Disorders of Macrophages And Dendritic Cells

Mapping Intimacies ◽

10.2310/im.1311 ◽

2017 ◽

Author(s):

Alexei A. Grom ◽

Michael B Jordan ◽

Jun Qin Mo

Keyword(s):

Dendritic Cells ◽

Hemophagocytic Lymphohistiocytosis ◽

Phagocytic Activity ◽

Langerhans Cells ◽

Langerhans Cell Histiocytosis ◽

Skin Lesions ◽

Dendritic Morphology ◽

Langerhans Cell ◽

Juvenile Xanthogranuloma ◽

Specific Cell

The mononuclear phagocytic system consists of dendritic cells (DCs) and monocytes/macrophages, historically referred to as histiocytes. The macrophages exhibit varying degrees of phagocytic activity. DCs typically have dendritic morphology. Their phagocytic activity is limited. Instead, they play a key role in antigen presentation to lymphocytes. The various populations of macrophages and DCs (such as Langerhans cells [LCs] and dermal dendrocytes) are usually distinguished based on characteristic morphology and patterns of expression of specific cell surface and intracellular markers. Abnormal accumulation and behavior of these cells may lead to the development of a spectrum of diseases collectively known as the histiocytoses. Clinically, histiocytic disorders comprise a wide variety of conditions that affect both children and adults and range from benign skin lesions to rapidly progressive life-threatening systemic disorders. LCs play a pivotal role in the development of Langerhans cell histiocytosis, dermal dendrocytes are the predominant cell population in the lesions of juvenile xanthogranuloma, and macrophages are central to the pathogenesis of hemophagocytic lymphohistiocytosis and related disorders. This review contains 5 figures, 3 tables, and 77 references. Key words: dendritic cells, dermal dendrocytes, hemophagocytic lymphohistiocytosis, histiocytes, juvenile xanthogranuloma, Langerhans cell histiocytosis, Langerhans cells, macrophage activation syndrome, macrophages

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Langerhans Cell Histiocytosis: Back to Histiocytosis X

Blood ◽

10.1182/blood.v120.21.sci-8.sci-8 ◽

2012 ◽

Vol 120 (21) ◽

pp. SCI-8-SCI-8

Author(s):

Carl E. Allen

Keyword(s):

Dendritic Cells ◽

Dendritic Cell ◽

Langerhans Cells ◽

Langerhans Cell Histiocytosis ◽

Conflicts Of Interest ◽

Langerhans Cell ◽

Histiocytosis X ◽

Specific Gene ◽

Myeloid Dendritic Cell ◽

Epidermal Langerhans Cells

Abstract Abstract SCI-8 Langerhans cell histiocytosis (LCH) is a disorder characterized by inflammatory lesions that include pathologic CD207+ dendritic cells. LCH has pleotropic clinical presentations ranging from single lesions cured by curettage to potentially fatal multisystem disease. The first descriptions of LCH, including Hand-Schüller-Christian disease and Letterer-Siwe disease, were based on anatomic location and extent of the lesions. Despite clinical heterogeneity, LCH lesions are generally indistinguishable by histology, which led to the notion that the spectrum of clinical manifestations represents a single disorder, histiocytosis X. The designation “Langerhans cell histiocytosis” was subsequently proposed with discovery of cytoplasmic Birbeck granules in the pathologic infiltrating dendritic cells in histiocytosis X lesions, a feature shared by epidermal Langerhans cells. The etiology of LCH remains elusive, and debate of LCH as an inflammatory versus malignant disorder remains unresolved. However, recent discoveries question the model of LCH arising from transformed or pathologically activated epidermal Langerhans cells. We found cell-specific gene expression signature in CD207+ dendritic cells within LCH lesions to be more consistent with immature myeloid dendritic cell precursors than epidermal Langerhans cells. Furthermore, recent mouse studies demonstrate that CD207+ is more promiscuous than previously appreciated. Langerin (CD207) expression can be induced in many dendritic cell lineages, supporting the plausibility of a spectrum of candidates for an LCH cell of origin, including circulating dendritic cell precursors. Finally, recurrent activating BRAF mutations in LCH lesions suggest a role for a hyperactive RAS pathway in LCH pathogenesis, and possibly in normal dendritic cell development. This presentation will discuss the historical background and recent advances in LCH biology, along with a proposal to reframe “histiocytosis X” as a myeloid neoplasia caused by aberrant maturation and migration of myeloid dendritic cell precursors. Disclosures: No relevant conflicts of interest to declare.

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Pulmonary Langerhans’ cell histiocytosis

Oxford Textbook of Medicine ◽

10.1093/med/9780198746690.003.0425 ◽

2020 ◽

pp. 4256-4257

Author(s):

S. J. Bourke

Keyword(s):

Bone Marrow ◽

Dendritic Cells ◽

Genital Tract ◽

Langerhans Cells ◽

Langerhans Cell Histiocytosis ◽

Female Genital Tract ◽

Cyst Formation ◽

Langerhans Cell ◽

Pulmonary Langerhans Cell Histiocytosis ◽

Systemic Symptoms

Pulmonary Langerhans’ cell histiocytosis is characterized by a reactive monoclonal proliferation of activated histiocytes in the distal bronchioles, resulting in inflammatory nodules, cyst formation, and fibrosis. Langerhans’ cells are a particular type of histiocyte derived from dendritic cells in the bone marrow. They normally migrate in the blood to the squamous epithelium of the skin, lungs, gastrointestinal, and female genital tract, where they are involved in antigen presentation to T cells. It presents with cough, breathlessness, and (sometimes) systemic symptoms. Chest radiography and CT typically show nodules which then cavitate and may rupture, causing pneumothorax. Corticosteroids and/or cytotoxic drugs are of some benefit, and lung transplantation is an option for progressive disease.

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Advanced ulcerative tumefactive lesions of the mucocutaneous surface in previously unrecognized Langerhans Cell Histiocytosis

Our Dermatology Online ◽

10.7241/ourd.20214.15 ◽

2021 ◽

Vol 12 (4) ◽

pp. 419-421

Author(s):

Fatima Azzahra Elgaitibi ◽

Soumaya Hamich ◽

Noureddine Mahiou

Keyword(s):

Langerhans Cells ◽

Langerhans Cell Histiocytosis ◽

Rare Case ◽

Clinical Presentation ◽

Histopathological Examination ◽

Systemic Disease ◽

Skin Lesions ◽

Clinical Suspicion ◽

Langerhans Cell ◽

Multiple Ulcers

Langerhans cell histiocytosis (LCH) is an uncommon systemic disease characterized by the infiltration of one organ or more by Langerhans cells. Its clinical presentation is heterogeneous and depends on the affected organs. We report the rare case of a 43-year-old female presenting herself with central diabetes insipidus and receiving nasal desmopressin spray. Later, the patient developed multiple papules and pustules progressing to multiple ulcers affecting the retroauricular and axillary folds, as well as the oral and genital mucosas. The skin lesions had been evolving for several months. A histopathological examination of a skin biopsy confirmed the clinical suspicion of LCH.

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Langerhans cell histiocytosis: Skin diseases and visceral lesions

Terapevticheskii arkhiv ◽

10.17116/terarkh2017894115-120 ◽

2017 ◽

Vol 89 (4) ◽

pp. 115-120

Author(s):

V D Elkin ◽

T G Sedova ◽

E A Kopytova ◽

E V Plotnikova

Keyword(s):

Langerhans Cells ◽

Langerhans Cell Histiocytosis ◽

Skin Diseases ◽

Clinical Manifestations ◽

Skin Lesions ◽

Langerhans Cell ◽

Literature Analysis ◽

Trigger Factors ◽

Statistical Variations ◽

The World

Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder that belongs to class I histiocytoses and is characterized by infiltration of one or many organs by Langerhans cells to form granulomas. The literature analysis could identify a lot of etiological, pathogenetic, and trigger factors and mechanisms for LCH development, which determine the diversity of the clinical picture and course of the disease. The clinical manifestations of LCH are very variable and depend on the severity of lesions and the age of patients. In addition to skin lesions, there is involvement of one or more visceral organs. Difficulties in diagnosing the disease lead to statistical variations of LCH in different countries of the world and require more attention by physicians of all specialties.

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Multisystem Langerhans Cell Histiocytosis With Hypothyroidism and Sclerosing Cholangitis: a Case Report and Literature Review

10.21203/rs.3.rs-439626/v1 ◽

2021 ◽

Author(s):

Xiaoyan Liu ◽

Shuangshuang Xie ◽

Wenjun Du

Keyword(s):

Dendritic Cells ◽

Sclerosing Cholangitis ◽

Langerhans Cell Histiocytosis ◽

Treatment Options ◽

Fine Needle Biopsy ◽

Skin Lesions ◽

Langerhans Cell ◽

Response To Treatment ◽

Treatment Needs ◽

Multiple Organs

Abstract Background: Langerhans cell histiocytosis (LCH) is a rare disease caused by a disorder of dendritic cells. LCH mainly occurs in children and the tissue damage is caused by proliferative dendritic cells. In most LCH patients, only a single organ is affected, whereas some cases may involve multiple organs, such as bone marrow, liver, lungs, and brain.Case presentation: Herein we present an LCH case, that involved a multisystem: the skin, lungs, thyroid, liver, nails, and spleen. Low appetite, fatigue, and jaundice were the main symptoms. The patient had extensive skin lesions, nail lesions, macular sclera, and bilateral goiter. The erythrocyte sedimentation rate (ESR) was higher than normal. Lymphocytic thyroiditis was diagnosed by fine needle biopsy and skin rash. A liver biopsy revealed sclerosing cholangitis (SC). Response to treatment with Etoposide（VP-16), Prednisone acetate tablets, cyclophosphamide, and vinblastine was excellent. With disease remission, the corresponding ESR levels decreased.Conclusions: LCH involved not only the skin and thyroid, but also liver, lungs, nails and possibly the spleen in this case. Currently there are no standard treatment options and the treatment needs to be individualized. It is rare that LCH with sclerosing cholangitis is the first presentation, and we should be vigilant to whether other organs are involved. An elevated ESR may be a valuable indicator of disease activity in patients with Langerhans cell histiocytosis.

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Langerhans cell histiocytosis in an infant: a case report from North-East India

International Journal of Research in Dermatology ◽

10.18203/issn.2455-4529.intjresdermatol20204569 ◽

2020 ◽

Vol 6 (6) ◽

pp. 792

Author(s):

Romita Bachaspatimayum ◽

Kago Yaku

Keyword(s):

Dendritic Cells ◽

Case Report ◽

Skin Biopsy ◽

Langerhans Cell Histiocytosis ◽

Reticuloendothelial System ◽

Skin Lesions ◽

Langerhans Cell ◽

Female Infant ◽

North East India ◽

North East

Langerhans cell histiocytosis (LCH) is a rare disorder of reticuloendothelial system characterized by uncontrolled proliferation and accumulation of CD1a and CD207 dendritic cells (DCs). 3-4 cases per million occur annually in children under 15 years of age, with a male:female ratio of 2:1 and peak incidence in infants aged 1 to 2 years. Report a case of LCH in a month-old female infant with skin lesions for 2-3 weeks. Skin biopsy was suggestive of LCH and it was confirmed with immunohistochemistry markers that were positive for S100, CD1A and negative for CD68.

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Unusual sites of bone involvement in Langerhans cell histiocytosis: a systematic review of the literature

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-020-01625-z ◽

2021 ◽

Vol 16 (1) ◽

Author(s):

Nahid Reisi ◽

Pouran Raeissi ◽

Touraj Harati Khalilabad ◽

Alireza Moafi

Keyword(s):

Systematic Review ◽

Dendritic Cells ◽

Rare Disease ◽

Langerhans Cell Histiocytosis ◽

Case Reports ◽

Langerhans Cell ◽

The Other ◽

Bone Involvement ◽

Intracranial Extension ◽

Pathologic Fractures

Abstract Background Langerhans cell histiocytosis (LCH) is a rare disease that originates from the uncontrolled proliferation and accumulation of bone marrow-derived immature myeloid dendritic cells. Dendritic cells are a type of histiocyte that play an important role in the human immune system and are found in the bone, skin, stomach, eyes, intestines, and lungs. Objective This systematic review aimed to collect and report published case reports of rare bone disease caused by LCH to avoid misdiagnoses or delays in diagnosis. Methods We systematically searched Scopus, PubMed, Embase, and Web of Sciences from August 1, 2000 to December 31, 2019. Studies reporting cases of LCH with rare bone involvement were included. Results We identified 60 articles including 64 cases. Of the identified cases, 31 (48.4%) involved children, and 33 (51.6%) involved adults. Additionally, 46.9% (30 individuals) were from Asian countries. The mean age of the children was 7.6 ± 4.3 years and that of the adults was 36 ± 12 years. The findings indicated that unifocal bone involvements were the most prevalent form of the disease (68.7%), and, overall, the skull and chest wall were the most commonly affected bones in both adults and children. The spine and long bones were the second most commonly affected bones in children, and the spine and jaw were the second most commonly affected bones in adults. Pain and swelling were the most frequent presenting signs among the investigated cases, and loss of consciousness, myelopathy, nerve palsy, visual loss, torticollis and clicking sounds were rare signs. Osteolytic lesions were the most frequent radiologic feature (62.5%), and intracranial hemorrhage, fluid–fluid level, dura and intracranial extension and pathologic fractures were rare radiological features. Total excision, curettage and observation in the unifocal group of patients and systemic chemotherapy in the other groups (i.e., multifocal and multisystem) were the most frequent management approaches. The recovery rates of the unifocal and multifocal groups were 77.3% and 81.8%, respectively, while that of the multisystem group was 55.5%. The rates of recurrence and mortality in the multisystem group were 11% and were higher than those in the other groups. Conclusions LCH is a rare disease that can affect any organ in the human body. However, bone is the most commonly involved organ, and rare bone involvements may be the first or only symptom of the disease due to the rarity of such lesions; a lack of familiarity with them may result in misdiagnosis or delayed diagnosis.

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