Jurnal Internasional Histositosit yang tidak biasa dalam efusi pleura: tanda dari penyakit langka
A 79-year-old woman was hospitalized for dyspnea, dry cough, and left chest pain. His medical history includes coronary artery disease, hypertension, and hypothyroidism. There is no palpable lymphadenopathy or organomegaly recorded on a physical examination. His chest radiograph showed lower left lobe atelectasis and pleural effusion, and his chest tomographic scan showed significant left-sided pleural effusion with associated volume loss (panel A). Analysis of pleural fluid shows histiocyte proliferation representing 40% of nucleated cells, with 15% showing emperipolesis by ingesting intact lymphocytes (panel B-C; hematoxylin and eosin staining, original enlargement × 1000). They are mixed with mesothelial cells with a background that is rich in lymphocytes. Positive histiocytes for CD68 and S100 and negative for CD1a. There is no indication feature of malignant lymphoproliferative disorders detected by flow cytometry. The findings are consistent with the extranodal variant of Rosai-Dorfman's disease. Patients treated with left pleurodesis and oral prednisone showed a significant increase.
Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a benign proliferation of known pathogenesis histiocytes. This mainly affects the cervical lymph nodes with the possibility of involvement of extranodal tissue. Pulmonary involvement occurred in NRAS  KRAS MAP2K and ARAF gene was found.