; 48(11 Pt 2): 319-327, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31759790

RESUMO

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease related to the formation of microvascular thrombosis and subsequent organ failure. The disease is accompanied with microangiopathic haemolytic anaemia, consumptive thrombocytopenia and lies on a severe deficiency in ADAMTS13, the von Willebrand factor-cleaving protease. In the acquired, immune-mediated form, this deficiency is due to the production of autoantibodies directed against the enzyme. Therapeutic plasma exchange has been used empirically for decades and still represents the cornerstone of TTP treatment. However, a better understanding of pathophysiological mechanisms underlying the disease has led these last years to the development of highly effective targeted therapies that might in the future restraint the use of therapeutic plasma exchange.


Assuntos
Proteína ADAMTS13/deficiência , Terapia de Alvo Molecular , Troca Plasmática , Púrpura Trombocitopênica Trombótica/terapia , Proteína ADAMTS13/imunologia , Acetilcisteína/uso terapêutico , Corticosteroides/uso terapêutico , Anemia Hemolítica/complicações , Autoanticorpos/imunologia , Ensaios Clínicos como Assunto , Previsões , Humanos , Fatores Imunológicos/uso terapêutico , Púrpura Trombocitopênica Trombótica/sangue , Púrpura Trombocitopênica Trombótica/complicações , Púrpura Trombocitopênica Trombótica/imunologia , Rituximab/uso terapêutico , Anticorpos de Domínio Único/uso terapêutico
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA