Artigo / Article Avaliação da eficácia do uso intravenoso de sacarato de hidróxido de ferro III no tratamento de pacientes adultos com anemia ferropriva Evaluation of the efficacy of intravenous iron III-hydroxide saccharate for treating adult patients with iron deficiency anemia
CONTEXT: Mucosa-associated lymphoid tissue (MALT) lymphomas are most commonly found in the stomach, lungs, orbital soft tissue, salivary glands and thyroid. Involvement of the trachea is extremely rare. CASE REPORT: This report describes a rare case of MALT lymphoma of the trachea in a 71-year-old woman who presented with a one-year history of coughing, dyspnea, hoarseness and weight loss. There was an infiltrative lesion in the mid-trachea. The anatomopathological diagnosis was only made from the fifth endoscopic biopsy attempt. Immunochemotherapy consisting of rituximab, cyclophosphamide, vincristine and prednisone (R-COP) induced complete remission of the symptoms and endoscopic lesion. CONCLUSIONS: MALT lymphoma of the trachea is extremely rare and indolent disease. It has to be considered in the differential diagnosis of airway lesions. It is crucial to obtain an anatomopathological diagnosis from a specialized pathologist. Immunochemotherapy with R-COP induced complete remission of the disease.
Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytic disorder with a prevalence of 1:200 000 people in the United States. The characteristic clinical presentation is a large and painful bilateral cervical mass in young adults, but more than a quarter of cases show extranodal manifestations. Skin, paranasal sinuses, soft tissue, bone, salivary glands, oral cavity, and central nervous system can be affected, resulting in a very distinct constellation of symptoms in each patient. Systemic manifestations such as fever and leukocytosis are also frequent. The usual course is benign, with spontaneous regression in many cases. However, a minority of patients suffer from a progressive disease, occasionally resulting in death, usually due to local infiltration of vital structures.
This study aims to describe the clinical, radiologic, and anatomopathological characteristics as well as individualized therapeutic procedures and outcomes of five cases of RDD disease that have been diagnosed in a reference hospital in São Paulo and compare these findings with recent bibliography on this rare disease.
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