Assuntos
Azacitidina/uso terapêutico , Leucemia Mieloide Aguda/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Leucemia Mieloide Aguda/genética , Masculino , Pessoa de Meia-Idade , Mutação , Prognóstico , Taxa de SobrevidaRESUMO
Isochromosome of the long arm of chromosome 20 with loss of interstitial material [ider(20q)] is a variant of deletion of chromosome 20q and a rare abnormality in myelodysplastic syndrome (MDS). We studied seven cases with an ider(20q) in MDS. Fluorescence in situ hybridization (FISH) studies showed all proximal breakpoints to be consistently located in 20q11.21 band whereas distal breakpoints were variable. Amplification of HCK, TNFRSF6B and DIDO1 genes included in retained regions associated with loss of tumour suppressor genes in deleted regions could explain cell tumour progression and possibly the less favourable prognosis of ider(20q) compared with del(20q).
Assuntos
Cromossomos Humanos Par 20 , Isocromossomos , Síndromes Mielodisplásicas/genética , Idoso de 80 Anos ou mais , Quebra Cromossômica , Proteínas de Ligação a DNA/genética , Feminino , Amplificação de Genes , Deleção de Genes , Humanos , Hibridização in Situ Fluorescente/métodos , Masculino , Pessoa de Meia-Idade , Proteínas Proto-Oncogênicas c-hck/genética , Membro 6b de Receptores do Fator de Necrose Tumoral/genéticaAssuntos
Inversão Cromossômica , Cromossomos Humanos Par 16/ultraestrutura , Leucemia Mielomonocítica Aguda/genética , Proteínas de Fusão Oncogênica/genética , Adulto , Biomarcadores Tumorais/sangue , Feminino , Humanos , Hibridização in Situ Fluorescente , Leucemia Mielomonocítica Aguda/sangue , Masculino , Pessoa de Meia-Idade , Proteínas de Neoplasias/sangue , Células-Tronco Neoplásicas/enzimologia , Peroxidase/sangue , RNA Mensageiro/sangue , RNA Mensageiro/genética , RNA Neoplásico/sangue , RNA Neoplásico/genéticaAssuntos
Transplante de Células-Tronco Hematopoéticas , Leucemia Plasmocitária/terapia , Neoplasias do Sistema Nervoso/terapia , Adulto , Idoso , Feminino , Humanos , Leucemia Plasmocitária/diagnóstico por imagem , Neoplasias do Sistema Nervoso/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
Anaplastic, CD30+, large-cell lymphoma is now a well-recognized pathologic entity that accounts for 2% to 8% of all lymphomas. Recent progress has been made in the understanding of certain biologic features found in anaplastic large-cell lymphoma, but information about its clinical behavior, in comparison to other large-cell lymphomas, is limited. The pathologic review of a large multicenter study of the treatment of aggressive lymphoma identified 146 cases of anaplastic large-cell lymphoma (ALCL) on the basis of morphology and CD30 expression. We compared initial presentation, immunophenotype, and clinical outcome of these cases with those of the 1,695 nonanaplastic diffuse large-cell lymphomas (non-ALCL) included in the same trial. Patients with ALCL were more likely to be male (P = .018) and were younger (P < .0001) than those with non-ALCL. B symptoms were more frequent in ALCL (P = .006). Skin (P < .0001) and lung (P < .05) involvement was also more frequent in ALCL, but frequency of bone marrow involvement was identical (P = . 5). Tumor cell phenotype was B in 56 cases (38%), T in 49 cases (34%), and null in 33 cases (22%). Response to chemotherapy (P = . 001), event-free survival (P = .006), and overall survival (P = . 0004) were better for ALCL than for non-ALCL. Multivariate analyses identified anaplastic character as an independent factor that predicted a longer survival. Tumor cell phenotype did not influence event-free survival (P = .72) or overall survival (P = .83). ALCL in adults is a clinicopathologic entity which, independent of its phenotypic characteristics, has a better outcome than other diffuse large-cell lymphomas.
Assuntos
Linfoma Anaplásico de Células Grandes , Adulto , Idoso , Terapia Combinada , Feminino , Humanos , Imunofenotipagem , Linfoma Anaplásico de Células Grandes/imunologia , Linfoma Anaplásico de Células Grandes/mortalidade , Linfoma Anaplásico de Células Grandes/fisiopatologia , Linfoma Anaplásico de Células Grandes/terapia , Masculino , Pessoa de Meia-Idade , Resultado do TratamentoRESUMO
We studied the survival of 195 patients with agnogenic myeloid metaplasia (AMM) diagnosed between 1962 and 1992 in an attempt to stratify patients into risk groups. Median survival was 42 months. Adverse prognostic factors for survival were age > 60 years, hepatomegaly, weight loss, low hemoglobin level (Hb), low or very high leukocyte count (WBC), high percentage of circulating blasts, male sex, and low platelet count. A new scoring system based on two adverse prognostic factors, namely Hb < 10 g/dL and WBC < 4 or > 30 x 10(9)/L, was able to separate patients in three groups with low (0 factor), intermediate (1 factor), and high (2 factors) risks, associated with a median survival of 93, 26, and 13 months, respectively. An abnormal karyotype (32 cases of 94 tested patients) was associated with a short survival, especially in the low-risk group (median survival of 50 v 112 months in patients with normal karyotype). The prognostic factors for acute conversion were WBC > 30 x 10(9)/L and abnormal karyotype. Thus, hemoglobin level and leukocyte count provide a simple prognostic model for survival in AMM, and the adverse prognostic value of abnormal karyotype may be related to a higher rate of acute conversion.
Assuntos
Mielofibrose Primária/mortalidade , Índice de Gravidade de Doença , Adulto , Idoso , Idoso de 80 Anos ou mais , Aneuploidia , Progressão da Doença , Feminino , Seguimentos , Humanos , Cariotipagem , Leucemia Mieloide Aguda/mortalidade , Leucemia Mieloide Aguda/patologia , Contagem de Leucócitos , Masculino , Pessoa de Meia-Idade , Mielofibrose Primária/genética , Mielofibrose Primária/patologia , Prognóstico , Fatores de Risco , Análise de SobrevidaRESUMO
OBJECTIVES: To evaluate the toxicity and effectiveness of piperacillin+tazobactam and amikacin compared with a reference treatment with ceftazidime and amikacin given as first line therapy in neutropenic patients with fever. METHODS: A multicentric randomized trial was conducted in 222 adults who had fever (38 degrees C for > 3 h) during a period of aplasia (white cell count < 0.5.10(9)/l for 22.9 +/- 10.4 days) induced by chemotherapy for acute leukaemia (68.1%) or by bone marrow autograft for lymphoma, myeloma or solid tumour (30.3%). 109 patients were assigned to the piperacillin (12 g/d)/tazobactam (1.5 g/d)+amikacin group and 113 to the ceftazidime (3 g/d)+amikacin group. Evaluation criteria were the frequency of apyrexia after a 72-hour antibiotic regimen and major infectious events defined as death due to infection and severe infections causing a delay in the chemotherapy protocol. RESULTS: Data obtained in 188 patients who fulfilled all the protocol criteria were evaluated. The episode of fever was controlled better with the piperacillin/tazobactam+amikacin combination (apyrexia achieved in 60.6% of the patients vs 44.7% in the ceftazidime+amikacin group, p = 0.028) and there were fewer superinfections (23% vs 41% respectively, p < 0.008). Tolerance was similar in the two groups. In vitro, 56% of the strains resistant to piperacillin and isolated prior to treatment were sensitive to the piperacillin/tazobactam combination. Among the strains isolated (41 Gram-, 61 Gram+), 72% were sensitive to ceftazidime and 84% were sensitive to the piperacillin/tazobactam combination. There were 16 deaths due to infection (8.5%) with no difference according to antibiotic regimen. There was no difference in toxicity. CONCLUSION: Tolerance was similar in the two groups. A combined regimen of piperacillin/tazobactam can be proposed as first line treatment for neutropenic patients with fever.
Assuntos
Quimioterapia Combinada/uso terapêutico , Febre/tratamento farmacológico , Neutropenia/tratamento farmacológico , Adulto , Idoso , Amicacina/efeitos adversos , Amicacina/uso terapêutico , Ceftazidima/efeitos adversos , Ceftazidima/uso terapêutico , Quimioterapia Combinada/efeitos adversos , Febre/complicações , Febre/microbiologia , Humanos , Pessoa de Meia-Idade , Neutropenia/complicações , Neutropenia/microbiologia , Ácido Penicilânico/efeitos adversos , Ácido Penicilânico/análogos & derivados , Ácido Penicilânico/uso terapêutico , Piperacilina/efeitos adversos , Piperacilina/uso terapêutico , Tazobactam , Inibidores de beta-LactamasesRESUMO
Haemophagocytic syndrome is a heterogenous disease characterized by disordered macrophage activation associated with viral, bacterial or parasitic infection. The few reports of haemophagocytosis occurring in the presence of mycobacterial infection show a high mortality rate and we present two further cases notable for their favourable issue. Rapidity of diagnosis and immediate treatment could explain the avoidance of a fatal outcome.
Assuntos
Histiocitose de Células não Langerhans/etiologia , Tuberculose/complicações , Idoso , Idoso de 80 Anos ou mais , Anti-Infecciosos/uso terapêutico , Humanos , Masculino , Tuberculose/tratamento farmacológicoRESUMO
We report our experience of treatment of acute myeloid leukemia (AML) with inv(16). Nineteen of 531 (3.6%) cases of newly diagnosed AML karyotyped over a 12 year period had inv(16)(p13q22) and none had t(16;16) or del 16q. Morphologically, all patients had M4eo. All patients were treated with conventional anthracycline-Ara-C chemotherapy, followed by moderate or more intensive consolidation chemotherapy. All patients received central nervous system (CNS) prophylaxis with intrathecal methotrexate and Ara-C, and cranial irradiation. Eighteen patients (95%) achieved complete remission (CR). Three had a bone marrow relapse, one had a CNS relapse and 14 patients remained in first CR, 11 of them with a follow-up greater than 44 months. Disease-free survival was 74% after 10 months, and actuarial survival 88% after 4 years, and 62% after 6 years. No other AML subgroup, in our experience, had a long-term survival approaching that of AML with inv(16) (although similar favorable outcome may be anticipated in acute promyelocytic leukemia treated by a combination of retinoic acid and chemotherapy).
Assuntos
Leucemia Mieloide/diagnóstico , Doença Aguda , Aberrações Cromossômicas , Transtornos Cromossômicos , Inversão Cromossômica , Cromossomos Humanos Par 16 , Seguimentos , Humanos , Leucemia Mieloide/tratamento farmacológico , Leucemia Mieloide/genética , Prognóstico , Análise de SobrevidaRESUMO
The authors report a case of a patient who in the 24th week of a twin pregnancy became sero-positive for toxoplasmosis. This was diagnosed by cordocentesis as being infected, and the treatment was therefore started with pyrimethamine and sulfadiazine and folic acid at the 28th week of pregnancy. At 35 weeks, the patient had an acute medullary aplasia due to the absence of the folates. The mother's state was improved rapidly by giving her folinic acid and the twins were normal haematologically. In this case, the authors point out how important the folates are in a pregnancy, especially in twin pregnancies, and point out the precautions that have to be taken when treatment with pyrimethamine and sulfadiazine is started for congenital toxoplasmosis.
Assuntos
Anemia Aplástica/induzido quimicamente , Deficiência de Ácido Fólico/complicações , Ácido Fólico/uso terapêutico , Complicações Infecciosas na Gravidez/tratamento farmacológico , Gravidez Múltipla , Pirimetamina/efeitos adversos , Sulfadiazina/efeitos adversos , Toxoplasmose/tratamento farmacológico , Adulto , Anemia Aplástica/sangue , Anemia Sideroblástica , Cordocentese , Feminino , Deficiência de Ácido Fólico/tratamento farmacológico , Humanos , Leucovorina/farmacologia , Leucovorina/uso terapêutico , Gravidez , Complicações Infecciosas na Gravidez/sangue , Segundo Trimestre da Gravidez , Pirimetamina/farmacologia , Espiramicina/uso terapêutico , Toxoplasmose/sangue , Toxoplasmose/complicações , GêmeosRESUMO
From 1969 to 1985 we discovered accessory spleens in 8 patients with chronic immune thrombocytopenic purpura (ITP) who relapsed or failed after splenectomy. Imaging of accessory spleen used a liver spleen scintigraphy with heat-treated RBC labeled with Tc-99m. Platelet kinetic studies with 51Cr or 111In, including sequestration index, were performed. Five patients had accessory splenectomy. Disappearance of bleeding symptoms was achieved in the 5 splenectomized patients but with only partial response of platelet counts. These results are discussed in the context of diagnosis and therapeutic management of accessory spleens in patients with chronic immune thrombocytopenic purpura who relapsed or failed after splenectomy.
Assuntos
Púrpura Trombocitopênica Idiopática/cirurgia , Baço/anormalidades , Esplenectomia , Esplenopatias/complicações , Adolescente , Adulto , Plaquetas , Criança , Pré-Escolar , Radioisótopos de Cromo , Doença Crônica , Feminino , Humanos , Incidência , Radioisótopos de Índio , Masculino , Púrpura Trombocitopênica Idiopática/epidemiologia , Cintilografia , Recidiva , Baço/diagnóstico por imagem , Baço/patologia , Esplenopatias/diagnósticoAssuntos
Inversão Cromossômica , Cromossomos Humanos Par 16/ultraestrutura , Leucemia Mielogênica Crônica BCR-ABL Positiva/genética , Leucemia Mieloide/genética , Doença Aguda , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Humanos , Cariotipagem , Leucemia Mielogênica Crônica BCR-ABL Positiva/tratamento farmacológico , Leucemia Mieloide/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Indução de RemissãoRESUMO
Based on encouraging results of previous combination regimens, we used a combination of VM26, ifosfamide, methyl GAG, mitoxantrone (or adriamycin), high-dose (HD) methotrexate and HD Ara C to treat 18 patients with relapsed or refractory NHL. Front-line therapy had been in most of them a reinforced CHOP regimen. Twelve patients (67 per cent) responded: there were nine (50 per cent) partial responses (PR) and three (17 per cent) complete remissions (CR). Nine of these 12 responders were grafted (eight autologous, one allogeneic transplants), one relapsed before autograft could be performed and the two remaining patients were excluded from autograft because of positive bone marrow. Five of nine patients remained free of disease after 11+ to 27+ months. Response rate was higher in patients who relapsed 'off' therapy (2/3), but CR was also obtained in two refractory NHL and persisted for 11+ and 26+ months, suggesting that VIM3-ARA C was, at least partially, non-cross-resistant with front-line adriamycin-containing regimens.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma não Hodgkin/tratamento farmacológico , Proteínas Repressoras/uso terapêutico , Terapia de Salvação , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Transplante de Medula Óssea , Terapia Combinada , Citarabina/administração & dosagem , Citarabina/uso terapêutico , Relação Dose-Resposta a Droga , Feminino , Humanos , Ifosfamida/administração & dosagem , Ifosfamida/uso terapêutico , Linfoma não Hodgkin/mortalidade , Linfoma não Hodgkin/cirurgia , Masculino , Metotrexato/administração & dosagem , Metotrexato/uso terapêutico , Hemissuccinato de Metilprednisolona/administração & dosagem , Hemissuccinato de Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Mitoguazona/administração & dosagem , Mitoxantrona/administração & dosagem , Mitoxantrona/uso terapêutico , Prednisolona/administração & dosagem , Prednisolona/uso terapêutico , Proteínas Repressoras/administração & dosagem , Taxa de Sobrevida , Teniposídeo/administração & dosagemRESUMO
Two cases of hepatosplenic candidiasis (HSC) are reported occurring after protracted episodes of neutropenia, induced by chemotherapy for acute leukemia in one case and drug hypersensitivity in the other. The disease presented with persistent or recurrent fever after correction of the neutropenia and with splenomegaly. The alkaline phosphatases were elevated. The diagnosis was strongly suggested by abdominal ultrasonography, CT scan and MRI, which showed multiple hepatosplenic defects. It was confirmed by serologic tests for candidiasis, the presence, in 1 case, of circulating candida antigens, and the rapid response to amphotericin B. The diagnosis of HSC should be considered in patients with persistent fever after an episode of neutropenia. Ideally, histologic confirmation is desirable, but this is often obtainable only by open liver biopsy, an aggressive procedure in such patients. Failing this, our 2 cases stress the diagnostic value of noninvasive imaging techniques, serological testing (in particular the discovery of circulating candida antigens) and the response to amphotericin B.