Hematologic Malignancy
Rosai-Dorfman-Destombes Disease
CI-CAN-00001469RDD · SHMLExplore in graph →
HematologicNon-malignant3 active trials
Loading cancer entity…
Loading cancer entity…
Hematologic Malignancy
CI-CAN-00001469RDD · SHMLExplore in graph →
Data completeness1 / 7 dimensions
Computed from entity_counters.completeness · refreshed after each connector run
Definition
A rare histiocytic disorder of unknown etiology, characterized by distention of the lymph node sinuses and sinusoidal histiocytic infiltration. The histiocytes characteristically contain ingested lymphocytes and are S100 and CD68 positive, and CD1a negative. Patients present with cervical lymphadenopathy, fever, leukocytosis, and hypergammaglobulinemia. It can affect extranodal sites, including skin, bones, and the respiratory tract. It occurs in isolation or may be associated with autoimmune disorders or malignancies. Mutations in NRAS, KRAS, MAP2K1, and ARAF genes have been identified in affected tissues, suggesting a clonal origin in some cases. It is a self-limited disorder, although some patients may have a poor prognosis.
Data updated 22 days agoSource updated 26.08e
Taxonomy
Several hierarchies coexist; edges are listed per hierarchy type with their source.
Parents
5 descendants in total; counters on this page aggregate over all of them.
Anatomy
Data updated 21 minutes agoSource updated unknowncounters aggregate over descendants
Names
CancerIndex is a research and information platform. It does not diagnose and does not recommend treatment.
Key figures
Each figure carries its unit, period and source; ranks link to "Why this rank?".
No registry-level ancestor: burden figures are published for the mutually exclusive top-level site groups only, and this entity does not descend from one. Global figures (IARC / GLOBOCAN) stay under license review and SEER awaits credentials.
Data updated 21 minutes agoSource updated unknownregistry figures: none · counters aggregate over descendants