Treatment depends on the site and extent of disease. Gastrointestinal involvement in langerhans cell histiocytosis. Any organ or system of the human body can be affected, but those more frequently involved are the skeleton 80% of cases, the skin 33%, and the pituitary 25%. Targeted therapy with an inhibitor of mutated braf vemurafenib improves. Langerhans cell histiocytosis lch treatment may include observation alone, surgery, radiation therapy, or oral, topical, and intravenous medication. Spanish pdf article in xml format article references how to cite this article automatic translation send this article by email. Langerhans cell histiocytosis lch is an orphan disease of clonal dendritic cells which may affect any organ of the body. Langerhans cell histiocytosis lch is a rare cancer involving clonal proliferation of langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Langerhans cell histiocytosis lch is a heterogeneous disease, characterized by accumulation of dendritic cells with features similar to epidermal langerhans cells in various organs. Langerhans cells, which help regulate the immune system, are normally found throughout the body, especially in the skin, lymph nodes, spleen, lungs, liver, and bone marrow. Histiocytoses are rare disorders of unknown origin with highly heterogeneous prognosis. What is nonlangerhans cell histiocytosis the term nonlangerhans cell histiocytosis refers to a group of conditions called histiocytoses that are caused by an overgrowth of cells called histiocytes. Langerhans cell histiocytosis genetic and rare diseases. In langerhans cell histiocytosis, excess immature langerhans cells usually.
These cells also accumulate in the lungs, liver, and spleen. Langerhans cell histiocytosis genetics home reference nih. Nonlangerhans cell histiocytosis has this name to differentiate it from langerhans cell histiocytosis. Get detailed treatment information for lch in this summary for clinicians. In the mouth, presenting celuulas include gingival hypertrophy and ulcers on the soft or hard palate, buccal mucosa, or tongue and lips. Nelson textbook histiocitosis x pediatrics 19th ed. In langerhans cell histiocytosis, excess immature langerhans cells usually form tumors called.
It typically has no extraskeletal involvement, but rarely an identical lesion histiiocitosis be found in the skin, lungs, or stomach. Use of systemic steroid is common, singly or adjunct to chemotherapy. Multifocal multisystem lch, also called letterersiwe diseaseis a rapidly progressing disease in which langerhans cell cells proliferate in many tissues. Langerhans cell histiocytosis lch is the most common dendritic cell disorder and is named because of its similarity to the langerhans cells found in the skin and mucosa. Micrograph showing a langerhans cell maoigna with the characteristic reniform langerhans cells accompanied by abundant eosinophils. Lch is an abnormal proliferation and dissemination of clonal langerhans cells in which they accumulate, along with other inflammatory cells, and form tissue granulomas in different organs. Muchas proliferaciones anteriormente etiquetadas como histiocitosis maligna. Nonlangerhans cell histiocytosis may also be called class ii histiocytosis. Pulmonary langerhans cell histiocytosis plch, previously called eosinophilic granuloma of the lung, pulmonary langerhans cell granulomatosis, and pulmonary histiocytosis x, is an uncommon cystic interstitial lung disease that primarily affects young adults.
Am j clin patholall the contents of this journal, except where otherwise noted, is licensed under a creative commons attribution license. Histiocytosis association is a global nonprofit organization dedicated to funding research leading to better treatments and a cure, raising awareness about histiocytic disorders, as well as providing educational and emotional support. Histiocitosis o enfermedades histiocitarias pediatria integral. The cur rent classification of langerhans cell histiocytosis divides it into three groups according to the number and location of organs involved highrisk.
Langerhans cell histiocytosis lchpreviously known as nlos x, is a rare disease. Lch is part of a group of syndromes called histiocytoses, which are characterized by an abnormal proliferation of. The name, however, originates back to its discoverer, paul langerhans. Based on the cytomorphologic findings and ancillary studies immunohistochemistry and flow cytometry analysis a cytological diagnosis of positive for neoplastic cells with features suggestive of monocytichistiocytic origin, possibly langerhans cell histiocytosis lch was rendered. Langerhans cell histiocytosis lch is a rare histiocytic disorder most commonly characterized by single or multiple osteolytic bone lesions or skin rashes demonstrating infiltration with histiocytes with beanshaped nuclei on biopsy with or without histiocytic infiltration of extraskeletal tissues most notably the skin, lungs, lymph nodes, liver, spleen. Langerhans cell histiocytosis is a disorder in which excess immune system cells called langerhans cells build up in the body. Langerhans cell histiocytosis lch is one of the histiocytosis disorders, as defined by the histiocyte society. On the other hand, the infiltration of organs by monoclonal population of pathologic cells, and the successful treatment of subset of disseminated disease using chemotherapeutic regimens are all consistent with a neoplastic process. The pathogenesis of langerhans cell histiocytosis lch is a matter of debate. Symptoms range from isolated bone lesions to multisystem disease.
Most of the knowledge about the diagnosis and therapy is based on. Langerhans cell histiocytosis lch is a disorder that primarily affects children, but is also found in adults of all ages. Tenyear experience at dallas childrens medical center. Pulmonary langerhans cell histiocytosis has variable appearance depending on the stage of disease, ranging from small peribronchiolar nodular opacities to multiple irregularlyshaped cysts. In people with lch, these cells multiply excessively and build up in certain areas. It is caused by a disorder of myeloid dendritic cells. Langerhans cell histiocytosis lch and the non lch, erdheim chester disease, are rare histiocytic neoplasms with distinctive clinical and immunophenotypic features, but with several overlapping molecular features. However, it was later discovered that the abnormal cells in lch are actually derived from myeloid dendritic cells that exhibit the same. Dramatic efficacy of vemurafenib in both multisystemic and. Pdf diagnosis and management of oral langerhans cell. People with lch produce too many langerhans cells or histiocytes, a form of white blood cell found in healthy people that is supposed to protect the body from infection. Braf v600e gainoffunction mutations have been observed in 57% of cases of langerhans cell histiocytosis lch and 54% of cases of erdheimchester disease ecd, but not in other types of histiocytoses.
912 1156 110 1257 228 174 855 155 1 698 616 1485 1011 761 248 337 1569 1325 122 917 1078 552 610 444 1538 832 1476 144 1356 1438 1267 1228 250 1106 334 194