Histiocytes and nonlangerhans cell histiocytoses in. We report a young woman with primary cerebral nonlangerhans cell histiocytosis of the juvenile xanthogranuloma family. The nonlangerhans cell histiocytoses sheila weitzman, mb 1,2 and ronald jaffe, mb, bch 3 introduction histiocytic disorders are currently identi. Get detailed treatment information for lch in this summary for clinicians. Nov 01, 2005 erdheimchester disease is a rare non langerhans histiocytosis with multisystem involvement. Nonlangerhans cell histiocytosis refers to a family of histiocytosis characterized by the absence of langerhans cells many manifest cutaneously. Treatment can include chemotherapy, surgery, radiation therapy, photodynamic therapy, immunotherapy, and targeted therapy. The clinical course was complicated by extensive infiltration of cranial nerves and meninges and epi and intramedullary spinal. A person of any age can get histiocytosis, but it is most common in children 1 to 3 years old. Medical management of langerhans cell histiocytosis from. Aug 28, langerhans cell histiocytosis lch is a rare histiocytic disorder most in erdheimchester disease but not in other nonlangerhans cell. Class iia histiocytosis involves dermal dendritic cells.
Langerhans cell histiocytosis is most likely to damage bones, but it can affect many body systems. Bilateral thickening of the trigeminal nerve was characterized by low signal intensity on t2weighted images and intense. Nonlangerhans cell histiocytosis nonlch is a rare multisystemic disorder thought to be derived from dendritic cells or macrophages. Langerhans cell histiocytosis, cranial diabetes insipidus, idiopathic thrombocytopenic, purpura, hypothalamicpituitary axis introduction langerhans cell histiocytosis lch is a rare disease, more so in adults, characterised by proliferation of large mononuclear cells. We report a yearold asymptomatic male smoker with a non specific interstitial infiltrate found in preventive chest x ray examination. Langerhans cell histiocytosis information from the histiocytosis association. The non langerhans cell histiocytoses non lch are a group of disorders defined by the accumulation of histiocytes that do not meet the phenotypic criteria for the diagnosis of langerhans cells lcs. 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. 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. Like other forms of histiocytosis, nonlangerhans cell histiocytosis tends to cause reddishbrown or reddishyellow bumps in the skin and may affect internal organs such as liver, kidneys, lungs. Erdheimchester disease ecd is a rare nonlangerhans cell histiocytosis that most commonly affects adults and is driven by a high frequency of mutations in braf, map2k1, and kinases promoting mapk signaling. Bilateral thickening of the trigeminal nerve was characterized by low signal intensity on t2weighted images and intense enhancement on t1weighted images after contrast. 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.
Juvenile xanthogranuloma jxg, erdheimchester disease ecd, and rosaidorfman disease rdd previously were categorized as nonlch disorders. Mri showed pachymeningeal and subependymal enhancement. Erdheimchester disease is a rare nonlangerhans histiocytosis with multisystem involvement. Treatment depends on the site and extent of disease. 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. Aug 24, 2017 erdheimchester disease ecd is a rare nonlangerhans cell histiocytosis that most commonly affects adults and is driven by a high frequency of mutations in braf, map2k1, and kinases promoting mapk signaling. Sep 28, 2006 generalized eruptive histiocytosis belongs to cutaneous nonlangerhans cell histiocytoses and is a rare, generalized, selfhealing disorder that usually follows a benign clinical course. Symptoms range from isolated bone lesions to multisystem disease. Because of the relative rarity of ecd, key clinical features of the disease may not be well defined. In contrast, nonlangerhans histiocytoses are thought to be derived from the monocytemacrophage lineage. A case of generalized eruptive histiocytosis html acta.
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. Langerhans cell histiocytosis in nontwin siblings request pdf. Histiocyte cells are a form of white blood cells that help the immune system destroy foreign materials and fight infection. 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. 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 non lch consist of a long list of diverse disorders which have been difficult to categorize. This group has generally been divided into langerhans cell histiocytosis lch and non langerhans histiocytosis. In these histiocytosis groups, perhaps the most intriging, controversial and unknown ones are non langerhans histiocytoses. Langerhans cell histiocytosis radiology reference article. Reviews unusual variants of nonlangerhans cell histiocytoses ruggero caputo, md,a angelo valerio marzano, md,a emanuela passoni, md,a and emilio berti, mdb milan, italy histiocytic syndromes represent a large, heterogeneous group of diseases resulting from proliferation of.
Langerhans cell histiocytosis is a rare, multisystem disease that shows a particular predilection for hypothalamopituitary axis involvement. Histiocyte society revised classification of histiocytoses and neoplasms of the macrophagedendritic cell lineages 3. Langerhans cell histiocytosis is a disorder in which excess immune system cells called langerhans cells build up in the body. Langerhans cells are a type of white blood cell that normally help the body fight infection. Langerhans cell histiocytosis is due to uncontrolled monoclonal proliferation of langerhans cells distinctive cells of monocytemacrophage lineage and should be considered a malignancy although its biological behavior is very variable 1,3. Like other forms of histiocytosis, non langerhans cell histiocytosis tends to cause reddishbrown or reddishyellow bumps in the skin and may affect internal organs such as liver, kidneys, lungs. Multisystem radiology 2008 more than just langerhans cell histiocytosis. Langerhans cell histiocytosis also called histiocytosis x is a rare disorder that primarily affects children. Request pdf langerhans cell histiocytosis in nontwin siblings langerhans cell histiocytosis lch, which has unknown pathogenesis, can manifest as many kinds of signs and symptoms at any age.
The signs and symptoms of lch depend on where it is in. Abstract histiocytoses are disorders characterised by inflammation and the accumulation of cells derived from the monocyte and macrophage lineages, which results in tissue damage. 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. Pdf primary cerebral nonlangerhans cell histiocytosis. Diabetes insipidus is the most frequent permanent consequence of langerhans cell histiocytosis, developing in around a quarter of patients. In this paper, non langerhans histiocytoses of particular interest for the dermatologist are examined. This group has generally been divided into langerhans cell histiocytosis lch and nonlangerhans histiocytosis. 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. Non langerhans cell histiocytosis refers to a family of histiocytosis characterized by the absence of langerhans cells many manifest cutaneously. Aug 28, langerhans cell histiocytosis lch is a rare histiocytic disorder most in erdheimchester disease but not in other non langerhans cell. It has been proposed that benign cephalic histiocytosis bch may be a variant of micronodular juvenile. Intherightclinicalcontext,lesionalcells that are cd1a.
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%. Langerhans cell histiocytosis lch treatment may include observation alone, surgery, radiation therapy, or oral, topical, and intravenous medication. We report a young woman with primary cerebral non langerhans cell histiocytosis of the juvenile xanthogranuloma family. Langerhans cell histiocytosis lch is a heterogeneous disease, characterized by accumulation of dendritic cells with features similar to epidermal langerhans cells in various organs. Langerhans cell histiocytosis is often diagnosed in childhood, usually between ages 2 and 3, but can appear at any age. To date, no largescale studies have been done on how often lch occurs in. We also recognized the correlation between pregnancychildbirth. Lch is sonamed for its presumed derivation from the langerhans cells specialized dendritic cells found in the skin and mucosa. Endolymphatic nonlangerhans cell histiocytosis of the. Histiocytic disorders are currently identified by their component cells. Non langerhans cell histiocytosis nlch, or class ii histiocytosis, represents a broad group of different disorders characterized by the proliferation of histiocytes other than langerhans cell lc table 1481. Histiocytosis association 332 north broadway pitman, nj 08071 tollfree.
To date, there is no standard treatment for this disorder, and more than half of the patients succumb within 3 years. In langerhans cell histiocytosis, excess immature langerhans cells usually form tumors called. Lch is part of a group of syndromes called histiocytoses, which are characterized. Endolymphatic nonlangerhans cell histiocytosis of the larynx. We found two major groups regarding the initial symptoms. Langerhans cell histiocytosis lch, secondary dendritic cell. A nonlangerhans cell histiocytosis, benign cephalic histiocytosis is characterized by scattered yellowbrown papules and macules concentrated on face, scalp, neck, and upper trunk that appear in the first 2 years of life, though typically after birth. These symptoms can also be caused by other health problems. It often damages bones, especially the skull, legs, hips, spine and arms.
A non langerhans cell histiocytosis, benign cephalic histiocytosis is characterized by scattered yellowbrown papules and macules concentrated on face, scalp, neck, and upper trunk that appear in the first 2 years of life, though typically after birth. Most individuals with adultonset langerhans cell histiocytosis are current or past smokers. Management of adult langerhans cell histiocytosis based on. 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. Progressive mucinous histiocytosis is a rare, benign, nonlangerhans cell histiocytosis limited to the skin. Histiocytes and nonlangerhans cell histiocytoses in dermatology. Aug 15, 2019 when found in the lungs, it should be. Herein, we describe a unique case of multisystem nonlangerhans cell histiocytic proliferation with a fulminant clinical course death occurred within 3 months of presentation that showed prominent hp and extensive involvement of multiple organs, including the. Erdheimchester disease is a rare, nonlangerhans cell histiocytosis histologically characterized by multisystemic proliferation of mature histiocytes in a background of. In contrast, non langerhans histiocytoses are thought to be derived from the monocytemacrophage lineage. Based on our series of 111 cases of non langerhans cell histiocytoses, we present a new unifying concept for this rare group of disorders. Herein, we report a case of generalized eruptive histiocytosis in a 41yearold woman with peculiar clinical and histological features. This proliferation is accompanied by inflammation and.
Unusual variants of nonlangerhans cell histiocytoses. C group cutaneous and mucocutaneous histiocytoses nonlangerhans cell histiocytosis of skin and mucosa m group malignant histiocytoses. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for non langerhans cell histiocytosis. Langerhans cell histiocytosis seattle childrens hospital. Apart from bch, the main types of nonlch include juvenile xanthogranuloma, generalized eruptive histiocytoma, and xanthoma disseminatum. Ten casesall womenin four families and one sporadic case have been described in. Langerhans cell histiocytosis genetics home reference nih. Progressive mucinous histiocytosis is a rare, benign, non langerhans cell histiocytosis limited to the skin.
Endocrine manifestations in langerhans cell histiocytosis. Langerhans cell histiocytosis lch treatment depends on where it is found in the body and whether the lch is low or high risk. The non langerhans cell histiocytoses non lch are a group of disorders defined by the accumulation of histiocytes that do not meet the phenotypic criteria for the diagnosis of langerhans cell. Lch is part of a group of syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes an. Histiocytosis occurs predominantly in children and young adults see table 20. C group cutaneous and mucocutaneous histiocytoses non langerhans cell histiocytosis of skin and mucosa m group malignant histiocytoses. Determinants of survival in pulmonary langerhans cell granulomatosis histiocytosis x.
Nonlangerhans types of histiocytosis also occur fig. Based on our series of 111 cases of nonlangerhans cell histiocytoses, we present a new unifying concept for this rare group of disorders. The nonlch consist of a long list of diverse disorders which have been dif. The nonlangerhans cell histiocytoses nonlch are a group of disorders defined by the accumulation of histiocytes that do not meet the phenotypic criteria for the diagnosis of langerhans cells lcs. The non lch consists of a long list of diverse disorders which have been difficult to categorize. Isolated bone marrow nonlangerhans cell histiocytosis. Fulminant multisystem nonlangerhans cell histiocytic. Langerhans cell histiocytosis treatment pdqpatient. The nonlangerhans cell histiocytoses nonlch are a group of disorders defined by the accumulation of histiocytes that do not meet the phenotypic criteria for the diagnosis of langerhans cell. This disorder is characterized by an abnormal increase in certain immune cells called histiocyte cells. Your childs symptoms will depend on how langerhans cell histiocytosis affects their body. Nonlangerhans cell histiocytosis nlch, or class ii histiocytosis, represents a broad group of different disorders characterized by the proliferation of histiocytes other than langerhans cell lc table 1481. Histiocytosis, nonlangerhanscell active research protocols.
Langerhans cell histiocytosis lch is one of the histiocytosis disorders, as defined by the histiocyte society. Successful treatment of erdheimchester disease, a non. Nonlangerhanscell histiocytosis genetic and rare diseases. The common denominator is the monocyte macrophage, which present with various histologic features probably due to the influence of cytokines. Not all children with the disease have the same symptoms. Nonlangerhans cell histiocytosis refers to a family of histiocytosis characterized by the absence of langerhans cells. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for nonlangerhanscell histiocytosis. Generalized eruptive histiocytosis belongs to cutaneous nonlangerhans cell histiocytoses and is a rare, generalized, selfhealing disorder that usually follows a benign clinical course. Although the exact prevalence of anterior pituitary hormone deficiencies is not known, it is probably high and.
The nonlch consist of a long list of diverse disorders which have been difficult to categorize. Family history of cancer or having a parent who was exposed to certain chemicals may increase the risk of lch. Oct 25, 2012 langerhans cell histiocytosis lch is a heterogeneous disease, characterized by accumulation of dendritic cells with features similar to epidermal langerhans cells in various organs. The nonlch consists of a long list of diverse disorders which have been difficult to categorize. High prevalence of myeloid neoplasms in adults with non. Non langerhans types of histiocytosis also occur fig.
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