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Background: Extracorporeal photopheresis (ECP) is a second-line therapy for steroid-refractory chronic graft-versus-host disease (cGVHD). Objective: We describe the long-term efficacy and tolerability of ECP according to the cutan...
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Background: Extracorporeal photopheresis (ECP) is a second-line therapy for steroid-refractory chronic graft-versus-host disease (cGVHD). Objective: We describe the long-term efficacy and tolerability of ECP according to the cutaneous phenotype of cGVHD and report on the reduced need for immunosuppressant drugs in this setting. Patients and Methods: Fourteen patients (8 females) with cutaneous and/or mucosal cGVHD, treated with ECP between October 2010 and May 2016 within a single center, were included. Final analyses included patients who had received ECP for at least 12 months. We prospectively evaluated the efficacy of ECP using lesion-specific clinical scores and by recording changed doses of systemic immunosuppressants. Results: Of the 14 patients, sclerotic skin lesions were present in 10 (71%). The mRODNAN score decreased in all patients from month 9 onwards, with 40 and 77% reductions at 12 and 36 months, respectively. Six patients (43%) presented with cutaneous lichenoid lesions: this score was reduced in all patients by month 3, reaching a 93% reduction by month 12. Five patients (36%) experienced oral mucosal lichenoid lesions: these scores were decreased by 55% at month 12 and by 100% by month 33. The use of systemic immunosuppressants was reduced in all patients; 4 patients could stop all immunosuppressant drugs after 2 years. ECP was stopped in 3 patients after a complete response. No major ECP-associated adverse effects were observed. Discussion and Conclusion: ECP was an effective long-term therapy for oral and cutaneous cGVHD: consequently, dose levels of therapeutic immunosuppression could be reduced. (C) 2018 S. Karger AG, Basel.
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Graft-versus-host disease has been reported to occur rarely in syngeneic hematopoietic stem cell transplant recipients. Clinical and histological changes consistent with graft-versus-host disease have been reported to occur in thi...
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Graft-versus-host disease has been reported to occur rarely in syngeneic hematopoietic stem cell transplant recipients. Clinical and histological changes consistent with graft-versus-host disease have been reported to occur in this patient population. We report a case of a 46-year-old Caucasian male with diffuse large B-cell lymphoma in complete remission who underwent a syngeneic hematopoietic stem cell transplant. He was diagnosed with grade III acute skin and gastrointestinal graft-versus-host disease requiring high-dose corticosteroids and immunosuppressive therapy and resulting in a complete response. Syngeneic graft-versus-host disease is an anomaly that needs to be considered as a differential diagnosis of patients experiencing dermatitis, gastroenteritis, or hepatitis after an identical twin hematopoietic stem cell transplant.
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Cutaneous chronic graft-versus-host disease (GvHD) has a broad spectrum of clinicopathological presentations, the most common ones being poikiloderma, lichen planus-like eruptions, lichen sclerosus-like lesions, morphea-like plaqu...
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Cutaneous chronic graft-versus-host disease (GvHD) has a broad spectrum of clinicopathological presentations, the most common ones being poikiloderma, lichen planus-like eruptions, lichen sclerosus-like lesions, morphea-like plaques, and deep sclerosis. New forms of chronic cutaneous GvHD with different clinicopathological characteristics have been described, most of them mimicking cutaneous manifestations of autoimmune diseases. We report the case of a 35-year-old man who underwent allogenic stem cell transplantation for a therapy-associated acute myeloid leukemia and developed an acute GvHD with involvement of skin and gastrointestinal tract. He subsequently presented with chronic sclerodermatous cutaneous GvHD, followed by the appearance of indurated erythematous papules and plaques located on his back, resembling the nodular/keloidal form of cutaneous scleroderma on both clinical and histopathological grounds. This peculiar clinicopathologic presentation of chronic cutaneous GvHD was never described previously.
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ABSTRACTAllogeneic haematopoietic stem cell transplantation is increasingly used in the treatment of malignant and non-malignant disorders. Despite ongoing advances in the field, morbidity and mortality related to graft-versus-hos...
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ABSTRACTAllogeneic haematopoietic stem cell transplantation is increasingly used in the treatment of malignant and non-malignant disorders. Despite ongoing advances in the field, morbidity and mortality related to graft-versus-host disease remains a major barrier to its application. Graft-versus-host disease is a difficult-to-diagnose disease. Dermatologists are involved due to its diverse cutaneous expression. In order to appropriately diagnose, classify and treat this complex disease, knowledge of its expanding cutaneous expression is required. This review provides a synopsis of the clinical manifestations of acute, lichenoid and sclerodermatous phases of graft-versus-host disease with a look at the current evidence surrounding its differential diagnosis.
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Purpose: To identify risk factors for ocular graft-versus-host disease (oGVHD) in children with graft-versus-host disease (GVHD). Methods: This retrospective cohort study identified 38 children diagnosed with GVHD who underwent an...
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Purpose: To identify risk factors for ocular graft-versus-host disease (oGVHD) in children with graft-versus-host disease (GVHD). Methods: This retrospective cohort study identified 38 children diagnosed with GVHD who underwent an ophthalmological examination. Survival to onset of oGVHD after transplant was analyzed using Kaplan-Meier analyses with log-rank tests. A multivariable Cox proportional hazards model was run for time to oGVHD using univariate risk factors. Results: The average age was 10.0 +/- 5.4 years at the time of transplant. Underlying illness was acute lymphoblastic leukemia in 19 (50%) and acute myeloid leukemia in 8 (21%). Nonocular GVHD organ involvement included skin (84%), lungs (16%), intestines (50%), liver (24%), and bone marrow (3%). Fifteen children (39%) had oGVHD, of which 47% were asymptomatic. oGVHD was diagnosed 601 +/- 878 days after GVHD. A significant association between risk of oGVHD and diagnosis of acute lymphoblastic leukemia (P = 0.10) or acute myeloid leukemia (P = 0.08) was not found. Organ involvement associated with oGVHD included skin (P = 0.03) and lungs (P = 0.02). Survival curves were significantly influenced by GVHD organ involvement (P = 0.02), but not underlying disease (P = 0.51). The adjusted Cox regression model yielded an independent hazard ratio of 8.82 (95% CI: 1.51-51.49; P = 0.016) for the presence of concomitant GVHD involvement of skin, lungs, and another organ. Conclusions: Children with multiorgan GVHD involvement including skin and lung disease are at increased risk for oGVHD. Given the proportion of asymptomatic cases found in this series, regular eye examinations are warranted in this population.
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Chronic Graft Versus Host Disease (cGVHD) occurs in over 50-70% of patients undergoing hematopoietic stem-cell transplantation (HSCT) and is the leading cause of late non-relapse mortality. cGVHD typically has insidious multi-orga...
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Chronic Graft Versus Host Disease (cGVHD) occurs in over 50-70% of patients undergoing hematopoietic stem-cell transplantation (HSCT) and is the leading cause of late non-relapse mortality. cGVHD typically has insidious multi-organ involvement and has been associated with a worse quality of life, functional status, and increased risk of subsequent comorbidities. The last several years have seen advances in the understanding of the disease, which provided a framework for the design of transla-tional and clinical studies with newer agents currently at different phases which that may hopefully change the course of the disease. This review provides an overview of more commonly used and newer second line options for the management of cGVHD.
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Our ability to give a patient an accurate risk assessment for GvHD is improving. Likewise, novel approaches to GvHD prevention and treatment are allowing for more intensive regimens for patients at greater risk. The ultimate goal ...
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Our ability to give a patient an accurate risk assessment for GvHD is improving. Likewise, novel approaches to GvHD prevention and treatment are allowing for more intensive regimens for patients at greater risk. The ultimate goal of individualizing therapy based on risk has not yet been achieved, but this goal is in sight.
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