Langerhans cell histiocytosis lch is a rare proliferative disorder in which. The skin of the neck, limbs and trunk was usually less frequently in volved. Malignant histiocytosis mh is the widespread proliferation of malignant histiocytes in various organs of the body. Nov 17, 2010 lch encompasses a spectrum of disorders with diverse clinical presentations ranging from single organ to multiple organ involvement.
Langerhans cell histiocytosis lch, which was formerly named histiocytosis x, is a relatively rare and unique disease characterized by an. Histiocytic disorders are a group of diseases that occur when there is an overproduction of white blood cells known as histiocytes that can lead to organ damage and tumor formation. Langerhans cell histiocytosis of an adult with tumors in liver and spleen article in hepatogastroenterology 4838. Langerhans cells are white blood cells in the immune system that normally play an important role in protecting the body against viruses, bacteria and other invaders. Langerhans cell histiocytosis treatment pdqpatient. Pdf littoral cell angioma of the spleen and seablue histiocytosis. Langerhans cell histiocytosis lch, formerly known under a variety of different names such as histiocytosis x, eosinophilic granuloma, handschullerchristian disease or letterersiwe disease, is a rare disease of the family of histiocytosis characterized by the accumulation of histiocytic cells in various tissues. Malignant histiocytosis and histiocytic sarcoma basic information description histiocytes are tissue cells that interact with white blood cells when tissues are inflamed. Langerhans refers to the doctor who first described normal langerhans cells. Langerhans cell histiocytosis womens and childrens hospital. The clinical scenario describes an aggressive presentation of multisystem langerhans cell histiocytosis in a young patient.
The newer term is preferred as its more descriptive of its cellular background, and removes the ambiguity of the connotation. Histiocytosis is also referred to as, langerhans cell histiocytosis lch, or more formally as, histiocytosis x, and represents a group of rare disorders involving specific cells that usually have important roles as part of a persons immune system. Langerhans cell histiocytosis lch, formerly known as histiocytosis x which included eosinophilic granuloma, letteresiwe disease and handschullerchristian disease, is a rare disease typically occurs in children and adolescents, although it can develop in all age groups. We present a case of langerhans cell histiocytosis lch of the liver and spleen in an adult. These histiocytes, along with lymphocytes, macrophages, and eosinophils may infiltrate nearly every organ most notably the. 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. Oral manifestation of langerhans cell histiocytosis. The sonographic appearance of lesions of the spleen and. Histiocytosis x is a group of disorders letterersiwe disease, handschullerchristian disease, eosinophilic granuloma in which abnormal scavenger cells called histiocytes and another immune system cell type called eosinophils proliferate, especially in the bone and lung, often causing scars to form.
Since recent research demonstrated langerhan cell involvement as well as histiocytes, this led to a proposal that the. It stains strongly with basic fuchsin and methylene blue. Multisystem radiology 2008 more than just langerhans cell histiocytosis. A histiocyte is a normal immune cell that is found in many parts of the body especially in the bone marrow, the blood stream, the skin, the liver, the lungs, the lymph glands and the spleen. Experimental studies have shown that ceroid is the result of oxidation and poly. Definition histiocytosis x is a generic term that refers to an increase in the number of histiocytes, a type of white blood cell, that act as scavengers to remove foreign material from the blood and tissues.
Includes ceroid sea blue histiocytosis, chronic myelogenous leukemia, follicular mineral oil lipidosis due to lipid in packaging of food, hum pathol 1984. A slice of the sectioned spleen showing a white nodule arrow underneath the splenic capsule. Langerhans cell histiocytosis lch treatment depends on where it is found in the body and whether the lch is low or high risk. When the cancerous histiocytes form a solid tumor, it is. Langerhans cell histiocytosis lch is a rare histiocytic disorder most commonly characterized by single or multiple osteolytic bone lesions demonstrating infiltration with histiocytes having beanshaped nuclei on biopsy. A case of solitary langerhans cell histiocytosis lch in the spleen of a 29 year old chinese man, discovered incidentally at necropsy, is reported. Langerhans cell histiocytosis histiocytosis x guide. It chiefly affects young adults, primarily occurring in the third or fourth decades of life. Langerhans cell histiocytosis histiocytosis x harvard health. Although langerhans cell histiocytosis lch is a familiar entity to most radiologists and to pediatric radiologists in particular, it is but one of a group of disorders caused by the overproduction of histiocytes, a subtype of white blood cells. Langerhans cell histiocytosis is a rare disease, which can affect children of any age. Langerhans cell histiocytosis is a rare disorder that can look like some types of cancer.
Imaging findings in langerhans cell histiocytosis of the. Mar 29, 2018 eosinophilic granuloma, also known as pulmonary histiocytosis x phx or pulmonary langerhans cell histiocytosis x plch, is an uncommon interstitial lung disease that is epidemiologically related to tobacco smoking. Thecommondenominatorofall formsoflangerhans cell histiocytosis lch, formerly known as histiocytosis x, is the presence of. Langerhans cell histiocytosis lch is a rare multisystem disease with a wide and heterogeneous clinical spectrum and variable extent of involvement. Histiocytosis x definition of histiocytosis x by medical. The imaging features are different from those in the few previously reported cases of individual organ involvement by lch. Pdf we describe a 46yearold female presenting with epigastric pain, anorexia and. Langerhans cell histiocytosis lch, the term now used to describe the group of diseases known as histiocytosis x, is a rare disorder of the bone marrowderived histiocytes that may involve the skin, bone, bone marrow, liver, spleen, lungs, lymph nodes, and rarely the pancreas. Langerhans cell histiocytosis lch, which was formerly named histiocytosis x, is a relatively rare and unique disease characterized by an abnormal.
This is the first documented case of lch confined to the spleen and suggests that lch should be included in the differential diagnosis of space occupying lesions in the spleen. Langerhans cell histiocytosis was previously known as histiocytosis x. Langerhans cells are normally present as a few cells in thymus, lymph nodes and skin. Langerhans cell histiocytosis forming an asymptomatic solitary. Pdf langerhans cell histiocytosis forming an asymptomatic. In contrast, in systemic malignant histiocytosis, secondary spleen involvement occurs more frequently but with diffuse infiltration. Langerhans cell histiocytosis of an adult with tumors in. Langerhans cell histiocytosis radiology reference article.
This group is made up of a wide variety of conditions that can affect both children and adults. Langerhans cell histiocytosis lch in adults is a rare disorder that occurs when the body produces too many langerhans cells histiocytes, which are a. Langerhans cell histiocytosis is a rare disease that includes a wide range of clinical manifestations. 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. Langerhans cell histiocytosis forming an asymptomatic. Liver involvement of langerhans cell histiocytosis in. Malignant histiocytosis is a neoplasm composed exclusively of cells showing morphological and immunophenotypic features similar to those of mature tissue histiocytes. To simplify confusing terminology previously used to describe lch eosinophilic granuloma, letterersiwe disease, handschullerchristian disease, histiocytosis x, it is preferable to define histiocytosis with regard to involvement of a single system ie, skin, bone, lymph node, multiple systems, a single organ, or multiple organs. Isolated langerhans cell histiocytosis of the spleen ncbi.
Langerhans cell histiocytosis lch is the most common of the histiocytic disorders and occurs when the body accumulates too many immature langerhans cells, a subset of the larger family of cells known as histiocytes. Liver involvement of langerhans cell histiocytosis in children. They are found in the skin, lymph nodes, spleen, bone marrow and lungs. Langerhans cell histiocytosis misdiagnosed as liver cancer and. Langerhans cell histiocytosis forming an asymptomatic solitary nodule in the spleen article pdf available in journal of clinical pathology 493.
J langerhans cell histiocytosis forming asymptomatic solitary. Other less familiar diseases in this category are erdheimchester disease ecd, juvenile. Sonographically demonstrable lesions of the spleen and pancreas have not been reported. Langerhans cell histiocytosis is a disorder in which excess immune system cells called langerhans cells build up in the body. The name of the disease is derived from the type of body cell involved, the histiocyte, osis meaning increased numbers. Treatment can include chemotherapy, surgery, radiation therapy, photodynamic therapy, immunotherapy, and targeted therapy.
The histologic findings are characterized by sinusoidal involvement and electron microscopy shows birbeck granules, i. Isolated langerhans cell histiocytosis of the spleen. Langerhans cell histiocytosis histiocytosis x of bone. In histiocytosis, the histiocytes move into tissues where they are not normally found and cause damage to those tissues. It is found mostly in the bone marrow, the blood stream, the skin, the liver, the lungs, the lymph glands and the spleen.
Histiocytic sarcoma of the spleen journal of pathology and. Langerhans cells are a type of white blood cell that normally help the body fight infection. Langerhans cell histiocytosis genetics home reference nih. In medicine, histiocytosis is an excessive number of histiocytes tissue macrophages, and the term is also often used to refer to a group of rare diseases which share this sign as a characteristic. Occasionally and confusingly, the term histiocytosis is sometimes used to refer to individual diseases. Langerhans cell histiocytosis womens and childrens. The association with a reactive histiocytosis with erythrophagocytosis corresponds to histiocytic medullary reticulosis, as previously described by scott and robbsmith. Systemic histiocytosis of bernese mountain dogs 557 apex, nasal planum, eyelids, and face.
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