Sinusová histiocytóza s masivní lymfadenopatií: FDG-PET/CT dokumentovaná parciální remise po léčbě 2-chlorodeoxyadenozinem
Title in English | Sinus histiocytosis with massive lymphadenopathy: FDC-PET/CT documented partial remission after treatment with 2-chlorodeoxyadenosine |
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Authors | |
Year of publication | 2016 |
Type | Article in Periodical |
Magazine / Source | Vnitřní lékařství |
MU Faculty or unit | |
Citation | |
Field | Oncology and hematology |
Keywords | Castleman's disease; lenalidomide; Rosai-Dorfman disease; rituximab; sinus lymphadenopathy with massive lymphadenopathy; thalidomide; 2-chlorodeoxyadenosine |
Description | Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a very rare disease belonging to a group of histiocytoses (more precisely non-Langerhans cell histiocytoses). Rosai-Dorfman disease is characterised by the presence of atypical histiocytic cells in the sinuses of lymph nodes or in the extranodal lymphoid tissue, absorbing lymphocytes and plasma cells. The structure and function of the absorbed cells is not impaired and they can leave histiocytes as viable cells. This effect is called emperipolesis, whereas ingestion of cells with their destruction is called phagocytosis. In our text we describe a patient with this disease located, characteristically, in supracla-vicular lymph nodes, but also in mediastinal lymph nodes. Along with lymphadenopathy skin alterations appeared which were both clinically and histologically described as eczema dermatitis. At the same time as lymphadenopathy also strong headaches started which the patient had never suffered before. |
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