Langerhans cell histiocytosis and malignancies of the mpire system. Pathology of langerhans cell histiocytosis histiocytosis. Langerhans cell histiocytosis lch is a group of rare disorders histologically characterized by the proliferation of langerhans cells. Treatment depends on the site and extent of disease. The disease spectrum results from clonal accumulation and proliferation of cells resembling the epidermal dendritic cells called langerhans cellssometimes called dendritic cell histiocytosis. Langerhans cell histiocytosis causes, symptoms, diagnosis. Langerhans cell histiocytosis is a malignancy of langerhans cells with immunoreactivity for cd1a and s100 protein and the presence of cytoplasmic birbeck granules. The lchmalignancy study group of the histiocyte society. The highly characteristic common and rare cytological. Langerhans cell histiocytosis is a clonal proliferation of cells that morphologically and immunophenotypically resemble langerhans cells. Langerhans cell histiocytosis also called histiocytosis x is a rare disorder that primarily affects children. Langerhans cell histiocytosis, abbreviated lch, is a rare disorder of tissue macrophages. Admixed are many mononuclear cells with reniform nuclei, nuclear grooves, fine, vesicular chromatin, indistinct cell borders and abundant eosinophilic cytoplasm. Langerhans cell histiocytosis lch is a rare cancer that begins in lch cells.
Sometimes there are mutations changes in lch cells as they form. Langerhans cell histiocytosis lch is currently regarded as a. The pathogenic cells are defined by constitutive activation of the mapk signaling pathway. 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 lch comprises a wide spectrum of clinical. Immunohistochemistry is essential to reach the correct diagnosis. Feb 28, 2017 langerhans cell histiocytosis lch is one of the histiocytosis disorders, as defined by the histiocyte society. Aug 28, langerhans cell histiocytosis lch is a rare histiocytic disorder most in erdheimchester disease but not in other non langerhans cell. Diagnosis of langerhans cell histiocytosis on fine needle. We report two cases of langerhanscell histiocytosis arising in the context of precursor t. Some lesions in children such as a vertebra plana or a solitary. Media in category histopathology of langerhans cell histiocytosis the following 36 files are in this category, out of 36 total. Includes all cases in which the lung is the principal site of involvement. 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.
It has been referred to by several eponyms handschullerchristian disease, abtletterersiwe disease or letterersiwe disease, and histiocytosis x. The cytomorphology of cases of langerhans cell histiocytosis is described. The relationship between langerhans cells and the other histiocytes from which they are derived seems to be determined by events initiated by surface antigens. 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. Langerhanscell histiocytosis has been associated with malignant disease especially acute leukaemias,1 but the biological basis for this link is unknown. It is reported to be between 26 54 cases per million children in the general population. 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.
However, it was later discovered that the abnormal cells in lch are actually derived from myeloid dendritic cells that exhibit the same. Langerhans cell histiocytosis of the gastrointestinal tract. Lch is part of a group of syndromes called histiocytoses, which are characterized. Apart from bch, the main types of nonlch include juvenile xanthogranuloma, generalized eruptive histiocytoma, and xanthoma disseminatum. Langerhans cell histiocytosis lch is a rare disorder in which lesions contain cells with features similar to the langerhans cell lc of the epidermis. 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. Approximately 1015% of cases also involve other sites primarily bone. This disorder is characterized by an abnormal increase in certain immune cells called histiocyte cells. These cells play a role in the bodys immune system. Vassallo r, ryu jh, colby tv, hartman t, limper ah. 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. Pulmonary langerhans cell histiocytosis surgical pathology. 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.
A langerhans cell is a type of white blood cell that normally helps the body fight off infection. Lch cells are arrested in an immature, partially activated stage and show a deviant regulation of cell division. Langerhans cell histiocytosis of the gastrointestinal tract article pdf available in diagnostic histopathology 244 march 2018 with 111 reads how we measure reads. 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.
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. Langerhans cell histiocytosis information from the histiocytosis association. Indeterminate cell histiocytosis and mycosis fungoides. Langerhans cell histiocytosis lch, is a rare disorder, clinically presents with heterogeneous manifestations, and has an unpredictable outcome. Pathology of langerhans cell histiocytosis histiocytosis x.
Langerhans cell histiocytosis treatment pdqpatient. Basic science, clinical features, and therapy, weitzman s, egeler rm eds, cambridge university press, cambridge 2005. Langerhans cell histiocytosis lch represents a disorder characterised by an abnormal accumulation of histiocytes in miscellaneous tissues. From the department of pathology, university of michigan health. Langerhans cell histiocytosis may develop at any age but most commonly occurs in childhood years of age. Clonal relationship between precursor tlymphoblastic. Although the pathogenesis of langerhans cell histiocytosis has been debated. Skin biopsy diagnosis of langerhans cell neoplasms.
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. They can be found in the skin, lungs, stomach, bone, eyes and intestines. Langerhans cell histiocytosis immunohistochemical expression of. The hallmark of lch is the proliferation and accumulation of a specific histiocyte. Langerhans cell histiocytosis lch is a rare disease, which occurs mainly in children. It has some characteristics of cancer but, unlike almost every other cancer, it may spontaneously resolve in some patients while being lifethreatening in others. Langerhanscell histiocytosis lch is caused by an uncontrolled pathogenic clonal proliferation of dendritic cells dcs with langerhanscell lc characteristics. Langerhans cell histiocytosis in the rare disease langerhans cell histiocytosis lch, an excess of cells similar to these cells are produced. From the department of pathology, university of michigan. Langerhans cell histiocytosis blood american society. Langerhans cell histiocytosis of the gastrointestinal.
The bone is commonly affected, especially the flat bones, the spine and the long bones. Differential diagnosis for manifestations of langerhans cell histiocytosis. Get detailed treatment information for lch in this summary for clinicians. Langerhans cell histiocytosis lch is a rare disorder in which the body makes too many dendritic cells. In langerhans cell histiocytosis, excess immature langerhans cells usually form tumors called granulomas. 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. 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.
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. Langerhans cell histiocytosis is a disorder in which excess immune system cells called langerhans cells build up in the body. Skin biopsy diagnosis of langerhans cell neoplasms intechopen. Langerhans cell histiocytosis, often called lch, is a disorder where the body produces too many langerhans cells. Langerhans cell histiocytosis show diagnoses week 651. Pathology of langerhans cell histiocytosis hematologyoncology. Sep 11, 2017 langerhans cell histiocytosis lch is a disorder that primarily affects children, but is also found in adults of all ages. Skeletal involvement in langerhans cell histiocytosis. Lesional cell phenotypes and the factors influencing variations are noted.
Histiocyte cells are a form of white blood cells that help the immune system destroy foreign materials and fight infection. To date, no largescale studies have been done on how often lch occurs in. 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. 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. The cells build up in the body, sometimes damaging organs or forming tumors. The relation of langerhans cell histiocytosis to acute. She related having had several episodes of papules. In people with lch, these cells multiply excessively and build up in certain areas of the body.
Langerhans cell histiocytosis lch is a disorder that primarily affects children, but is also found in adults of all ages. Langerhanscell tumours are controversial with regard to the state of differentiation, cause, and whether they represent true neoplastic disease. 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. 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. Langerhans cell histiocytosis lch lch is an unusual condition. Dendritic cells are a form of histiocyte, or white blood cell. In 20, we reported in the american journal of dermatopathology a case of selfregressing s100negative cutaneous indeterminate cell histiocytosis ich in a 48yearold woman. Selected aspects of the histopathology of langerhans cell histiocytosis representing diagnostic difficulty andor controversy are presented with emphasis on the composition of pathological lesions. Langerhans cell histiocytosis involving the thyroid and. In bone this may cause pain and adjacent softtissue swelling, but. 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. The relation of langerhans cell histiocytosis to acute leukemia, lymphomas, and other solid tumors. 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. Lch cells are a type of dendritic cell which fights infection.
These include mutations of the braf, map2k1, ras and. 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. 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 archives of pathology. 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. 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. Multiple organs and systems may be involved by the disease. 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. We report a case of a 28 yearold lady who presented with a history of perianal pain and discharge per rectum over several months. However lch cells stain positive to cd14 which is a monocyte marker and shows a different, hematopoietic origin for the disorder.
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 is a type of cancer that can damage tissue or cause lesions to form in one or more places in the body. The histopathology of langerhans cell histiocytosis. 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 lch treatment may include observation alone, surgery, radiation therapy, or oral, topical, and intravenous medication. It broadly fits into the category of histiocytoses. Their aberrant interactions with t cells and the lesional microenvironment are typified by high level production of. Four years before this she had developed stage 1a mixed cellularity hodgkin lymphoma affecting the right inguinal region, which was treated by. Four years before this she had developed stage 1a mixed cellularity hodgkin lymphoma affecting the right inguinal region, which was treated by irradiation and. Medical management of langerhans cell histiocytosis from. Although topical steroid creams were used, these lesions did not improve. 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. Symptoms range from isolated bone lesions to multisystem disease.
Selected aspects of the histopathology of langerhans cell histiocytosis. The cytology of langerhans cell histiocytosis histiocytosis. Langerhans cell histiocytosis johns hopkins surgical. Langerhanscell histiocytosis insight into dc biology. 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. 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.
Langerhans cell histiocytosis in sequential discordant. 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. A ninemonthold boy baby was admitted to our dermatology outpatient clinic with persistent. 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. Pdf langerhans cell histiocytosis of the gastrointestinal tract. 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. After six months of follow up, the patient is in good conditions. Lch is classified as a cancer and sometimes requires treatment with chemotherapy. The diagnostic histopathology of langerhans cell histiocytosis. Get a printable copy pdf file of the complete article 2. Langerhans cell histiocytosis involving the gastrointestinal tract.
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