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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 ]
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.
Before the Histiocyte Society classified histiocytoses in the 1980s, the condition was also known as "Histiocytosis X", where "X" denoted the then unknown cause. [9] [10] [11] It is now known as chronic multifocal Langerhans cell histiocytosis, a subtype of LCH. [3] The disease is rare. [12] Most present between the ages of two and six. [2]
Langerhans cell is represented by a yellow oval; blue arrows correspond to is_a relations, and orange arrows correspond to develops_from relations. Only a subset of Langerhans cell parent types are included in the figure. [1] A Langerhans cell (LC) is a tissue-resident macrophage of the skin [2] once thought to be a resident dendritic cell. [3]
According to the Histiocytosis Association, 1 in 200,000 children in the United States are born with histiocytosis each year. [2] HAA also states that most of the people diagnosed with histiocytosis are children under the age of 10, although the disease can afflict adults. The disease usually occurs from birth to age 15. [3]
First described in 1961 (where they were simply termed "characteristic granules"), [2] they are solely found in Langerhans cells. [1] Although part of normal Langerhans cell histology, they also provide a mechanism to differentiate Langerhans cell histiocytoses (which are a group of rare conditions collectively known as histiocytoses ) from ...
Diffuse large B-cell lymphoma leg type: CD117: Mast cell: Mastocytosis: CD138: Plasma cell: Multiple myeloma Plasmablastic lymphoma: CD207 (Langerin) Langerhans cell: Langerhans cell histiocytosis: Chloracetate esterase (Leder) Mast cell: Mastocytosis: Chromogranin A: Merkel cell carcinoma: Cystokeratin 7: Paget's disease (breast and ...
X-type histiocytoses are a clinically well-defined group of cutaneous syndromes characterized by infiltrates of Langerhans cells, as opposed to Non-X histiocytosis in which the infiltrates contain monocytes/macrophages. [1]: 720 Conditions included in this group are: [1]: 720–4 Congenital self-healing reticulohistiocytosis