Langerhanscell histiocytosis insight into dc biology. Langerhans cell histiocytosis involving the thyroid and. Pdf langerhans cell histiocytosis of the gastrointestinal tract. Langerhans cell histiocytosis lch is a rare disease, which occurs mainly in children. Medical management of langerhans cell histiocytosis from. Langerhans cell histiocytosis is a malignancy of langerhans cells with immunoreactivity for cd1a and s100 protein and the presence of cytoplasmic birbeck granules. To date, no largescale studies have been done on how often lch occurs in. Lesional cell phenotypes and the factors influencing variations are noted. 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. Pulmonary langerhans cell histiocytosis surgical pathology. 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. Basic science, clinical features, and therapy, weitzman s, egeler rm eds, cambridge university press, cambridge 2005.
Involvement manifestation possible other condition skin vesicles and bullae most common in early infancy erythema toxicum herpes simplex chickenpox infection dermatitis most frequently scalp, diaper or axilla, may occur up to late infancy seborrheic dermatitis. Langerhans cell histiocytosis is a type of cancer that can damage tissue or cause lesions to form in one or more places in the body. Pathology of langerhans cell histiocytosis hematologyoncology. However lch cells stain positive to cd14 which is a monocyte marker and shows a different, hematopoietic origin for the disorder. Langerhans cell histiocytosis lch treatment may include observation alone, surgery, radiation therapy, or oral, topical, and intravenous medication. This disorder is characterized by an abnormal increase in certain immune cells called histiocyte cells. Langerhans cell histiocytosis in sequential discordant. The cytology of langerhans cell histiocytosis histiocytosis. Langerhans cell histiocytosis is a rare condition, slightly more common in boys than girls, affecting around one in 5 million children and fewer adults. Langerhans cell histiocytosis of the gastrointestinal tract can be a challenging diagnosis to make histologically, particularly when the lesional langerhans cells are obscured by a dense inflammatory cell infiltrate. Jul 28, 2004 langerhans cell histiocytosis lch is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. Langerhans cell histiocytosis information from the histiocytosis association.
Langerhanscell histiocytosis has been associated with malignant disease especially acute leukaemias,1 but the biological basis for this link is unknown. Langerhans cell histiocytosis lch is currently regarded as a. Langerhans cell histiocytosis is a clonal proliferation of cells that morphologically and immunophenotypically resemble langerhans cells more common in childhood 1 3 years old and involves nodal and extranodal sites most common site is bone. Langerhans cell histiocytosis lch is a rare disease marked by proliferation of langerhans type cells that share immunophenotypic and ultrastructural similarities with antigenpresenting langerhans cells of mucosal sites and skin. The relationship between langerhans cells and the other histiocytes from which they are derived seems to be determined by events initiated by surface antigens. Aug 28, langerhans cell histiocytosis lch is a rare histiocytic disorder most in erdheimchester disease but not in other non langerhans cell. Skin biopsy diagnosis of langerhans cell neoplasms intechopen. They can be found in the skin, lungs, stomach, bone, eyes and intestines. 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. Dendritic cells are a form of histiocyte, or white blood cell. Four years before this she had developed stage 1a mixed cellularity hodgkin lymphoma affecting the right inguinal region, which was treated by irradiation and. Apart from bch, the main types of nonlch include juvenile xanthogranuloma, generalized eruptive histiocytoma, and xanthoma disseminatum.
Langerhans cell histiocytosis may develop at any age but most commonly occurs in childhood years of age. Langerhans cell histiocytosis of the gastrointestinal tract. After six months of follow up, the patient is in good conditions. Although the pathogenesis of langerhans cell histiocytosis has been debated. Pathology of langerhans cell histiocytosis histiocytosis x. Approximately 1015% of cases also involve other sites primarily bone. Langerhans cell histiocytosis immunohistochemical expression of. These include mutations of the braf, map2k1, ras and. Histiocyte cells are a form of white blood cells that help the immune system destroy foreign materials and fight infection. Indeterminate cell histiocytosis and mycosis fungoides. We report a case of a 28 yearold lady who presented with a history of perianal pain and discharge per rectum over several months. Selected aspects of the histopathology of langerhans cell histiocytosis. Pulmonary langerhans cell histiocytosis plch is a form of langerhans cell histiocytosis lch that typically occurs in the lungs of adult cigarette smokers 1. Langerhans cell histiocytosis treatment pdqpatient.
Langerhans cell histiocytosis lch represents a disorder characterised by an abnormal accumulation of histiocytes in miscellaneous tissues. Langerhans cell histiocytosis causes, symptoms, diagnosis. Symptoms range from isolated bone lesions to multisystem disease. Pulmonary langerhans cell histiocytosis is a rare interstitial lung disease in adults that is a part of diseases caused by infiltration of langerhans cell across multiple organs, such as the lungs, bones, skin, pituitary gland, and lymph node. Pathology of langerhans cell histiocytosis histiocytosis. Skin biopsy diagnosis of langerhans cell neoplasms. Langerhans cell histiocytosis of the gastrointestinal. Langerhans cell histiocytosis is a disorder in which excess immune system cells called langerhans cells build up in the body. However, a few cases of mycosis fungoides in patients with langerhans cell histiocytosis can be found in the literature, either preceding or presenting after the dendritic cell proliferation. Multiple organs and systems may be involved by the disease. The lchmalignancy study group of the histiocyte society.
The relation of langerhans cell histiocytosis to acute. It is reported to be between 26 54 cases per million children in the general population. Mar 02, 2020 langerhans cell histiocytosis is a clonal proliferation of cells that morphologically and immunophenotypically resemble langerhans cells more common in childhood 1 3 years old and involves nodal and extranodal sites most common site is bone. Langerhans cell histiocytosis genetics home reference nih. Noah brown, md langerhans cell histiocytosis lch comprises a wide spectrum of clinical disorders that have in common a proliferation of langerhans type cells with characteristic morphologic, immunophenotypic, and ultrastructural. The diagnostic histopathology of langerhans cell histiocytosis. Get detailed treatment information for lch in this summary for clinicians. 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. Langerhans cell histiocytosis lch is a disorder that primarily affects children, but is also found in adults of all ages. Langerhans cell histiocytosis lch is a very rare form of disorder that involves clonal proliferation of the langerhans cells, the abnormal cells that are derived from the bone marrow which are capable of moving from the skin to the lymph nodes. Frequently children affected are between years old and boys are more often affected. Lch cells are a type of dendritic cell which fights infection. Noah brown, md langerhans cell histiocytosis lch comprises a wide spectrum of clinical disorders that have in common a proliferation of langerhanstype cells with characteristic morphologic, immunophenotypic, and ultrastructural. It has been referred to by several eponyms handschullerchristian disease, abtletterersiwe disease or letterersiwe disease, and histiocytosis x.
Lch cells are arrested in an immature, partially activated stage and show a deviant regulation of cell division. Langerhanscell histiocytosis lch is caused by an uncontrolled pathogenic clonal proliferation of dendritic cells dcs with langerhanscell lc characteristics. Their aberrant interactions with t cells and the lesional microenvironment are typified by high level production of. Infiltration by langerhans cells polygonal cells with eosinophilic cytoplasm, oval nuclei with longitudinal grooves resembling coffee beans eosinophils, giant cells, neutrophils, foam cells, lymphocytes, plasma cells, fibrosis, necrosis. In 20, we reported in the american journal of dermatopathology a case of selfregressing s100negative cutaneous indeterminate cell histiocytosis ich in a 48yearold woman. Media in category histopathology of langerhans cell histiocytosis the following 36 files are in this category, out of 36 total. Dec 16, 2012 langerhans cell histiocytosis lch is a very rare form of disorder that involves clonal proliferation of the langerhans cells, the abnormal cells that are derived from the bone marrow which are capable of moving from the skin to the lymph nodes. From the department of pathology, university of michigan. Langerhans cell histiocytosis lch, is a rare disorder, clinically presents with heterogeneous manifestations, and has an unpredictable outcome. She related having had several episodes of papules. Feb 28, 2017 langerhans cell histiocytosis lch is one of the histiocytosis disorders, as defined by the histiocyte society. Treatment depends on the site and extent of disease. A langerhans cell is a type of white blood cell that normally helps the body fight off infection. Sometimes there are mutations changes in lch cells as they form.
Commonly seen in infancy or early childhood, the disorder is characterized by proliferation of abnormal and clonal langerhans cell in skin, bone, lymph nodes, lungs, liver, spleen, and bone marrow. The cells build up in the body, sometimes damaging organs or forming tumors. Langerhans cell histiocytosis lch is a rare disorder in which lesions contain cells with features similar to the langerhans cell lc of the epidermis. Langerhans cell histiocytosis lch is a group of rare disorders histologically characterized by the proliferation of langerhans cells. It used to known as eosinophilic granuloma it has been referred to by several eponyms handschullerchristian disease, abtletterersiwe disease or letterersiwe disease, and histiocytosis x. Langerhans cell histiocytosis genetic and rare diseases. Langerhans cell histiocytosis also called histiocytosis x is a rare disorder that primarily affects children.
Pdf langerhans cell histiocytosis lch is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often. In langerhans cell histiocytosis, excess immature langerhans cells usually form tumors called granulomas. Lch is part of a group of syndromes called histiocytoses, which are characterized. It broadly fits into the category of histiocytoses. The relation of langerhans cell histiocytosis to acute leukemia, lymphomas, and other solid tumors. Langerhans cell histiocytosis, abbreviated lch, is a rare disorder of tissue macrophages. Langerhans cell histiocytosis lch is a rare illness, and the disease afflicting the thyroid gland is very uncommon, even in the presence of multisystem involvement.
Sep 11, 2017 langerhans cell histiocytosis lch is a disorder that primarily affects children, but is also found in adults of all ages. Get a printable copy pdf file of the complete article 2. The pathogenic cells are defined by constitutive activation of the mapk signaling pathway. Jan 21, 2020 pulmonary langerhans cell histiocytosis plch is a form of langerhans cell histiocytosis lch that typically occurs in the lungs of adult cigarette smokers 1. More common in childhood 1 3 years old and involves nodal and extranodal sites most common site is bone lesional cells show prominent nuclear grooves with admixed eosinophils and are positive for cd1a, langerin cd207 and s100.
Clonal relationship between precursor tlymphoblastic. Lch is classified as a cancer and sometimes requires treatment with chemotherapy. Langerhans cell histiocytosis lch comprises a wide spectrum of clinical. We report two cases of langerhanscell histiocytosis arising in the context of precursor t. Langerhans cell histiocytosis lch lch is an unusual condition. Vassallo r, ryu jh, colby tv, hartman t, limper ah. However, it was later discovered that the abnormal cells in lch are actually derived from myeloid dendritic cells that exhibit the same. The bone is commonly affected, especially the flat bones, the spine and the long bones.
Langerhans cell histiocytosis show diagnoses week 651. In people with lch, these cells multiply excessively and build up in certain areas of the body. Langerhans cell histiocytosis of the gastrointestinal tract article pdf available in diagnostic histopathology 244 march 2018 with 111 reads how we measure reads. The pathogenesis of plch is unclear, but the discovery of mutations in braf and map2k1 in a subset of plch suggests that at least some cases are dendritic cell neoplasms 2,3, although whether all are neoplastic is not certain 3. In bone this may cause pain and adjacent softtissue swelling, but. Langerhanscell tumours are controversial with regard to the state of differentiation, cause, and whether they represent true neoplastic disease. Langerhans cell histiocytosis johns hopkins surgical. Letterersiwe disease, handschullerchristian disease, and solitary eosinophilic granuloma.
Langerhans cell histiocytosis blood american society. Langerhans cell histiocytosis in the rare disease langerhans cell histiocytosis lch, an excess of cells similar to these cells are produced. 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. Langerhans cell histiocytosis is a clonal proliferation of cells that morphologically and immunophenotypically resemble langerhans cells.
Langerhans cell histiocytosis lch is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. In langerhans cell histiocytosis, excess immature langerhans cells usually form tumors. The disease spectrum results from clonal accumulation and proliferation of cells resembling the epidermal dendritic cells called langerhans cellssometimes called dendritic cell histiocytosis. Differential diagnosis for manifestations of langerhans cell histiocytosis.
Selected aspects of the histopathology of langerhans cell histiocytosis representing diagnostic difficulty andor controversy are presented with emphasis on the composition of pathological lesions. B cell nonhodgkin lymphoma of the follicular subtype grade 33 affecting the nasopharynx and breast, and containing foci of langerhans cell histiocytosis, was diagnosed in a 56 year old white woman who was a longstanding heavy smoker. This can avoid unnecessary biopsy and guide the management especially where access to histopathology is limited. From the department of pathology, university of michigan health. Immunohistochemistry is essential to reach the correct diagnosis. A ninemonthold boy baby was admitted to our dermatology outpatient clinic with persistent. Langerhans cell histiocytosis lch is a rare disease marked by proliferation of langerhanstype cells that share immunophenotypic and ultrastructural similarities with antigenpresenting langerhans cells of mucosal sites and skin. Diagnosis of langerhans cell histiocytosis on fine needle.
Four years before this she had developed stage 1a mixed cellularity hodgkin lymphoma affecting the right inguinal region, which was treated by. Langerhans cell histiocytosis archives of pathology. The cytomorphology of cases of langerhans cell histiocytosis is described. Although topical steroid creams were used, these lesions did not improve. Biopsies of ulcers observed endoscopically at 50 cm and at 30 cm from the anal verge, showed colonic mucosa containing mononuclear cells, with reniform nuclei, fine vesicular chromatin, nuclear grooves, indistinct cell borders and abundant eosinophilic cytoplasm. These cells play a role in the bodys immune system. Includes all cases in which the lung is the principal site of involvement.
Langerhans cell histiocytosis lch is a rare cancer that begins in lch cells. A case of multifocal langerhans cell histiocytosis in a twoyearold child is presented where fine needle aspiration was helpful in achieving a rapid and accurate diagnosis in an appropriate clinical and radiological setting. Benign cephalic histiocytosis bch falls into the group of nonlangerhans cell histiocytosis nonlch, which is characterized by a benign course and tendency toward spontaneous remission. The highly characteristic common and rare cytological. Mar 15, 2018 we report a case of a 28 yearold lady who presented with a history of perianal pain and discharge per rectum over several months. Some lesions in children such as a vertebra plana or a solitary.
Skeletal involvement in langerhans cell histiocytosis. Langerhans cell histiocytosis, often called lch, is a disorder where the body produces too many langerhans cells. The histopathology of langerhans cell histiocytosis. It has some characteristics of cancer but, unlike almost every other cancer, it may spontaneously resolve in some patients while being lifethreatening in others. 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.
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