Which histiocytosis disorders are classified as R Group (Rosai-Dorfman disease)?

Updated: Sep 16, 2020
  • Author: Cameron K Tebbi, MD; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Answer

R group histiocytosis includes the following:

  • Familial Rosai-Dorfman disease
  • Classical (nodal) Rosai-Dorfman disease
  • Extranodal Rosai-Dorfman disease
  • Neoplasia-associated Rosai-Dorfman disease
  • Immune disease–associated Rosai-Dorfman disease
  • Other non-C, non-L, non-M, non-H histiocytoses

Familial Rosai-Dorfman disease

Familial Rosai-Dorfman disease includes the following:

  • Faisalabad (or H) syndrome (OMIM #602782)
  • Fas protein deficiency or autoimmune lymphoproliferative syndrome (ALPS)–related Rosai-Dorfman disease (OMIM #601859)
  • Familial Rosai-Dorfman disease not otherwise specified  

Classical (nodal) Rosai-Dorfman disease 

Classical (nodal) Rosai-Dorfman disease includes the following:

  • Without immunoglobulin G4 (IgG4) syndrome
  • IgG4 associated

Extranodal Rosai-Dorfman disease

Extranodal Rosai-Dorfman disease includes the following:

  • Bone Rosai-Dorfman disease
  • CNS with and without IgG4 syndrome association
  • Single-organ Rosai-Dorfman disease (other than lymph node, skin, and CNS) without IgG4 syndrome
  • Single-organ Rosai-Dorfman disease (other than lymph node, skin, and CNS) with IgG4 association
  • Disseminated Rosai-Dorfman disease

Neoplasia-associated Rosai-Dorfman disease

Neoplasia-associated Rosai-Dorfman disease includes the following:

  • Rosai-Dorfman disease postleukemia
  • Rosai-Dorfman disease postlymphoma
  • Rosai-Dorfman disease associated with malignant histiocytosis
  • Rosai-Dorfman disease associated with Langerhans cell histiocytosis or ECD

Immune disease–associated Rosai-Dorfman disease

Immune disease–associated Rosai-Dorfman disease includes the following:

  • Systemic lupus erythematosus related
  • Idiopathic juvenile arthritis related
  • Autoimmune hemolytic anemia associated
  • Human immunodeficiency virus associated

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