Langerhans cell histiocytosis: Case report with emphasis in differential diagnosis
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Abstract
Introduction: Langerhans cell histiocytosis is an inflammatory neoplasm caused by the multiplication of aberrant cells in the phagocytic mononuclear system. It is the most common histiocytic disorder, affecting about 5 children per million, aged 0 to 15 years, similar to the frequency of Hodgkins lymphoma.Objective: To report a case of high-risk Langerhans cell histiocytosis, highlighting the importance of differential diagnosis in cases of lymphadenopathy and the geographical difficulties of Amazon State for adequate patient follow-up. Case description: A 13-year-old teenager, from the interior of the Amazon State, presented lymphadenopathy in the cervical, axillary and supra-clavicular area. Treatment for ganglionic tuberculosis was performed, but the lesions worsened over time, indicating the need for biopsy. Then, Langerhans cell histiocytosis was confirmed, confirmed by immunohistochemistry. At the moment, the patient is undergoing treatment for this condition, with a good therapeutic response despite the geographical and socioeconomic difficulties.Discussion: The wide clinical spectrum of histiocytosis hinders its early diagnosis. The patient presented with lymph node involvement, rare for this disease and a fact that led to the differential diagnosis with ganglionic tuberculosis, the first hypothesis suggested for the child. Therefore, it is extremely important to consider histiocytosis in the group of diseases that present cervical lymphadenopathy. In addition, the case raises the discussion around geographic issues and how they can interfere for proper diagnosis and treatment.
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