Case Reports in Immunology最新文献_第6页

A First Case Report of DiGeorge Syndrome from Ethiopia Highlights Challenges in Identifying and Treating Children with Primary T-Cell Deficiencies in Low Resource Settings. 来自埃塞俄比亚的首例迪乔治综合征病例报告强调了在低资源环境中识别和治疗原发性t细胞缺陷儿童的挑战。

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Case Reports in Immunology Pub Date : 2020-02-26 eCollection Date: 2020-01-01 DOI: 10.1155/2020/8157212

Tinsae Alemayehu, Solomie Jebessa Deribessa

引用次数: 0

Posttransplantation Lymphoproliferative Disease Treated by Retransplantation. 再移植治疗移植后淋巴细胞增生性疾病。

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Case Reports in Immunology Pub Date : 2020-02-25 eCollection Date: 2020-01-01 DOI: 10.1155/2020/9403123

Ingerid Weum Abrahamsen, Bjørn Christer Grønvold, Else Marit Inderberg, Nadia Mensali, Jonas Mattsson, Tobias Gedde-Dahl

{"title":"Posttransplantation Lymphoproliferative Disease Treated by Retransplantation.","authors":"Ingerid Weum Abrahamsen, Bjørn Christer Grønvold, Else Marit Inderberg, Nadia Mensali, Jonas Mattsson, Tobias Gedde-Dahl","doi":"10.1155/2020/9403123","DOIUrl":"https://doi.org/10.1155/2020/9403123","url":null,"abstract":"Epstein-Barr virus- (EBV-) induced posttransplantation lymphoproliferative disease (PTLD) is a life-threatening complication following allogeneic stem cell transplantation. The main risk factor is anti-thymocyte globulin (ATG). Patients who fail first-line treatment with rituximab have a poor prognosis. Though adoptive transfer of EBV-specific T cells is a potentially effective option, it is not readily available. In this case report, the patient developed PTLD following transplantation for aplastic anemia using ATG as part of the conditioning. He failed rituximab treatment and developed graft failure. We were aware that the stem cell donor had a recent EBV infection prior to transplantation, whereas the patient most likely was EBV negative before transplant. We describe our strategy to meet the patient's urgent need for EBV-specific T cells, as well as new hematopoietic stem cells. The same donor was used for a second transplant, using peripheral blood stem cells. The conditioning used was thiotepa/busulfan/fludarabin with a single dose of cyclophosphamide after transplant as graft-versus-host disease (GVHD) prophylaxis. The EBV DNA levels fell when conditioning was started, and have been undetectable since day +15 and remained so till 18 months after transplantation. The patient is doing well. This case reports successful use of cyclophosphamide after transplantation as GVHD prophylaxis, preserving virus-specific immunity.","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2020-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/9403123","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37726338","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Cerebellar Ataxia Followed by Stiff Person Syndrome in a Patient with Anti-GAD Antibodies. 抗gad抗体患者小脑性共济失调并发僵硬人综合征。

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Case Reports in Immunology Pub Date : 2020-02-08 eCollection Date: 2020-01-01 DOI: 10.1155/2020/8454532

Sinali O Seneviratne, Katherine A Buzzard, Belinda Cruse, Mastura Monif

引用次数: 2

Primary Immunodeficiency with Severe Multi-Organ Immune Dysregulation. 原发性免疫缺陷伴严重多器官免疫失调。

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Case Reports in Immunology Pub Date : 2019-12-28 eCollection Date: 2019-01-01 DOI: 10.1155/2019/8746249

Tatyana Gavrilova

引用次数: 1

Fatal Hypogammaglobulinemia 3 Years after Rituximab in a Patient with Immune Thrombocytopenia: An Underlying Genetic Predisposition? 免疫血小板减少患者服用利妥昔单抗后3年致死性低丙种球蛋白血症:潜在的遗传易感性?

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Case Reports in Immunology Pub Date : 2019-12-28 eCollection Date: 2019-01-01 DOI: 10.1155/2019/2543038

Jean-François Viallard, Marie Parrens, Frédéric Rieux-Laucat

引用次数: 6

Sirolimus for the Treatment of Airway Obstruction due to Indolent T-Lymphoblastic Proliferation. 西罗莫司治疗惰性t淋巴细胞增殖引起的气道阻塞。

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Case Reports in Immunology Pub Date : 2019-12-09 eCollection Date: 2019-01-01 DOI: 10.1155/2019/1724083

Eric Moughames, Ana P Kiess, Lee M Akst, Antoine Azar

{"title":"Sirolimus for the Treatment of Airway Obstruction due to Indolent T-Lymphoblastic Proliferation.","authors":"Eric Moughames, Ana P Kiess, Lee M Akst, Antoine Azar","doi":"10.1155/2019/1724083","DOIUrl":"https://doi.org/10.1155/2019/1724083","url":null,"abstract":"Introduction: Indolent T-lymphoblastic proliferation (iT-LBP) is a rare nonmalignant entity that presents as a proliferation of T-lymphoblasts. We report a first such case with a recurrent laryngeal obstruction presentation that was successfully controlled with Sirolimus.Case presentation: This is the case of a 29-year-old female who presented with a recurrent significant lymphoid hyperplasia in the adenoid and tongue base region as well as a right cervical lymph node. After repeated adenoidectomies and tonsillectomies, and based on pathological and clinical findings she was diagnosed with iT-LBP. Trials of radiotherapy and immunotherapy with cyclosporine and rituximab all failed to control the progression of the disease. Sirolimus was finally able to restrict the growth and improve her symptoms.Conclusion: While It-LBP does not usually require treatment, it is important to report cases in which treatment was crucial for the survival of the patient, and the effective role of Sirolimus in doing so, without any major adverse effects.","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2019-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/1724083","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37574505","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 3

Deficiency of Interleukin-1 Receptor Antagonist: A Case with Late Onset Severe Inflammatory Arthritis, Nail Psoriasis with Onychomycosis and Well Responsive to Adalimumab Therapy 白细胞介素-1受体拮抗剂缺乏:1例迟发性严重炎性关节炎，甲银屑病伴甲真菌病，对阿达木单抗治疗反应良好

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Case Reports in Immunology Pub Date : 2019-08-04 DOI: 10.1155/2019/1902817

N. Kutukculer, A. Puel, S. Eren Akarcan, K. Moriya, N. Edeer Karaca, M. Migaud, J. Casanova, G. Aksu

{"title":"Deficiency of Interleukin-1 Receptor Antagonist: A Case with Late Onset Severe Inflammatory Arthritis, Nail Psoriasis with Onychomycosis and Well Responsive to Adalimumab Therapy","authors":"N. Kutukculer, A. Puel, S. Eren Akarcan, K. Moriya, N. Edeer Karaca, M. Migaud, J. Casanova, G. Aksu","doi":"10.1155/2019/1902817","DOIUrl":"https://doi.org/10.1155/2019/1902817","url":null,"abstract":"DIRA (deficiency of the IL-1Ra) is a rare condition that usually presents in the neonatal period. Patients with DIRA present with systemic inflammation, respiratory distress, joint swelling, pustular rash, multifocal osteomyelitis, and periostitis. Previously, we reported a patient with a novel mutation in IL1RN with a healthy neonatal period, a late-onset of pustular dermatosis, inflammatory arthritis, and excellent response to canakinumab treatment. Herein, we are presenting a new case of late-onset DIRA syndrome, carrying a different mutation and showing different clinical findings. This patient is the first one in the literature with the inflammatory arthritis, nail psoriasis, and onychomycosis and with her remarkable response to monoclonal antibodies. The case responded well and fully recovered after treatment with adalimumab, but not with canakinumab. The DIRA disease can lead to death from multiple organ failures and if recognized early, the treatment with replacement of the deficient protein with biologic agents induces rapid and complete remission. Therefore, clinical symptoms should be learned exactly by the pediatricians, pediatric rheumatologists, and immunologists; and molecular analysis targeting this defect must be performed as early as possible.","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2019-08-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77691644","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 12

Partial and Transient Clinical Response to Omalizumab in IL-21-Induced Low STAT3-Phosphorylation on Hyper-IgE Syndrome Omalizumab对il -21诱导的高ige综合征低stat3磷酸化的部分和短暂临床反应

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Case Reports in Immunology Pub Date : 2019-07-04 DOI: 10.1155/2019/6357256

C. D. Alonso-Bello, M. Jiménez-Martínez, M. E. Vargas-Camaño, S. Hierro-Orozco, M. Ynga-Durand, L. Berrón-Ruiz, J. C. Alcántara-Montiel, L. Santos‐Argumedo, Diana Andrea Herrera-Sánchez, Fernando Lozano-Patiño, María Isabel Castrejón-Vázquez

引用次数: 10

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Case Reports in Immunology Pub Date : 2019-06-18 DOI: 10.1155/2019/3618510

Nooraldin Merza, A. Taha, J. Lung, Anthony W. Benderman, S. Wright

{"title":"IgG4-Related Sclerosing Disease Causing Spinal Cord Compression: The First Reported Case in Literature","authors":"Nooraldin Merza, A. Taha, J. Lung, Anthony W. Benderman, S. Wright","doi":"10.1155/2019/3618510","DOIUrl":"https://doi.org/10.1155/2019/3618510","url":null,"abstract":"Immunoglobulin G4-related disease (IgG4-RD) is known for forming soft tissue mass lesions that may have compressive effects. It is an extremely rare disease that most frequently affects the pancreas causing autoimmune pancreatitis. It can also affect the gallbladder, salivary glands, and lacrimal glands causing respective organ-specific complications. In our report, we describe an IgG4-RD case that affected the spinal cord. A 60-year-old female presented with cervical spinal cord compression caused by IgG4-RD leading to several neurological deficits. Pathological examination of the excisional biopsy of the mass revealed dense lymphoplasmacytic cells infiltration and stromal fibrosis with IgG4 and plasma cells. The patient showed a dramatic response to the administration of systemic steroids with almost resolution of her neurological symptoms. This case highlights the first case in literature for IgG4-RD of the extradural tissue causing spinal compression. Hereby, we also demonstrate the dramatic response of IgG4-RD to the administration of systemic steroids as the patient had no recurrence after 5 years of close follow-up, the longest reported period of follow-up reported in the literature to date.","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2019-06-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82534311","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 8

Severe Myositis, Myocarditis, and Myasthenia Gravis with Elevated Anti-Striated Muscle Antibody following Single Dose of Ipilimumab-Nivolumab Therapy in a Patient with Metastatic Melanoma. 转移性黑色素瘤患者单剂量伊匹单抗-纳武单抗治疗后抗横纹肌抗体升高的严重肌炎、心肌炎和重症肌无力

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Case Reports in Immunology Pub Date : 2019-04-30 eCollection Date: 2019-01-01 DOI: 10.1155/2019/2539493

Mahdieh Fazel, Patrick M Jedlowski

{"title":"Severe Myositis, Myocarditis, and Myasthenia Gravis with Elevated Anti-Striated Muscle Antibody following Single Dose of Ipilimumab-Nivolumab Therapy in a Patient with Metastatic Melanoma.","authors":"Mahdieh Fazel, Patrick M Jedlowski","doi":"10.1155/2019/2539493","DOIUrl":"https://doi.org/10.1155/2019/2539493","url":null,"abstract":"Immune checkpoint inhibitors targeting programmed cell death protein 1 and cytotoxic T-lymphocyte associated protein 4 have improved survival in patients with metastatic melanoma, especially in combination (i.e., ipilimumab-nivolumab). Postmarketing surveillance has identified rare but at times life-threatening adverse effects associated with these agents in combination and as monotherapy, which include myocarditis, myositis, myasthenia gravis (MG), and hepatotoxicity. Further evaluation of immune checkpoint therapy-induced MG identified the rapid clinical progression, prolonged treatment/supportive therapy course, and higher frequency of myasthenic crisis in these patients versus those with idiopathic MG. More rapid incorporation of aggressive treatment options (i.e., intravenous immunoglobulin, plasmapheresis) may be necessary in these cases. Anti-striational antibodies are often detected in individuals with myasthenia gravis and concurrent myositis and myocarditis. A high-index of suspicion is necessary to assist with rapid treatment initiation as these patients can rapidly deteriorate into respiratory compromise. A case of a 78-year-old woman with metastatic melanoma status after combination therapy with ipilimumab-nivolumab that developed transaminitis, myositis, myocarditis, and myasthenia gravis (with positive anti-striational antibodies) five days after the first cycle, is presented. Despite high dose intravenous methylprednisolone and intravenous immunoglobulin treatment, she ultimately entered hospice care eight days after hospital admission, 36 days after her first cycle.","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2019-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/2539493","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37320191","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 40