Keywords
Mediastinal diseases
Pathology
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Abstract
Objective: To report a clinical case of a young patient diagnosed with Rasai-Dorfman Disease, a rare pathology, with unusual lymph node involvement. Case details: A 15-year-old male patient was admitted to the emergency room via the emergency room due to asthenia, dyspnea on medium and light exertion associated with weight loss and ventilatory-dependent chest pain for approximately 30 days, which progressed to intensification of symptoms, cyanosis of extremities and hemoptoic sputum. After clinical investigation, the immunohistochemical study revealed a Rasai-Dorfman disease pattern. The patient then progresses to outpatient follow-up and clinical decision regarding treatment. Final considerations: Also referred to as Sinus Histiocytosis with Massive Lymphadenopathy (SHML), Rasai-Dorfman Disease is rare and the therapeutic plan is still controversial. In the reported case, the lymphatic chain in the mediastinum was affected, sparing the cervical lymph nodes, something unusual for this rare disease. The importance of the multidisciplinary study of this pathology is highlighted with the aim of providing early diagnosis in other cases and incentives for research into the treatment offered.
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