{"title":"治疗一例继发于对环孢菌素和皮质类固醇联合耐药的自身免疫原因的获得性纯红细胞发育不全的未来计划","authors":"K. Kaur, M. Singh","doi":"10.52793/acmr.2020.1(1)-01","DOIUrl":null,"url":null,"abstract":"Pure red cell aplasia (PRCA) is an orphan disease, and as such there are no rationally developed standard treatments. Most of the cases are idiopathic with a subset being antibody mediated. An overlap exists among idiopathic cases with those of T cell large granular lymphocytic leukemia, hypogamma globulinemia as well a slow grade lymphoma. Here we report a case of acquired Pure red cell aplasia (aPRCA), out of viral causes, congenital disease as well as drug reactionidiopathic PRCA would be the commonest cause, with the majority of cases believed to be by auto reactive T cells. This is mainly through selective T or NK cell mediated killing of erythroid colony (CFU-E) and burst (BFU-E) forming units, thus inhibiting red cell precursor progression to mature erythrocytes (Figure1) [7,17]. endometriomas and has now displayed partial remission (PR) with a combination of prednisolone 50mg with anabolic steroid danazol 200mg bd for 4 months along with metoclopramide 10mg od and is being continued on same therapy for minimum of 18 months for getting a CR as did another case of SLE as well as a case of anky losing spondylitis. a mass in ovaries trans vaginal sonography (TVS) revealed a solid mass in left ovarymeasuring 50x35.8 mm and right ovary vague mass multiseptate 33x30.5 mm with fluid in pouch of douglas. Only latest LFT increased SGOT/PT was observed.","PeriodicalId":72085,"journal":{"name":"Advances in clinical and medical research (Chandigarh, India)","volume":" ","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2020-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Future Plans Of Treating A Case Of Acquired Pure Red Cell Aplasia Secondary To Autoimmune Causes Resistant To Combination Of Cyclosporine And Corticosteroid\",\"authors\":\"K. Kaur, M. Singh\",\"doi\":\"10.52793/acmr.2020.1(1)-01\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Pure red cell aplasia (PRCA) is an orphan disease, and as such there are no rationally developed standard treatments. Most of the cases are idiopathic with a subset being antibody mediated. An overlap exists among idiopathic cases with those of T cell large granular lymphocytic leukemia, hypogamma globulinemia as well a slow grade lymphoma. Here we report a case of acquired Pure red cell aplasia (aPRCA), out of viral causes, congenital disease as well as drug reactionidiopathic PRCA would be the commonest cause, with the majority of cases believed to be by auto reactive T cells. This is mainly through selective T or NK cell mediated killing of erythroid colony (CFU-E) and burst (BFU-E) forming units, thus inhibiting red cell precursor progression to mature erythrocytes (Figure1) [7,17]. endometriomas and has now displayed partial remission (PR) with a combination of prednisolone 50mg with anabolic steroid danazol 200mg bd for 4 months along with metoclopramide 10mg od and is being continued on same therapy for minimum of 18 months for getting a CR as did another case of SLE as well as a case of anky losing spondylitis. a mass in ovaries trans vaginal sonography (TVS) revealed a solid mass in left ovarymeasuring 50x35.8 mm and right ovary vague mass multiseptate 33x30.5 mm with fluid in pouch of douglas. Only latest LFT increased SGOT/PT was observed.\",\"PeriodicalId\":72085,\"journal\":{\"name\":\"Advances in clinical and medical research (Chandigarh, India)\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2020-05-10\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Advances in clinical and medical research (Chandigarh, India)\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.52793/acmr.2020.1(1)-01\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Advances in clinical and medical research (Chandigarh, India)","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.52793/acmr.2020.1(1)-01","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
摘要
纯红细胞再生障碍性疾病(PRCA)是一种孤儿病,因此尚无合理开发的标准治疗方法。大多数病例是特发性的,其中一部分是抗体介导的。特发性病例与T细胞大颗粒淋巴细胞白血病、低γ -球蛋白血症以及慢级淋巴瘤存在重叠。这里我们报告一例获得性纯红细胞发育不全(aPRCA),病毒、先天性疾病以及药物反应性特发性PRCA是最常见的原因,大多数病例被认为是由自身反应性T细胞引起的。这主要是通过选择性T或NK细胞介导的红细胞集落(CFU-E)和破裂(BFU-E)形成单位的杀伤,从而抑制红细胞前体向成熟红细胞的进展(图1)[7,17]。经强的松龙50mg +合成代谢类固醇达那唑200mg / d +甲氧氯普胺10mg / d联合治疗4个月,目前已显示部分缓解(PR),并继续接受相同治疗至少18个月,以获得CR,另一例SLE患者和一例失去性脊柱炎患者也是如此。卵巢肿块经阴道超声(TVS)示左卵巢实性肿块,大小为50x35.8 mm,右卵巢多间隔模糊肿块,大小为33x30.5 mm,道格拉斯囊内有液体。只有最近的LFT增加了SGOT/PT。
Future Plans Of Treating A Case Of Acquired Pure Red Cell Aplasia Secondary To Autoimmune Causes Resistant To Combination Of Cyclosporine And Corticosteroid
Pure red cell aplasia (PRCA) is an orphan disease, and as such there are no rationally developed standard treatments. Most of the cases are idiopathic with a subset being antibody mediated. An overlap exists among idiopathic cases with those of T cell large granular lymphocytic leukemia, hypogamma globulinemia as well a slow grade lymphoma. Here we report a case of acquired Pure red cell aplasia (aPRCA), out of viral causes, congenital disease as well as drug reactionidiopathic PRCA would be the commonest cause, with the majority of cases believed to be by auto reactive T cells. This is mainly through selective T or NK cell mediated killing of erythroid colony (CFU-E) and burst (BFU-E) forming units, thus inhibiting red cell precursor progression to mature erythrocytes (Figure1) [7,17]. endometriomas and has now displayed partial remission (PR) with a combination of prednisolone 50mg with anabolic steroid danazol 200mg bd for 4 months along with metoclopramide 10mg od and is being continued on same therapy for minimum of 18 months for getting a CR as did another case of SLE as well as a case of anky losing spondylitis. a mass in ovaries trans vaginal sonography (TVS) revealed a solid mass in left ovarymeasuring 50x35.8 mm and right ovary vague mass multiseptate 33x30.5 mm with fluid in pouch of douglas. Only latest LFT increased SGOT/PT was observed.