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Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Symptoms range from isolated bone lesions to multisystem disease . [ 1 ]
Treatment may involve surgery, chemotherapy, radiation therapy, and certain medicines. [ 7 ] Hand–Schüller–Christian disease was named for the American pediatrician Alfred Hand Jr. , the Austrian neuroradiologist Arthur Schüller , and the American internist Henry Asbury Christian , who described it in 1893, 1915 and 1919, respectively. [ 8 ]
Indeterminate cell histiocytosis (LCH) is an uncommon proliferative illness where the predominant cells have characteristics from both non-Langerhans cell histiocytosis (NLCH) and Langerhans cell histiocytosis (LCH) in terms of morphology and immunophenotypic characteristics. [1]
Letterer–Siwe disease, (LSD) or Abt-Letterer-Siwe disease, is one of the four recognized clinical syndromes of Langerhans cell histiocytosis (LCH) and is the most severe form, involving multiple organ systems such as the skin, bone marrow, spleen, liver, and lung. Oral cavity and gastrointestinal involvement may also be seen.
Rosai–Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy or sometimes as Destombes–Rosai–Dorfman disease, [1] [2] [3] is a rare disorder of unknown cause that is characterized by abundant histiocytes in lymph nodes or other locations including the skin, sinuses, brain and heart.
Treatment is therefore based on the patient's symptoms. [1] If there is an underlying monoclonal gammopathy, it is treated according to relevant guidelines. [ 1 ] A 51-year-old with crystal-storing histiocytosis in the left upper lip and cheek had the tumor removed in 2010.
Erdheim–Chester disease (ECD) is an extremely rare disease characterized by the abnormal multiplication of a specific type of white blood cells called histiocytes, or tissue macrophages (technically, this disease is termed a non-Langerhans-cell histiocytosis). It was declared a histiocytic neoplasm by the World Health Organization in 2016. [1]
Langerhans cell sarcoma (LCS) is a rare form of malignant histiocytosis. It should not be confused with Langerhans cell histiocytosis, which is cytologically benign. [1] It can present most commonly in the skin and lymphatic tissue, but may also present in the lung, liver, and bone marrow. [2] [3] Treatment is most commonly with surgery or ...
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