Stem cell transplantation for fanconi anemia: 20 years of progressive decrease in the dose of cyclophosphamide without irradiation

La fesomia actual és de finals del segle XVIII.Primer pla, contrapicat, d'un senzill edifici d'habitatges entre mitgeres. Consta de planta baixa, quatre plantes pis i terrat, amb un volum reculat respecte el pla de façana. S'organitza en tres eixos verticals. Combina balcons amb finestretes

Confounding factors affecting the National Institutes of Health (NIH) chronic Graft-Versus-Host Disease Organ-Specific Score and global severity

The 2005 NIH chronic GVHD (cGVHD) organ severity is based on the assessment of current status regardless of whether abnormalities are due to GVHD. The score assignment does not require knowledge of past manifestations, attribution or whether cGVHD is still active. The aim of this study is to describe confounding factors affecting organ scores in patients with cGVHD. The study included 189 consecutive cGVHD patients evaluated at our center in 2013. Providers completed the NIH 0-3 organ-specific scoring evaluation with two questions added for each organ to identify abnormalities that were (i) not attributed to cGVHD or (ii) attributed to cGVHD plus other causes. Abnormalities attributed to causes other than GVHD were recorded. Eighty (14%) abnormalities were not attributed to cGVHD in at least one organ, and 41 (7%) abnormalities were attributed to cGVHD plus other causes in at least one organ. A total of 436 (78%) abnormalities were attributed only to cGVHD. Abnormalities not attributed to cGVHD were observed most frequently in the lung, gastrointestinal tract and skin. Most common abnormalities included pre-transplant condition, sequelae from GVHD, deconditioning, infections and medications. Our results support the 2014 NIH consensus recommendation to consider attribution when scoring organ abnormalities

Brazilian Workshop Model To Train Investigators In Chronic Graft-versus-host Disease Clinical Trials According To The 2005-2006 National Institutes Of Health Recommendations

Background: The lack of standardization of clinical diagnostic criteria, classification and severity scores of chronic graft-versus-host disease led the National Institutes of Health to propose consensus criteria for the purpose of clinical trials. Method: Here we describe a one-day workshop model conducted by the Chronic Graft-versus-Host Disease Brazil-Seattle Consortium Study Group to train investigators interested in participating in multicenter clinical trials in Brazil. Workshop participants included eight transplant physicians, one dermatologist, two dentists, three physical therapists and one psychologist from five institutions. Workshop participants evaluated nine patients with varying degrees of severity of mucocutaneous lesions and other manifestations of the disease followed by a training session to review and discuss the issues encountered with the evaluation and scoring of patients and in the methods used to evaluate grip strength and the 2-minute walk test. Results: Most participants had difficulties in rating the percentage of each type of mucocutaneous lesion and thought 20 minutes was insufficient to evaluate and record the scores of each patient using the National Institutes of Health criteria and other cutaneous assessments. Several specific areas of difficulties encountered by the evaluators were: 1) determining the percentage of erythema in movable and non-movable sclerosis, 2) whether to score all cutaneous findings in a particular area or just the dominant lesion; 3) clarification of the definition of poikiloderma in chronic graft-versus-host disease; 4) discrepant interpretation of the mouth score and 5) clarification on the methodology used for the evaluation of grip strength and the 2-minute walk tests. Conclusions: Results of this workshop support the need to train investigators participating in clinical trials on chronic graft-versus-host disease.335358366Cutler, C., Antin, J.H., Chronic graft-versus-host disease (2006) Curr Opin Oncol, 18 (2), pp. 126-131Lee, S.J., Vogelsang, G., Flowers, M.E., Chronic graft-versus-host disease (2003) Biol Blood Marrow Transplant, 9 (4), pp. 215-233. , Comment in: Biol Blood Marrow Transplant. 2003;9(8):540Filipovich, A.H., Weisdorf, D., Pavletic, S., Socie, G., Wingard, J.R., Lee, S.J., National Institutes of Health consensus development project on criteria for clinical trials in chronic graft-versus-host disease: I. Diagnosis and staging working group report (2005) Biol Blood Marrow Transplant, 11 (12), pp. 945-956Pavletic, S.Z., Martin, P., Lee, S.J., Mitchell, S., Jacobsohn, D., Cowen, E.W., Turner, M.L., Vogelsang, G.B., Measuring therapeutic response in chronic graft-versus-host disease: National Institutes of Health consensus development project on criteria for clinical trials in chronic graftversus-host disease: IV (2006) Biol Blood Marrow Transplant, 12 (3), pp. 252-266. , Response Criteria Working GroupMartin, P.J., Weisdorf, D., Przepiorka, D., Hirschfeld, S., Farrell, A., Rizzo, J.D., Foley, R., Lee, S.J., National Institutes of Health Consensus Development Project on Criteria for Clinical Trials in Chronic Graft-versus-Host Disease: VI. Design of Clinical Trials Working Group report (2006) Biol Blood Marrow Transplant, 12 (5), pp. 491-505. , Design of Clinical Trials Working GroupBouzas, L.F., Silva, M.M., Tavares, R.C., Moreira, M.C., Correa, M.E., Funke, V.A., Diretrizes para o diagnóstico, classificação, profilaxia e tratamento da doença enxerto contra hospedeiro crônica (2010) Hemoter Rev Bras Hematol, 32 (SUPPL. 1), pp. 22-39Vigorito, A.C., Miranda, E.C.M., Bouzas, L.F., Moreira, R.C., Silva, M.M., Tavares, R.C.B.S., Feasibility of NIH consensus criteria for chronic graft-versus-host disease (GVHD): Establishing a Successful Efforts in Brazil-Seattle collaborative multicenter consortium (2010) 2010 BMT Tandem Meetings, 16, pp. S47. , In:, Orlando, Florida. Biology of Blood and Marrow Transplantation. New York: ElsevierVigorito, A.C., Miranda, E.C., Bouzas, L.F., Moreira, R.C., Silva, M.M., Tavares, R.C., Feasibility of NIH consensus criteria for chronic graftversus-host disease (GVHD): Establishing a Successful Efforts in Brazil-Seattle collaborative multicenter consortium (2010) 2010 BMT Tandem Meetings, 16, pp. S47. , In:, Orlando, Florida. Biology of Blood and Marrow Transplantation. New York: ElsevierAlborghetti, M.R., Corrêa, M.E., Adam, R.L., Metze, K., Coracin, F.L., de Souza, C.A., Late effects of chronic graft-vs.-host disease in minor salivary glands (2005) J Oral Pathol Med., 34 (8), pp. 486-493Hymes, S.R., Turner, M.L., Champlin, R.E., Couriel, D.R., Cutaneous manifestations of chronic graft-versus-host disease (2006) Biol Blood Marrow Transplant, 12 (11), pp. 1101-1113Rationale and Design of the Chronic GVHD Cohort Study: Improving Outcomes Assessment in Chronic GVHD (2011) Biol Blood Marrow Transplant, 17 (8), pp. 1114-1120. , The Chronic GVHD ConsortiumGreinix, H.T., Pohlreich, D., Maalouf, J., Soukup, P., Supper, V., Kalhs, P., A Single-Center Pilot Validation Study of a New Chronic GVHD Skin Scoring System (2007) Biol Blood Marrow Transplant, 13 (6), pp. 715-723Arai, S., Jagasia, M., Storer, B., Chai, X., Pidala, J., Cutler, C., Global and organ-specific chronic graft-versus-host disease severity according to the 2005 NIH Consensus Criteria Blood, , In press online in, doi:10.1182/blood-2011-03-344390Inamoto, Y., Chai, X., Kurland, B.F., On behalf of the Chronic GVHD Consortium: Validation of Measurement Scales in Ocular Graft-versus-host Disease (2011) Ophthalmology, , in pressMitchell, S.A., Jacobsohn, D., Powers, K.E., A Multicenter Pilot Evaluation of the National Institutes of Health Chronic Graftversus-Host Disease (cGVHD) Therapeutic Response Measures Feasibility, Interrater Reliability, and Minimum Detectable Change Biol Blood Marrow Transplant, , In press: doi:10.1016/ j.bbmt.2011.04.002Vogelsang, G.B., Wolff, D., Altomonte, V., Farmer, E., Morison, W.L., Corio, R., Treatment of chronic graft-versus-host disease with ultraviolet irradiation and psoralen (PUVA) (1996) Bone Marrow Transplant, 17 (6), pp. 1061-1067Volc-Platzer, B., Hönigsmann, H., Hinterberger, W., Wolff, K., Photochemotherapy Improves chronic cutaneous graft-versushost disease (1990) J Am Acad Dermatol., 23 (2 PART. 1), pp. 220-22