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1.
Clin Ter ; 161(2): 169-71, 2010.
Artigo em Italiano | MEDLINE | ID: mdl-20499034

RESUMO

The Budd-Chiari Syndrome (BCS) and the splanchnic vein thrombosis are characterized by hepatic venous outflow obstruction, generally due to venous thrombosis. These rare diseases are usually caused by multiple concurrent factors, including acquired and inherited thrombophilias. Since the diagnosis of myeloproliferative neoplasms (MPNs) is often difficult in patients with BCS and splanchnic vein thrombosis because of spleen enlargement, secondary pancytopenia and bleeding disorders, recent observations have included in the diagnostic work-up the analysis of the JAK2 mutation. The revision of several recent reports clarify the importance of the JAK2V617F detection in the diagnostic work-up of the BCS and splanchnic vein thrombosis, allowing the demonstration of masked MPNs among these cases that may benefit, in the near future, of target molecular therapies directed toward the JAK2 mutation.


Assuntos
Neoplasias da Medula Óssea/diagnóstico , Neoplasias da Medula Óssea/genética , Síndrome de Budd-Chiari/diagnóstico , Síndrome de Budd-Chiari/genética , Janus Quinase 2/genética , Transtornos Mieloproliferativos/diagnóstico , Transtornos Mieloproliferativos/genética , Circulação Esplâncnica , Trombose Venosa/diagnóstico , Trombose Venosa/genética , Diagnóstico Diferencial , Humanos
2.
Clin Ter ; 157(2): 105-9, 2006.
Artigo em Italiano | MEDLINE | ID: mdl-16817498

RESUMO

AIMS: To analyze clinical and laboratory features at presentation in correlation to treatment response and overall survival; evaluation of different treatment approaches. METHODS: The data of 151 consecutive HCL patients observed between 1982 and 2005 were retrospectively analyzed. RESULTS: The following data at presentation were analyzed and compared to response, DFS, PFS and OS: Hb < 10 g/dl (observed in 27% of patients); Plt < 100,000/microl (72%); WBC > 10,000/microl (15%); Splenomegaly (75%); Bone marrow involvement > 70% (27%). At univariate analysis only WBC > 10,000/microl resulted significantly correlated to reduced PFS. 88 Pts received as first line treatment alpha2-interferon (IFN) alone, 49 purine analogues (PA) alone or in combination with IFN, 5 were treated with splenectomy. Among IFN treated patients CR, PR and SD were obtained in 21.6%, 73.8%, 4.5% respectively of the patients; while among PA treated patients in: 26.5%, 71.4%, 2.0% respectively. DFS was significantly prolonged in patients treated with PA with respect to IFN. No significant difference in OS was observed. Median PFS was 27.6 months, median OS is projected at 238 months after a median follow up of 131 months. CONCLUSIONS: Among the routine clinical and hematochemical baseline features only the presence of WBC > 10,000/microl was correlated to a lower PFS. First line treatment with purine analogues is correlated to prolonged PFS and DFS with respect to IFN; nevertheless no difference is observed in OS.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia de Células Pilosas/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Medula Óssea/patologia , Feminino , Seguimentos , Hemoglobinas/metabolismo , Humanos , Interferon-alfa/administração & dosagem , Leucemia de Células Pilosas/sangue , Leucemia de Células Pilosas/mortalidade , Leucemia de Células Pilosas/patologia , Contagem de Leucócitos , Masculino , Pessoa de Meia-Idade , Pentostatina/administração & dosagem , Contagem de Plaquetas , Prognóstico , Estudos Retrospectivos , Esplenectomia , Análise de Sobrevida
3.
Eur J Haematol ; 76(3): 210-6, 2006 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-16412138

RESUMO

Idiopathic thrombocytopenic purpura (ITP) is often diagnosed in the elderly (age >or=65 yr), where it generally presents as a chronic disease. The objective of the present study was to describe the natural history of ITP in the elderly and to evaluate the risk of bleeding and the possible occurrence of other pathologies. We retrospectively evaluated 178 ITP patients (82 men, 96 women; mean age: 72 yr) diagnosed between 1981 and 1998. Therapy was started at diagnosis or during follow-up, depending on the platelet count and/or bleeding events. Sixty-six out of one hundred and seventy-eight patients (37%) initiated therapy at diagnosis; whereas in 11 of the 112 untreated patients (9.8%) therapy was necessary during the follow-up. Low-dose of prednisone was the first-line treatment in all patients (mean daily dose of 0.43 mg/kg). Forty-nine (63.6%) of the seventy-seven treated patients showed a response, 14 of these (28.6%) suffered a relapse. Another pathology occurred in 19 of the 178 patients (10.7%). We conclude that low-dose prednisone is an appropriate initial treatment for elderly persons. We also stress that an adequate follow-up is advisable, given that isolated thrombocytopenia could in some cases be the first sign of another underlying pathology.


Assuntos
Prednisona/uso terapêutico , Púrpura Trombocitopênica Idiopática/patologia , Idoso , Idoso de 80 Anos ou mais , Doenças Autoimunes , Progressão da Doença , Avaliação de Medicamentos , Feminino , Hemorragia/etiologia , Humanos , Incidência , Masculino , Síndromes Mielodisplásicas , Contagem de Plaquetas , Prognóstico , Púrpura Trombocitopênica Idiopática/complicações , Púrpura Trombocitopênica Idiopática/epidemiologia , Púrpura Trombocitopênica Idiopática/terapia , Estudos Retrospectivos , Resultado do Tratamento
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