Systemic histiocytosis of bernese mountain dogs 557 apex, nasal planum, eyelids, and face. Langerhans cell histiocytosis radiology reference article. Langerhans cell histiocytosis misdiagnosed as liver cancer and. 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. Nov 17, 2010 lch encompasses a spectrum of disorders with diverse clinical presentations ranging from single organ to multiple organ involvement. Occasionally and confusingly, the term histiocytosis is sometimes used to refer to individual diseases. Since recent research demonstrated langerhan cell involvement as well as histiocytes, this led to a proposal that the.
Oral manifestation of langerhans cell histiocytosis. It stains strongly with basic fuchsin and methylene blue. This group is made up of a wide variety of conditions that can affect both children and adults. Langerhans cell histiocytosis histiocytosis x of bone. 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. Malignant histiocytosis is a neoplasm composed exclusively of cells showing morphological and immunophenotypic features similar to those of mature tissue histiocytes. Langerhans cell histiocytosis lch, which was formerly named histiocytosis x, is a relatively rare and unique disease characterized by an abnormal.
It is found mostly in the bone marrow, the blood stream, the skin, the liver, the lungs, the lymph glands and the spleen. A slice of the sectioned spleen showing a white nodule arrow underneath the splenic capsule. 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. Langerhans refers to the doctor who first described normal langerhans cells. Langerhans cell histiocytosis of an adult with tumors in. Langerhans cell histiocytosis forming an asymptomatic solitary. Malignant histiocytosis mh is the widespread proliferation of malignant histiocytes in various organs of the body.
Langerhans cells are a type of white blood cell that normally help the body fight infection. Langerhans cell histiocytosis treatment pdqpatient. In histiocytosis, the histiocytes move into tissues where they are not normally found and cause damage to those tissues. Langerhans cell histiocytosis is a rare disease that includes a wide range of clinical manifestations. Langerhans cell histiocytosis womens and childrens. In langerhans cell histiocytosis formerly known as histiocytosis x, langerhans cells multiply abnormally. The newer term is preferred as its more descriptive of its cellular background, and removes the ambiguity of the connotation. Langerhans cell histiocytosis of an adult with tumors in liver and spleen article in hepatogastroenterology 4838. Primary histiocytic sarcoma of the spleen associated with. 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. Experimental studies have shown that ceroid is the result of oxidation and poly. 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 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.
The name of the disease is derived from the type of body cell involved, the histiocyte, osis meaning increased numbers. These histiocytes, along with lymphocytes, macrophages, and eosinophils may infiltrate nearly every organ most notably the. Isolated langerhans cell histiocytosis of the spleen ncbi. In contrast, in systemic malignant histiocytosis, secondary spleen involvement occurs more frequently but with diffuse infiltration. Sonographically demonstrable lesions of the spleen and pancreas have not been reported. Langerhans cell histiocytosis is a rare disease, which can affect children of any age. Langerhans cell histiocytosis womens and childrens hospital.
Isolated langerhans cell histiocytosis of the spleen. 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. J langerhans cell histiocytosis forming asymptomatic solitary. Langerhans cells are normally present as a few cells in thymus, lymph nodes and skin. We present a case of langerhans cell histiocytosis lch of the liver and spleen in an adult.
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. 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. 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. A case of solitary langerhans cell histiocytosis lch in the spleen of a 29 year old chinese man, discovered incidentally at necropsy, is reported. 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 genetics home reference nih. 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. The histologic findings are characterized by sinusoidal involvement and electron microscopy shows birbeck granules, i. 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. 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. The association with a reactive histiocytosis with erythrophagocytosis corresponds to histiocytic medullary reticulosis, as previously described by scott and robbsmith. Langerhans cell histiocytosis histiocytosis x harvard health. The sonographic appearance of lesions of the spleen and. Langerhans cells capture antigens and present them to lymphocytes. Langerhans cell histiocytosis was previously known as histiocytosis x. Histiocytosis x definition of histiocytosis x by medical. 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.
Other less familiar diseases in this category are erdheimchester disease ecd, juvenile. They are found in the skin, lymph nodes, spleen, bone marrow and lungs. Imaging findings in langerhans cell histiocytosis of the. Pdf littoral cell angioma of the spleen and seablue histiocytosis. Thecommondenominatorofall formsoflangerhans cell histiocytosis lch, formerly known as histiocytosis x, is the presence of. Langerhans cell histiocytosis histiocytosis x guide. Langerhans cell histiocytosis lch, which was formerly named histiocytosis x, is a relatively rare and unique disease characterized by an. The skin of the neck, limbs and trunk was usually less frequently in volved. Histiocytic sarcoma of the spleen journal of pathology and. 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. Treatment can include chemotherapy, surgery, radiation therapy, photodynamic therapy, immunotherapy, and targeted therapy. Langerhans cell histiocytosis is a disorder in which excess immune system cells called langerhans cells build up in the body. Malignant histiocytosis and histiocytic sarcoma basic information description histiocytes are tissue cells that interact with white blood cells when tissues are inflamed. When the cancerous histiocytes form a solid tumor, it is.
Langerhans cell histiocytosis forming an asymptomatic solitary nodule in the spleen article pdf available in journal of clinical pathology 493. Langerhans cell histiocytosis lch in adults is a rare disorder that occurs when the body produces too many langerhans cells histiocytes, which are a. Includes ceroid sea blue histiocytosis, chronic myelogenous leukemia, follicular mineral oil lipidosis due to lipid in packaging of food, hum pathol 1984. Multisystem radiology 2008 more than just langerhans cell histiocytosis. 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. The clinical scenario describes an aggressive presentation of multisystem langerhans cell histiocytosis in a young patient.
Langerhans cell histiocytosis is a rare disorder that can look like some types of cancer. It chiefly affects young adults, primarily occurring in the third or fourth decades of life. 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. Langerhans cell histiocytosis lch treatment depends on where it is found in the body and whether the lch is low or high risk. Langerhans cell histiocytosis lch is a rare multisystem disease with a wide and heterogeneous clinical spectrum and variable extent of involvement. Langerhans cell histiocytosis forming an asymptomatic. Langerhans cell histiocytosis lch is a rare proliferative disorder in which. Pdf langerhans cell histiocytosis forming an asymptomatic.