Non-infectious non-malignant lymphadenopathy – sinus histiocytosis with massive lymphadenopathy, Rosai-Dorfman disease
Authors:
Z. Adam 1; R. Koukalová 2; Z. Řehák 2; A. Čermák 3; M. Krejčí 1; L. Pour 1
Authors‘ workplace:
Interní hematologická a onkologická klinika LF MU a FN Brno
1; Oddělení nukleární medicíny, Centrum PET-CT, Masarykův onkologický ústav, Brno
2; Klinika urologie LF MU a FN Brno
3
Published in:
Transfuze Hematol. dnes,24, 2018, No. 3, p. 166-173.
Category:
Overview
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.
Key words:
sinus lymphadenopathy with massive lymphadenopathy Rosai-Dorfman disease – 2-chlorodeoxyadenosine – thalidomide – lenalidomide – rituximab
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Transfusion and Haematology Today
2018 Issue 3
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