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- ItemAcesso aberto (Open Access)Hyperfibrotic myelodysplasia: case report with response to steroid therapy(Sociedade Brasileira de Patologia ClínicaSociedade Brasileira de PatologiaSociedade Brasileira de Citopatologia, 2002-01-01) Romeo, Maura [UNIFESP]; Chauffaille, Maria de Lourdes Lopes Ferrari [UNIFESP]; Bahia, Daniella Marcia Maranhão [UNIFESP]; Silva, Maria Regina Regis; Universidade Federal de São Paulo (UNIFESP)Context: Bone marrow fibrosis is observed in different clonal hematological disorders including myeloproliferative diseases, acute leukemias and myelodysplastic syndromes. In myelodysplastic syndrome a new clinical-pathological entity with significant increase in reticulin fibers has been suggested, and the term hyperfibrotic myelodysplasia was used to define it. Bone marrow biopsy shows increased reticulin fibers, megakaryocytic hyperplasia and dysplasia. Differential diagnosis with primary myelofibrosis may be difficult and hybrid cases may occur. Patients with hyperfibrotic myelodysplastic syndrome responding to treatment with steroids have been reported. In the majority of cases there was only hematological remission, although resolution of fibrosis occurred in one patient. Design: Case report. Case report: A 62-year old male presented in June 95 with a 6-month history of lethargy and dispnea. On examination he was pale without hepato-splenomegaly. Hemoglobin concentration was 3g/dL with marked anisocytosis without teardrop cells. Bone marrow aspirates resulted in dry tap. Bone marrow biopsy showed hypercellularity with increased fibrosis (grade IV) obliterating the normal marrow architecture. Megakaryocytes were increased in number, with abnormal morphology. Monoclonal antibodies against factor VIII and CD31 revealed that both were expressed in megakaryocytes. Prednisone (1mg/Kg) was introduced in June 1996, after what his symptoms lessened and hemoglobin increased. Bone marrow fibrosis decreased (grade IV to grade II). He has become transfusion independent till Jan/1999, when hemoglobin fell to 6g/dL and prednisone was reintroduced with a prompt rise in hemoglobin concentration.
- ItemAcesso aberto (Open Access)Radioterapia para pacientes portadores de oftalmopatia de Graves: avaliação clínica e radiológica(Universidade Federal de São Paulo (UNIFESP), 2009-09-30) Cardoso, Cejana Casimiro de Deus [UNIFESP]; Segreto, Roberto Araujo [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)Purpose: To evaluate the clinical and radiological response of patients with Graves’ ophthalmopathy underwent to orbital radiotherapy concomitant or not with corticotherapy. Methods and materials: Eighteen patients (36 orbits) were underwent to orbital radiotherapy with a total dose of 10 Gy, fractioned in 1 Gy once a week over 10 weeks; nine of these patients made concomitant use of corticoids. The patients were evaluated clinically by means of a verbal questionnaire and by the evaluation of the clinical activity score (CAS), and radiologically, by magnetic resonance (MR) imaging parameters, before treatment and six months following it. Results: After treatment, total improvement of ocular pain, palpebral edema, visual acuity and ocular motility was observed. The patients presented a significant decrease in the symptoms such as tearing, diplopia, conjunctival hyperemia and ocular grittiness. There was a significant reduction of the inflammatory activity in the left eye, and all patients were classified as quiescent in the right eye. The MR indicated a marked decrease in ocular muscle thickness and in signal intensity bilaterally. The comparative analysis between groups I (radiotherapy exclusively) and II (radiotherapy combined with corticotherapy) showed no statistical difference regarding the clinical and radiological parameters evaluated. Conclusion: Whether associated or not with corticotherapy, radiotherapy delivered in low dose and in a protracted scheme suggest to be effective and safe, as verified by the clinical and radiological improvements, and is an efficient therapeutic option for patients with Graves’ ophthalmopathy.