Histiocytic Syndromes At A Glance

The histiocytic syndromes are a clinically heterogeneous group of diseases caused by the abnormal proliferation of histiocytes, either monocytes / macrophages (antigen-processing cells) or dendritic cells (antigen presenting cells) are produced. The classification of these diseases is difficult (s. Some histiocytic syndromes) and has evolved over time through an improved understanding of cell biology. There are other rare diseases such. As the histiocytic Erdheim-Chester disease and juvenile xanthogranuloma. Some histiocytic syndromes group Common diseases * examples or description Histiocytic diseases with different biological behavior with dendritic cells associated Langerhans cell histiocytosis Includes diseases with the former designations eosinophilic granuloma, Crohn’s Abt-Letterer-Siwe and Crohn’s Hand-Schuller-Christian associated with macrophages primary haemophagocytic Syndrome Family Sporadic Secondary haemophagocytic Syndrome infection tumor diseases autoimmune diseases Rosai-Dorfman disease Also known as sinus histiocytosis with massive lymphadenopathy called malignant histiocytic diseases leukemia Acute monocytic and myelomonocytic leukemia – Chronic myelomonocytic leukemia (CMML) CMML adult CMML in childhood (juvenile chronic myelomonocytic leukemia) * Other, rare diseases are there in each group. After Komp DM, Perry MC: Introduction: The histiocytic syndromes. Seminars in Oncology 18: 1, 1991, Favara BE, Feller AC, Pauli M, eds .: Contemporary classification of histiocytic disorders. Medical and Pediatric Oncology 29: 157. 1997

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