Histiocyte Society classification of histiocytoses
Updates to Article Attributes
Body
was changed:
The 2016 Histiocyte Society classification of histiocytoses and neoplasms of macrophage-dendritic cell lineages is a proposed revision of the organisation's initial classification system from 1987.
Terminology
Histiocytes are mononuclear phagocytes that reside in tissues, including macrophages and dendritic cells. Histiocytoses are disorders characterized by the accumulation of cells derived from macrophages or dendritic cells.
Classification
L (Langerhans) group
- Langerhans cell histiocytosis
- Erdheim-Chester disease
- indeterminate cell histiocytosis
- mixed Langerhans cell histiocytosis and Erdheim-Chester disease
C group: cutaneous and mucocutaneous non-Langerhans cell histiocytoses
- cutaneous non-Langerhans cell histiocytoses
- xanthogranuloma family
- juvenile xanthogranuloma
- adult xanthogranuloma
- solitary reticulohistiocytoma
- benign cephalic histiocytosis
- generalized eruptive histiocytosis
- progressive nodular histiocytosis
- non-xanthogranuloma family
- cutaneous Rosai-Dorfman disease
- necrobiotic xanthogranuloma
- cutaneous histiocytoses not otherwise specified
- xanthogranuloma family
- cutaneous non-Langerhans cell histiocytoses with a major systemic component
- xanthogranuloma family
- xanthoma disseminatum
- non-xanthogranuloma family
- xanthogranuloma family
M group: malignant histiocytoses
- primary malignant histiocytoses
- secondary malignant histiocytoses (following or associated with another haematologic neoplasia)
- subtypes
- histiocytic
- interdigitating cell
- Langerhans cell
- indeterminate cell
- not specified
R group: Rosai-Dorfman disease and miscellaneous noncutaneous, non-Langerhans cell histiocytoses
- familial Rosai-Dorfman disease
- sporadic 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
H group: haemophagocytic lymphohistiocytosis and macrophage activation syndrome
- primary haemophagocytic lymphohistiocytosis (Mendelian inherited conditions)
- secondary haemophagocytic lymphohistiocytosis (apparently non-Mendelian)
- haemophagocytic lymphohistiocytosis of unknown/uncertain origin
-<li>non-xanthogranuloma family<ul><li><a title="Multicentric reticulohistiocytosis" href="/articles/multicentric-reticulohistiocytosis">multicentric reticulohistiocytosis</a></li></ul>- +<li>non-xanthogranuloma family<ul><li><a href="/articles/multicentric-reticulohistiocytosis">multicentric reticulohistiocytosis</a></li></ul>
-<li>sporadic Rosai-Dorfman disease<ul>- +<li>sporadic <a href="/articles/rosai-dorfman-disease">Rosai-Dorfman disease</a><ul>
-<li>primary haemophagocytic lymphohistiocytosis (Mendelian inherited conditions)</li>- +<li>primary <a title="Haemophagocytic lymphohistiocytosis" href="/articles/haemophagocytic-lymphohistiocytosis">haemophagocytic lymphohistiocytosis</a> (Mendelian inherited conditions)</li>
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