Neinfekční nemaligní lymfadenopatie - sinusová histiocytóza s masivní lymfadenopatií, nemoc Rosaiova-Dorfmanova
| Title in English | Non-infectious non-malignant lymphadenopathy - sinus histiocytosis with massive lymphadenopathy, Rosai-Dorfman disease |
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| Authors | |
| Year of publication | 2018 |
| Type | Article in Periodical |
| Magazine / Source | Transfúze a hematologie dnes |
| MU Faculty or unit | |
| Citation | |
| Web | https://www.prolekare.cz/casopisy/transfuze-hematologie-dnes/2018-3-21/neinfekcni-nemaligni-lymfadenopatie-sinusova-histiocytoza-s-masivni-lymfadenopatii-nemoc-rosaiova-dorfmanova-106494 |
| Keywords | sinus lymphadenopathy with massive lymphadenopathy Rosai-Dorfman disease; 2-chlorodeoxyadenosine; thalidomide; lenalidomide; rituximab |
| Description | Rosai-Dorfman disease (also known as sinus histiocytosis with massive lymphadenopathy) is a rare non-Langerhans’- -cell histiocytosis of unknown cause that often manifests as massive cervical lymphadenopathy in children and adults, but infiltration of other lymphatic nodes can occur. The disease can affect the skin, lungs and central nervous system. Spontaneous remissions occur, but patients with bulky or symptomatic disease require treatment. Prednisone, methotrexate and cytotoxic agents such as 2-chlorodeoxyadenosine and clofarabine are used with variable success as first line therapy. Immunomodulatory drugs (thalidomide, lenalidomide and rituximab) have shown some positive results and can be used as second line treatment. |