We report the first case of a patient with hepatitis C virus(HCV) infection and idiopathic thrombocytopenic purpura(ITP), who later developed acquired amegakaryocytic thrombocytopenia(AAMT), with autoantibodies to the...We report the first case of a patient with hepatitis C virus(HCV) infection and idiopathic thrombocytopenic purpura(ITP), who later developed acquired amegakaryocytic thrombocytopenia(AAMT), with autoantibodies to the thrombopoietin(TPO) receptor(c-Mpl). A 64-year-old woman, with chronic hepatitis C, developed severe thrombocytopenia and was diagnosed with ITP. She died of liver failure. Autopsy revealed cirrhosis and liver carcinoma. In the bone marrow, a marked reduction in the number of megakaryocytes was observed, while other cell lineages were preserved. Therefore, she was diagnosed with AAMT. Additionally, autoantibodies to c-Mpl were detected in her serum. Autoantibodies to c-Mpl are one of the causes of AAMT, acting through inhibition of TPO function, megakaryocytic maturation, and platelet formation. HCV infection induces several autoantibodies. HCV infection might also induce autoantibodies to c-Mpl, resulting in the development of AAMT. This mechanism may be one of the causes of thrombocytopenia in patients with HCV infection.展开更多
Background Neuropathological diagnosis of argyrophilic grain disease(AGD)is currently based primarily on the combination of argyrophilic grain(AG)visualized using Gallyas-Braak silver staining,phosphorylated tau-posit...Background Neuropathological diagnosis of argyrophilic grain disease(AGD)is currently based primarily on the combination of argyrophilic grain(AG)visualized using Gallyas-Braak silver staining,phosphorylated tau-positive pretangles,coiled bodies,and ballooned neuron detection.Although AGD is common in patients with dementia and/or prominent psychiatric symptoms,whether it is a distinct neurological disease entity or a by-product of the aging process remains unclear.Methods In 1449 serial forensic autopsy cases>40 years old(823 males and 525 females,aged 40-101 years,mean age 70.0±14.1 years),we examined the frequency and comorbid pathology of AGD cases and investigated the clini-cal appearance by comparing those with non-AGD cases using the propensity score.Results Of the 1449 cases,we detected 342 AGD cases(23.6%;mean age 79.7 years;177 males and 165 females).The AGD frequency and stage increased with age(P<0.001).Among AGD cases,80(23.4%)patients had dementia,and 51(15.2%)had a history of psychiatric hospital visits.The frequency of suicide and history of psychiatric disorders were significantly higher in AGD cases than in AGD-negative cases,matched for age,sex,and comorbidity pathol-ogy,with a relative risk of suicide of 1.72(1.30-2.26).The frequency of suicide was significantly higher in AGD cases than in non-AGD cases in female but not male cases.The relative risk of suicide increased to 2.27(1.20-4.30)and 6.50(1.58-26.76)in AGD patients with Lewy and progressive supranuclear palsy pathology,respectively,and decreased to 0.88(0.38-2.10)in those with advanced AD pathology.In AGD cases,23.4%had dementia;however,the difference was not significant after controlling for age,sex,and comorbid pathology.Conclusion Our study demonstrated that AGD is a significant and isolated risk factor for psychiatric hospital visits and suicide completion.In older adults,AGs may contribute to the progression of functional impairment of the limbic system,which leads to psychiatric disorders and suicide attempts.展开更多
文摘We report the first case of a patient with hepatitis C virus(HCV) infection and idiopathic thrombocytopenic purpura(ITP), who later developed acquired amegakaryocytic thrombocytopenia(AAMT), with autoantibodies to the thrombopoietin(TPO) receptor(c-Mpl). A 64-year-old woman, with chronic hepatitis C, developed severe thrombocytopenia and was diagnosed with ITP. She died of liver failure. Autopsy revealed cirrhosis and liver carcinoma. In the bone marrow, a marked reduction in the number of megakaryocytes was observed, while other cell lineages were preserved. Therefore, she was diagnosed with AAMT. Additionally, autoantibodies to c-Mpl were detected in her serum. Autoantibodies to c-Mpl are one of the causes of AAMT, acting through inhibition of TPO function, megakaryocytic maturation, and platelet formation. HCV infection induces several autoantibodies. HCV infection might also induce autoantibodies to c-Mpl, resulting in the development of AAMT. This mechanism may be one of the causes of thrombocytopenia in patients with HCV infection.
文摘Background Neuropathological diagnosis of argyrophilic grain disease(AGD)is currently based primarily on the combination of argyrophilic grain(AG)visualized using Gallyas-Braak silver staining,phosphorylated tau-positive pretangles,coiled bodies,and ballooned neuron detection.Although AGD is common in patients with dementia and/or prominent psychiatric symptoms,whether it is a distinct neurological disease entity or a by-product of the aging process remains unclear.Methods In 1449 serial forensic autopsy cases>40 years old(823 males and 525 females,aged 40-101 years,mean age 70.0±14.1 years),we examined the frequency and comorbid pathology of AGD cases and investigated the clini-cal appearance by comparing those with non-AGD cases using the propensity score.Results Of the 1449 cases,we detected 342 AGD cases(23.6%;mean age 79.7 years;177 males and 165 females).The AGD frequency and stage increased with age(P<0.001).Among AGD cases,80(23.4%)patients had dementia,and 51(15.2%)had a history of psychiatric hospital visits.The frequency of suicide and history of psychiatric disorders were significantly higher in AGD cases than in AGD-negative cases,matched for age,sex,and comorbidity pathol-ogy,with a relative risk of suicide of 1.72(1.30-2.26).The frequency of suicide was significantly higher in AGD cases than in non-AGD cases in female but not male cases.The relative risk of suicide increased to 2.27(1.20-4.30)and 6.50(1.58-26.76)in AGD patients with Lewy and progressive supranuclear palsy pathology,respectively,and decreased to 0.88(0.38-2.10)in those with advanced AD pathology.In AGD cases,23.4%had dementia;however,the difference was not significant after controlling for age,sex,and comorbid pathology.Conclusion Our study demonstrated that AGD is a significant and isolated risk factor for psychiatric hospital visits and suicide completion.In older adults,AGs may contribute to the progression of functional impairment of the limbic system,which leads to psychiatric disorders and suicide attempts.