Pdf malignant histiocytosis in a dog case report researchgate. Langerhans cell histiocytosis lch describes a spectrum of clinical presentations ranging from a single bone lesion or trivial skin rash to an explosive disseminated disease. This disorder is characterized by an abnormal increase in certain immune cells called histiocyte cells. Langerhans cell histiocytosis in the maxillofacial area in. Langerhans cell histiocytosis boston childrens hospital.
In langerhans cell histiocytosis, excess immature langerhans cells usually form tumors called. Pulmonary langerhans cell histiocytosis plch is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic cells were found to involve an almost universal activation of the mapkerk pathway, with. Langerhanscell histiocytosis a spectrum of diseases formerly known as histiocytosis x and now called langerhanscell histiocytosis is characterized by erk pathway activation including braf v600e. Hence it is present in multiple clinical appearances with variable course and outcome. Diagnosis and management of langerhans cell histiocytosis. Lch is part of a group of syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes an. The disease prognosis and its response to treatment is better in the single system form, particularly with bone involvement, and much worse in the multisystem langerhajs, especially histiocotosis several risk organs are involved liver, spleen, bone marrow and lung. Langerhans cell histiocytosis lch is a rare disorder in which the body makes too many dendritic cells. While the cause of lch is unknown, lch can frequently behave like cancer and so is treated by cancer specialists. Erdheimchester disease, identified by william chester in 1930, is a rare, systemic, nonfamilial nonlangerhans cell histiocytosis of unclear, but possibly clonal, etiology that occurs predominantly in middleaged and older adults. Langerhans cell histiocytosis is a rare disorder that can look like some types of cancer. Medical management of langerhans cell histiocytosis from.
An ongoing debate over the grouping of lch was finally settled in favor of neoplasm after the discovery of the braf v600e mutation in 2010. Langerhans cell histiocytosis presenting in the neonatal. The cells build up in the body, sometimes damaging organs or forming tumors. Periodontal disease associated with langerhans cell histiocytosis. Treatment of langerhans cell histiocytosis uptodate. This disease was previously designated as histiocytosis x, eosinophilic granuloma, letterersiwe disease and many more.
Langerhans cell histiocytosis lch is a rare condition characterized by abnormal growth of the langerhans cells. Langerhans cell histiocytosis is defined as a clonal proliferation of. Langerhans cell histiocytosis with pancreatic involvement. Langerhans cell histiocytosis is a rare group of disorders without a wellunderstood etiology. Langerhans cell histiocytosis hematology and oncology.
As opposed to juvenile xanthogranuloma, cells in the former condition are more spindly, with coffeebean nuclei. Langerhans cell histiocytosis lch is a rare disease, of unknown pathogenesis, characterized by intense and abnormal proliferation of bone marrowderived histiocytes langerhans cells. Histiocyte cells are a form of white blood cells that help the immune system destroy foreign materials and fight infection. Introduction langerhanscell histiocytosis histiocytosis x is a clonal proliferative disorder of the langerhans cells 1,2. The pathogenic cells are defined by activation of the mapk signaling pathway. These cells usually are in the skin and help fight infection and destroy foreign substances in the body.
Liver involvement in langerhans cell histiocytosis. Histiocytosis, also referred to as langerhans cell histiocytosis lch, and formally called histiocytosis x, represents a group of rare disorders involving specific cells that normally have important roles as part of the immune system. Langerhans cell histiocytosis previously called histiocytosis x involving the skin is the main differential diagnosis of juvenile xanthogranuloma. About half of the people with the disorder have a faulty gene that makes the langerhans immune cells. Several theories have been suggested to explain the pathophysiology. 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, bone marrow, brain. The clinical spectrum includes the acute fulminant, disseminated letterersiwe disease, solitary or few, indolent and chronic.
Langerhans cell histiocytosis is a disorder in which excess immune system cells called langerhans cells build up in the body. Current treatment of langerhans cell histiocytosis. 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. They can be found in the skin, lungs, stomach, bone, eyes and intestines. Imaging findings including diffusionweighted imaging junhwee kim1, myungjoon kim1, chuhl joo lyu2, mijung lee1 1department of radiology and research institute of radiological science, severance childrens hospital, yonsei university, college of medicine. The pathogenesis of lch remains obscure and the optimal treatment for lch has not been established, although incremental progress has been made. A langerhans cell is a type of white blood cell that normally helps the body fight off infection. The histopathology of langerhans cell histiocytosis. Langerhans cell histiocytosis lch, formerly known as histiocytosis x, denotes a group of diseases characterized by infiltration or proliferation of histiocytes in various body tissues. Pdf periodontal disease associated with langerhans cell. 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. These cells play a role in the bodys immune system. Langerhans cell histiocytosis lch of bone is a disorder of histiocytic proliferation with variable and often unpredictable behavior.
Langerhans cell histiocytosis lch is a rare disorder that occurs when a child has too many of a certain cell type called langerhans cells. 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%. The lesions of langerhans cell histiocytosis have a wide spectrum of histologic changes, but a constant feature is a sinusoidal proliferation of langerhans cells in the appropriate cellular background fig. Langerhans cell histiocytosis lch is a heterogeneous disease, characterized by accumulation of dendritic cells with features similar to epidermal langerhans cells in various organs. Symptoms range from isolated bone lesions to multisystem disease. Langerhans cell histiocytosis lch is a dendritic cell antigenpresenting cell disorder. Automatic update in unifocal lch, also called eosinophilic granuloma an older term histiocitosis x is now known to histiocitosis x a misnomeris a slowly progressing disease characterized by an expanding proliferation of langerhans cells in various bones. Known formerly as histiocytosis x, the disease has a wide spectrum of clinical presentations, including eosinophilic granuloma solitary bone lesion, diabetes insipidus, and exophthalmos. Langerhans cell histiocytosis and malignancies of the mpire system. However, it was later discovered that the abnormal cells in lch are actually derived from myeloid dendritic cells that exhibit the same. Dendritic cells are a form of histiocyte, or white blood cell.
Langerhans cell histiocytosis genetics home reference nih. 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. Langerhans cell histiocytosis is a rare disease with different clinical and pathological presentation, is clinically divided into three groups. The pathologic hallmark of plch is the accumulation of langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. Langerhans cell histiocytosis, often called lch, is a disorder where the body produces too many langerhans cells.
The most familiar classification includes langerhans cell histiocytosis lch, nonlangerhans cell histiocytoses, and malignant histiocytic disorders. 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. Pdf malignant histiocytosis is a proliferation of histiocitic cells occurring in. Langerhans cell histiocytosis also called histiocytosis x is a rare disorder that primarily affects children. The proliferation of these cells leads to the formation of tumors called. Langerhans cell histiocytosis langerhans cell histiocytosis in the maxillofacial area in adults. The presence or absence of the pathognomonic langerhans cell organelle, the birbeck granule, within the pathologic histiocyte, and the findings on immunohistochemical studies help in. Langerhans cell histiocytosis lch is a rare clonal proliferative disease characterized by the infiltration of a single organ or multiple organs by cells. Langerhans cell histiocytosis, maxillary sinus introduction langerhans cell histiocytosis lch is a rare disease.
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