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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 ]
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]
Congenital self-healing reticulohistiocytosis is a condition that is a self-limited form of Langerhans cell histiocytosis. [2]: ... Up to 80% of patients have ...
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.
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 ...
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 ...
Patients and families can gain support and educational materials from the Histiocytosis Association. [ citation needed ] The Histiocyte Society , a nonprofit organization, is a group of more than 200 physicians and scientists from around the world committed to improving the lives of patients with histiocytic disorders by conducting clinical 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