Chronic immune thrombocytopenic purpura (ITP) is a condition based on an immune-mediated mechanism that determines the premature hyperdestruction of the thrombocytes in peripheral blood, as well as their deficient syn...Chronic immune thrombocytopenic purpura (ITP) is a condition based on an immune-mediated mechanism that determines the premature hyperdestruction of the thrombocytes in peripheral blood, as well as their deficient synthesis at the level of the bone marrow. The chronic immune purpura could be of primary, idiopathic cause, as well as of secondary cause, occurring in the context of other pathologies. The characteristic of the primary form of the disease is the presence of isolated thrombocytopenia, defined by a platelet count under 100,000/mm3 in peripheral blood, in the absence of supporting causes for thrombocytopenia. In the secondary form of the disease, the decreased platelet count is due to associated pathologies involving an immune mechanism, responsible for the occurrence of thrombocytopenia. This study aims to emphasize the involvement of autoimmune diseases, such as systemic lupus erythematosus (SLE), dermatomyositis, rheumatoid polyarthritis or antiphospholipid syndrome in the pathogenesis of secondary thrombocytopenia. Furthermore, the study was conducted on a sample of 40 patients, divided into two groups: The first group comprising asymptomatic patients diagnosed with thrombocytopenia following routine tests, and the second group comprising patients with hemorrhagiparous symptomatology (petechiae, ecchymoses, epistaxis, gingivorrhagia), who went to the doctor in order to determine the etiology of the hemorrhagiparous syndrome. The average value of the thrombocytopenia of the patients included in the study was of 60.20 ± 19.75 × 103/μL. Laboratory investigations performed in order to establish the etiology of thrombocytopenia showed that 80% of patients presented positive antiplatelet antibodies. Moreover, 20% of the patients in the study showed positive anti-double-stranded DNA, 20% were identified with IgG anticardiolipin antibodies, while antinuclear antibodies were present in 10% of the patients.展开更多
Background Adult chronic idiopathic thrombocytopenic purpura (ITP) is a common hematologic disease characterized by persistent thrombocytopenia. So far, there were only a few reports on adult Chinese patients with ch...Background Adult chronic idiopathic thrombocytopenic purpura (ITP) is a common hematologic disease characterized by persistent thrombocytopenia. So far, there were only a few reports on adult Chinese patients with chronic ITP. This study aimed at defining the treatment outcome and prognostic factors for chronic ITP based on a large cohort of Chinese patients followed up for over 25 years at a single center Methods The medical records of 1791 patients aged 14 years or older who were diagnosed as having chronic ITP at our hospital from 1974 to 1999 were retrospectively analyzed Results The female to male ratio was 2∶1, with a median age of 34 years (ranging from 14 to 80 years), median platelet count of 38×10 9/L [range (1-99)×10 9/L], and median follow up of 36 months (range 1-220 months) Steroids were used in 689 patients, among them 209 (30.3%) achieved complete remission (CR) A splenectomy was performed in 124 patients, and response to steriod pre splenectomy was not available in 14 patients The CR rate after a splenectomy was lower in steroid nonresponders (29 of 90, 32.2%) than in those who relapsed after successful steroid treatment (12 of 20, 60.0%) ( P <0 05) In comparison with patients negative for antinuclear antibody (ANA), those who were ANA positive had similar responses to steroids, but a significantly shorter remission period after a splenectomy ( P <0 01) Conclusions Adult Chinese chronic ITP patients can have long term remission after steroid therapy and splenectomies Primary steroid refractoriness is a prognostic factor predicting poor subsequent response to a splenectomy展开更多
文摘Chronic immune thrombocytopenic purpura (ITP) is a condition based on an immune-mediated mechanism that determines the premature hyperdestruction of the thrombocytes in peripheral blood, as well as their deficient synthesis at the level of the bone marrow. The chronic immune purpura could be of primary, idiopathic cause, as well as of secondary cause, occurring in the context of other pathologies. The characteristic of the primary form of the disease is the presence of isolated thrombocytopenia, defined by a platelet count under 100,000/mm3 in peripheral blood, in the absence of supporting causes for thrombocytopenia. In the secondary form of the disease, the decreased platelet count is due to associated pathologies involving an immune mechanism, responsible for the occurrence of thrombocytopenia. This study aims to emphasize the involvement of autoimmune diseases, such as systemic lupus erythematosus (SLE), dermatomyositis, rheumatoid polyarthritis or antiphospholipid syndrome in the pathogenesis of secondary thrombocytopenia. Furthermore, the study was conducted on a sample of 40 patients, divided into two groups: The first group comprising asymptomatic patients diagnosed with thrombocytopenia following routine tests, and the second group comprising patients with hemorrhagiparous symptomatology (petechiae, ecchymoses, epistaxis, gingivorrhagia), who went to the doctor in order to determine the etiology of the hemorrhagiparous syndrome. The average value of the thrombocytopenia of the patients included in the study was of 60.20 ± 19.75 × 103/μL. Laboratory investigations performed in order to establish the etiology of thrombocytopenia showed that 80% of patients presented positive antiplatelet antibodies. Moreover, 20% of the patients in the study showed positive anti-double-stranded DNA, 20% were identified with IgG anticardiolipin antibodies, while antinuclear antibodies were present in 10% of the patients.
文摘Background Adult chronic idiopathic thrombocytopenic purpura (ITP) is a common hematologic disease characterized by persistent thrombocytopenia. So far, there were only a few reports on adult Chinese patients with chronic ITP. This study aimed at defining the treatment outcome and prognostic factors for chronic ITP based on a large cohort of Chinese patients followed up for over 25 years at a single center Methods The medical records of 1791 patients aged 14 years or older who were diagnosed as having chronic ITP at our hospital from 1974 to 1999 were retrospectively analyzed Results The female to male ratio was 2∶1, with a median age of 34 years (ranging from 14 to 80 years), median platelet count of 38×10 9/L [range (1-99)×10 9/L], and median follow up of 36 months (range 1-220 months) Steroids were used in 689 patients, among them 209 (30.3%) achieved complete remission (CR) A splenectomy was performed in 124 patients, and response to steriod pre splenectomy was not available in 14 patients The CR rate after a splenectomy was lower in steroid nonresponders (29 of 90, 32.2%) than in those who relapsed after successful steroid treatment (12 of 20, 60.0%) ( P <0 05) In comparison with patients negative for antinuclear antibody (ANA), those who were ANA positive had similar responses to steroids, but a significantly shorter remission period after a splenectomy ( P <0 01) Conclusions Adult Chinese chronic ITP patients can have long term remission after steroid therapy and splenectomies Primary steroid refractoriness is a prognostic factor predicting poor subsequent response to a splenectomy
