Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018133 (graft-versus-host disease)
 18,032 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This report discusses the different types of apheresis used to treat skin diseases and focuses specifically on photopheresis or ECP (extracorporeal photochemotherapy). ECP is a systemic immunomodulatory therapy used successfully to treat many different, mainly autoimmune diseases such as cutaneous T-cell lymphomas, graft-versus-host disease, systemic sclerosis, atopic dermatitis, and pemphigus vulgaris. It has also proved effective against graft rejection after transplant. The exact mechanism by which the ECP performs its therapeutic activity is not yet entirely clear. However, at least 2 mechanisms have been identified that may explain the therapeutic effect. Firstly, ECP is able to induce downregulation of the self-allogeneic immune response that occurs in graft-versus-host disease, systemic sclerosis and atopic dermatitis, and secondly, it can stimulate an immune response against the neoplastic clones in patients with cutaneous T-cell lymphomas. At the Dermatology Clinic of the University of Siena, more than 100 patients with different immune-mediated diseases were treated with ECP. The results obtained are very interesting and our 20 years of experience confirm that the treatment is well tolerated. ECP can therefore be considered of great utility in the modulation of the immune system.
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PMID:[Apheresis in dermatologic diseases]. 2238 42

Extracorporeal photopheresis (ECP or photopheresis) is an advanced therapeutic apheresis procedure in which blood is separated into its various components and the isolated buffy coat is treated with 8-methoxypsoralen (a photoactivating drug), exposed to ultraviolet light and returned to the patient. All other remaining blood components are also returned to the patient. The purpose of this procedure is immunomodulation. The treated leukocytes, specifically T-cells, are returned to the patient's circulation and will induce cytotoxicity and reduce proliferation of new T-cells. In the United States, ECP was initially approved for the treatment of cutaneous T-cell lymphoma by the US Food and Drug Administration in the late 1980s. Since that time, it has been used as an "off-label" therapy to treat several other autoimmune diseases in the United States and even more extensively in Europe and Asia. The following review is limited to the current clinical use of ECP in cutaneous T-cell lymphoma, Crohn's disease, systemic sclerosis, graft versus host disease, and emerging data on nephrogenic systemic fibrosis.
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PMID:Extracorporeal photopheresis: clinical use so far. 2246 83

The optimal therapy for steroid-refractory (SR) acute graft-versus-host disease (aGVHD) is undefined. We studied patients with SR aGVHD, comparing extracorporeal photopheresis (ECP; n = 57) and anticytokine therapy (n = 41). In multivariate analyses, ECP, adjusted for steroid dose (odds ratio, 3.42; P = .007), and grade >II aGVHD (odds ratio, 68; P < .001) were independent predictors of response. ECP therapy, adjusted for conditioning regimen intensity and steroid dose, was associated with superior survival (hazard ratio [HR], 4.6; P = .016) in patients with SR grade II aGVHD. Grade >II aGVHD at onset of salvage therapy (HR, 9.4; P < .001) and lack of response to therapy (HR, 3.09; P = .011) were associated with inferior survival. These findings require validation in a prospective randomized study.
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PMID:Extracorporeal photopheresis versus anticytokine therapy as a second-line treatment for steroid-refractory acute GVHD: a multicenter comparative analysis. 2362 92

DLIs are frequently used following haematopoietic SCT (HSCT) in patients with risk of relapse but data on GVHD following DLI are scarce. We report on 68 patients who received DLI following HSCT. Most patients developed GVHD following DLI (71%), which was acute in 22 patients (32%) almost half of whom had grade III-IV acute GVHD (aGVHD). Thirty patients (44%) developed cGVHD which followed aGVHD in four patients and was graded severe in nine patients. Corticosteroids were the most common first-line therapy for both acute and chronic GVHD. A wide range of second/third-line agents included cyclosporin, mycophenolate, tacrolimus, imatinib, infliximab and ECP. Relapse of initial malignancy occurred in 37%. Relapse was significantly less frequent in those receiving pre-emptive DLI. Relapse rates were also lower in those with GVHD (31%) than those without GVHD (50%), but this did not reach statistical significance. At 55 months post DLI, 34% of patients had died most commonly from relapse and 22% had on-going GVHD. Although GVHD was an important cause of morbidity post DLI (71%), only 6% died from GVHD. Although most patients develop GVHD post DLI and may require consecutive therapies, mortality from GVHD is infrequent. DLI remains an important option for relapse post transplant and manipulation of the GVT effect needs to be optimised to induce remission without morbidity from GVHD.
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PMID:A multicentre UK study of GVHD following DLI: rates of GVHD are high but mortality from GVHD is infrequent. 2531 Mar 8

Acute graft versus host disease (GVHD), a common complication after allogeneic hematopoietic cell transplantation (HCT), occurs in as many as 70% of recipients of this life saving treatment. Front line therapy for GVHD with corticosteroids will fail in up to 40% of patients, which leads to high morbidity and mortality. Traditional prevention and treatment strategies have focused on reducing alloreactivity, typically with therapy to reduce cytotoxic T-cell function. Emerging evidence exists that promotion of regularly T-cell function, through treatments such as extracorporeal photopheresis, is effective for GVHD treatment and has potential for prevention as well. This review will focus on literature reporting the success of ECP for steroid refractory acute GVHD and the potential for delivery of ECP in the early pre and post-transplant periods that shows promise as a less immunosuppressive strategy to reduce rates of acute GVHD.
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PMID:Extracorporeal photopheresis in prevention and treatment of acute GVHD. 2574 31

BACKGROUND Toxic epidermal necrolysis (TEN) is characterized by widespread erythematous and bullous lesions on the skin. Nowadays, considerable progress has been made in the understanding of its pathogenesis. Immunologically it is similar to graft-versus-host disease. Therefore, we may propose that TEN is a disorder of cell-mediated immunity. CASE REPORT Our patient was a 74-year-old white female who had pneumonia and was positive for hepatitis C virus (HCV), and who had been on levofloxacin therapy. After the first levofloxacin dose, erythematous dusky red macules occurred on her extremities and trunk, and on the following day, confluent purpuric lesions tended to run together over 85% of her body. Her biopsy results indicated TEN. Laboratory testing for serum ECP (eosinophil cationic peptide) and serum immunoglobulin (Ig) levels were performed, and blister fluid was investigated. The patient responded positively to omalizumab treatment and after treatment laboratory tests revealed decreased high sensitive CRP, ECP, IgG1, IgG2, IgG3, IgG4, IgA, and IgM levels. CONCLUSIONS To the best of our knowledge, this is the first case of a patient with HCV who developed cutaneous adverse drug reaction on levofloxacin medication and recovered with omalizumab treatment. This is the first documentation of omalizumab treatment of a TEN patient.
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PMID:Levofloxacin Induced Toxic Epidermal Necrolysis: Successful Therapy with Omalizumab (Anti-IgE) and Pulse Prednisolone. 2763 12

The incidence of bronchiolitis obliterans syndrome (BOS), a devastating manifestation of chronic graft-versus-host-disease, may rise globally due to steady increases in utilization of allogeneic hematopoietic cell transplantation (HCT). Though some advances have occurred in the past decade regarding understanding of the pathogenesis, diagnosis and treatment of BOS, the overall mortality and morbidity remain very high. We sought to determine the current diagnostic and therapeutic challenges, which can potentially hinder optimal management of BOS both in developed and developing countries. We performed a comprehensive systematic review of both modern diagnostic modalities and treatments and then assessed which of them would be universally accessible. The 2014 National Institutes of Health chronic GVHD criteria remains the gold standard tool for diagnosing BOS. Important elements of treatment involve early and accurate detection, as well as utilizing the treatment modalities with known (but variable efficacy) e.g. fluticasone-azithromycin-montelukast [FAM] combination, etanercept, extra-corporeal photopheresis [ECP], lung transplantation, and prompt treatment of complications including infections in sufferers of BOS. Our results indicate that optimum diagnostic tools are not readily available in some parts of the world for early detection, which include a lack of CT scanners, unavailability of pulmonary function testing tools, absence of sub-specialists, lack of certain effective treatments and late referral for lung transplant. We present a systematic review of current literature along with recommendations for available therapies to guide practitioners to optimize the long-term outcomes in HCT survivors regardless of access to experts and expensive therapies.
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PMID:Diagnosis and treatment of bronchiolitis obliterans syndrome accessible universally. 3003 55


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