11,575 results match your criteria Langerhans Cell Histiocytosis


Cutaneous Langerhans Cell Histiocytosis as Presenting Sign of Systemic B-Cell Lymphoma.

Am J Dermatopathol 2021 May 11. Epub 2021 May 11.

Department of Dermatology, West Virginia University, Morgantown, WV; Department of Pathology, Penrose-St. Francis Health Services, Colorado Springs, CO; and Department of Pathology, Kaiser Permanente Washington, Seattle, WA.

Abstract: Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder most commonly involving the bone and skin; however, it can arise in many other locations. It is predominantly a pediatric disease, but adult cases occur. Here, we present a case of adult-onset cutaneous LCH with systemic symptoms, believed to represent disseminated LCH. Read More

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Serial assessments of anterior pituitary hormones in a case of mixed histiocytosis representing Langerhans cell histiocytosis overlapping with Erdheim-Chester disease.

Endocrinol Diabetes Metab Case Rep 2021 May 1;2021. Epub 2021 May 1.

Department of Endocrinology and Metabolism, Tsukuba University Hospital Mito Clinical Education and Training Center, Mito Kyodo General Hospital, Miyamachi, Mito-shi, Ibaraki, Japan.

Summary: A 61-year-old man developed central diabetes insipidus caused by mixed histiocytosis (MH) representing Langerhans cell histiocytosis overlapping with Erdheim-Chester disease. Bone, skin, vascular, and retroperitoneal involvements were also observed. Dynamic hormonal testing showed normal responses for anterior pituitary hormones, except for impaired secretion of growth hormone (GH). Read More

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Intra-arterial Melphalan for Neurologic Non-Langerhans-Cell Histiocytosis.

Neurology 2021 May 12. Epub 2021 May 12.

Jasmine H Francis MD FACS, Ophthalmic Oncology Service, Memorial Sloan Kettering Cancer Center, New York, NY; Y Pierre Gobin MD, Department of Neurosurgery, Weill Cornell Medical Center, New York, NY; Ruham A Nasany MD, Department of Neurology, Memorial Sloan Kettering Cancer Center, New York, NY; Jared Knopman MD, Department of Neurosurgery, Weill Cornell Medical Center, New York, NY; Gary A Ulaner MD, Department of Radiology, Memorial Sloan Kettering Cancer Center, New York, NY and Molecular Imaging and Therapy, Hoag Family Cancer Institute, Newport Beach, CA; Katherine S. Panageas DrPH, Department of Epidemiology and Statistics, Memorial Sloan Kettering Cancer Center, New York, NY; Vaios Hatzoglou MD, Department of Radiology, Memorial Sloan Kettering Cancer Center, New York, NY; Kimberly Salvaggio MSN FNP, Department of Neurosurgery, Weill Cornell Medical Center, New York, NY; David H Abramson MD FACS, Ophthalmic Oncology Service, Memorial Sloan Kettering Cancer Center, New York, NY; Athos Patsalides MD, Department of Neurosurgery, Weill Cornell Medical Center, New York, NY and Eli L Diamond MD, Department of Neurology, Memorial Sloan Kettering Cancer Center, New York, NY.

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Presentation of External Ear Rosai-Dorfman Disease With Laryngeal Involvement.

Ear Nose Throat J 2021 May 11:1455613211016704. Epub 2021 May 11.

Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, TX, USA.

Rosai-Dorfman disease (RDD) is a rare benign systemic histiocytic proliferation characterized by massive lymph node enlargement and sometimes associated with extranodal involvement. Even though it is considered to be benign, death can occur depending on the extent and location. Our case highlights a primary extranodal site of the right pinna with extension through the Eustachian tube to the subglottis. Read More

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Radiologic findings that aid in the reduction of misdiagnoses of Langerhans cell histiocytosis of the bone: a retrospective study.

World J Surg Oncol 2021 May 10;19(1):146. Epub 2021 May 10.

Department of Radiology, The Affiliated Hospital of Qingdao University, Qingdao, 266003, China.

Background: This study aimed to identify the characteristic radiological signs for the diagnosis of Langerhans cell histiocytosis (LCH) of the bone.

Methods: We retrospectively studied 82 cases of LCH with bone lesions confirmed by pathology. Clinical and radiological features of the patients were analyzed. Read More

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BRAF-induced senescence drives Langerhans cell histiocytosis pathophysiology.

Nat Med 2021 May 6. Epub 2021 May 6.

Precision Immunology Institute, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Langerhans cell histiocytosis (LCH) is a potentially fatal condition characterized by granulomatous lesions with characteristic clonal mononuclear phagocytes (MNPs) harboring activating somatic mutations in mitogen-activated protein kinase (MAPK) pathway genes, most notably BRAF. We recently discovered that the BRAF mutation can also affect multipotent hematopoietic progenitor cells (HPCs) in multisystem LCH disease. How the BRAF mutation in HPCs leads to LCH is not known. Read More

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Coexisting -Mutated Langerhans Cell Histiocytosis and Primary Myelofibrosis with Shared Mutation.

Case Rep Hematol 2021 16;2021:6623706. Epub 2021 Apr 16.

Department of Hematology, Aarhus University Hospital, Palle Juul-Jensens Boulevard 35, Aarhus N 8200, Denmark.

Langerhans cell histiocytosis (LCH) is an infrequent disease, characterized by oligoclonal proliferation of immature myeloid-derived cells. However, the exact pathogenesis remains unknown. In rare cases, LCH is present in patients with concomitant myeloid proliferative neoplasms. Read More

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Ultrasound-assisted diagnosis of Langerhans cell hyperplasia of the sternum: A case report.

Radiol Case Rep 2021 Jun 18;16(6):1535-1538. Epub 2021 Apr 18.

Department of Ultrasound, Jinan Central Hospital, Shandong First Medical University & Shandong Academy of Medical Sciences, Jinan 250012, P.R. China.

Langerhans cell hyperplasia is a group of diseases characterized by the proliferation or dissemination of the Langerhans cell, which can come in the form of localized benign lesions, or extensive disseminated invasive neoplastic lesions. These lesions mainly invade the patient's bone. In this paper, we describe the case of a 3-year-old boy who was admitted to the pediatric department with sternum pain. Read More

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Otologic Manifestations of Langerhans Cell Histiocytosis: A Systematic Review.

Otolaryngol Head Neck Surg 2021 May 4:1945998211004590. Epub 2021 May 4.

Hackensack University Medical Center, Hackensack, New Jersey, USA.

Objective: To perform a systematic review to investigate common otologic manifestations of Langerhans cell histiocytosis, the incidence of these findings, methods for diagnosis, as well as medical and surgical management.

Data Sources: PubMed/MEDLINE, Embase, and Cochrane Library.

Review Methods: A search of PubMed/MEDLINE, Embase, and Cochrane Library for all articles published between 1963 to 2020 was performed with variations and combinations of the following search terms: Langerhans cell histiocytosis, eosinophilic granuloma, Letterer-Siwe, Hand-Schüller-Christian, otitis, otologic, ear. Read More

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Pulmonary Langerhans Cell Histiocytosis in a young Non-Smoking Female --Too many Rituals spoil the Lung.

Acta Biomed 2021 Apr 30;92(S1):e2021138. Epub 2021 Apr 30.

Department of Pulmonary Medicine Sarojini Naidu Medical College, Agra.

Pulmonary Langerhans cell histiocytosis (PLCH) is a rare idiopathic cystic interstitial granulomatous lung disease seen almost exclusively in cigarette smokers. It typically occurs between 20-40 years of age and equally distributed among both sexes. Management includes smoking cessation, avoidance of second-hand smoke, and close follow-up. Read More

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Recent advances in the understanding of the molecular pathogenesis and targeted therapy options in Langerhans cell histiocytosis.

Blood Res 2021 Apr;56(S1):S65-S69

Division of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder caused by the clonal expansion of myeloid precursors that differentiate into CD1a+/CD207+ cells in the lesion. Advances in genomic sequencing techniques have improved our understanding of the pathophysiology of LCH. Activation of the mitogen-activated protein kinase (MAPK) pathway is a key molecular mechanism involved in the development of LCH. Read More

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Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective Study.

Medicina (Kaunas) 2021 Apr 7;57(4). Epub 2021 Apr 7.

Department of Orthopedic Surgery, Kindai University Hospital, Osaka-Sayama City, Osaka 589-8511, Japan.

: Langerhans cell histiocytosis (LCH) is a rare disease characterized by the infiltration of one or more organs by Langerhans cell-like dendritic cells. LCH often involves the bone, and its clinical evidence is limited. The purpose of this study is to report on the treatment of LCH at our institution and to add to the evidence for LCH. Read More

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Histiocytic and Dendritic Cell Sarcomas of Hematopoietic Origin Share Targetable Genomic Alterations Distinct from Follicular Dendritic Cell Sarcoma.

Oncologist 2021 Apr 27. Epub 2021 Apr 27.

Foundation Medicine, Inc., Cambridge, Massachusetts, USA.

Background: Histiocytic and dendritic cell neoplasms are a diverse group of tumors arising from monocytic or dendritic cell lineage. Whereas the genomic features for Langerhans cell histiocytosis and Erdheim-Chester disease have been well described, other less common and often aggressive tumors in this broad category remain poorly characterized, and comparison studies across the World Health Organization diagnostic categories are lacking.

Methods: Tumor samples from a total of 102 patient cases within four major subtypes of malignant histiocytic and dendritic cell neoplasms, including 44 follicular dendritic cell sarcomas (FDCSs), 41 histiocytic sarcomas (HSs), 7 interdigitating dendritic cell sarcomas (IDCSs), and 10 Langerhans cell sarcomas (LCSs), underwent hybridization capture with analysis of up to 406 cancer-related genes. Read More

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Adult multifocal Langerhan's cell histiocytosis involving periodontal tissues.

J Indian Soc Periodontol 2021 Mar-Apr;25(2):166-170. Epub 2021 Mar 1.

Department of Periodontology, Narayana Dental College and Hospital, Nellore, Andhra Pradesh, India.

Langerhans cell histiocytosis (LCH) is a rare inflammatory neoplasia characterized by uncontrolled stimulation and abnormal proliferation of Langerhans cells (LCs). It can present as unifocal or multifocal with local and systemic manifestations which involves various internal organs and mucosal tissues. The clinical course varies among individuals, ranges from spontaneous resolution to life-threatening conditions with multisystem involvement. Read More

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BRAFV600E mutation in cell-free DNA, rather than in lesion tissues, at diagnosis is an independent prognostic factor in children with Langerhans cell histiocytosis.

Mol Cancer Ther 2021 Apr 20. Epub 2021 Apr 20.

Laboratory of Hematologic Diseases, Beijing Children's Hospital, Capital Medical University

The aim of this study was to investigate the prognostic significance of BRAFV600E in cell-free (cf) DNA (cfBRAFV600E) and lesion tissues (ltBRAFV600E) in pediatric LCH. This study included a total of 140 patients with successfully detected cfBRAFV600E and ltBRAFV600E at diagnosis. Treatment response at week 6 was correlated with both cfBRAFV600E and ltBRAFV600E. Read More

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Infantile Eosinophilic Pustular Folliculitis: A Case Report.

J Cosmet Dermatol 2021 Apr 20. Epub 2021 Apr 20.

Department of Pathology, Selcuk University Faculty of Medicine, Konya, Turkey.

Background: Infantile eosinophilic pustular folliculitis (I-EPF) is a rare disease characterized by pruritic vesicles and sterile pustules on the erythematous surface of the scalp and facial localization, usually seen in the neonatal period. It is essential to show the presence of dense eosinophils in the diagnosis of pustules. Histopathological examination of the hair follicles by eosinophils infiltration is determined. Read More

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Oral Langerhans Cell Histiocytosis in Two Pediatric Patients.

J Dent Child (Chic) 2021 Jan;88(1):66-71

Dr. Silva-Junior is an adjunct professor, all in the Department of Diagnosis and Therapeutics, School of Dentistry, State University of Rio de Janeiro, Rio de Janeiro, Brazil;, Email:

We report two cases of Langerhans cell histiocytosis (LCH) in the oral cavity of children. An 11-month-old girl was referred for evaluation of a nodular and sessile lesion in the alveolar ridge covering the primary left mandibular central incisor. The second patient was a seven-year-old boy with an ulcerated lesion in the gingiva covered by necrotic areas, extending to the sulcus of the vestibule. Read More

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January 2021

Adult-onset asthma and periocular xanthogranuloma - A rare infiltrative disease of the orbit and eyelid.

Am J Ophthalmol Case Rep 2021 Jun 21;22:101043. Epub 2021 Mar 21.

Veterans Affairs Boston Healthcare System, Department of Ophthalmology, 150 South Huntington Avenue, Boston, MA, 02130, USA.

Purpose: To present a case of adult onset asthma with periocular xanthogranuloma (AAPOX), and discuss existing literature on adult orbital xanthogranulomatous diseases (AOXGDs) and their treatment.

Observations: A 63 year old male presented with progressive bilateral eyelid swelling with overlying yellow plaques associated with asthma. CT scan showed periorbital swelling with enlargement of the superior and lateral rectus muscles bilaterally. Read More

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Multicentric reticulohistiocytosis masquerading as cutaneous connective tissue disease.

Dermatol Online J 2021 Mar 15;27(3). Epub 2021 Mar 15.

Department of Dermatology, HCA Healthcare/USF Morsani College of Medicine Largo, FL.

Multicentric reticulohistiocytosis (MRH) is a rare type of non-Langerhans cell histiocytosis characterized by coral-toned papules with predilection for dorsal surfaces in addition to severe arthropathy. It sometimes proves difficult to differentiate these joint and skin findings clinically from certain rheumatologic diseases, primarily dermatomyositis. Herein, we present an 82-year-old woman who presented with the clinical findings described above and was subsequently diagnosed with MRH after biopsy and review of relevant clinical history. Read More

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Localised renal Langerhans cell histiocytosis coexisting with unilateral renal clear cell carcinoma.

BMJ Case Rep 2021 Apr 16;14(4). Epub 2021 Apr 16.

Department of Urology, Carle Foundation Hospital, Urbana, Illinois, USA.

Langerhans cell histiocytosis (LCH) is an uncommon group of disorders, which can be either localised or systemic, characterised by abnormal proliferation of monocytes, macrophages and dendritic cells. These disorders represent an aberrant response of myeloid progenitor cells. Bones are the most commonly affected organ but there can be involvement of the skin, lungs, liver and spleen. Read More

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Rosai-Dorfman-Destombes (RDD) disease presenting as palindromic rheumatism.

BMC Med Imaging 2021 Apr 15;21(1):72. Epub 2021 Apr 15.

Department of Radiology, University of California, UCSD Medical Center, 200 W Arbor Drive, San Diego, CA, 92103-2686, USA.

Background: Rosai-Dorfman-Destombes (RDD) disease, is a rare proliferative and inflammatory disorder of non-Langerhans cell histiocytes.

Case Presentation: We report a 35-year-old woman, who originally presented with recurrent episodes of lower extremity joint/bone pain and chronic nasal stuffiness and congestion. Her worsening nasal congestion was due to an obstructing nasal cavity lesion which was subsequently biopsied. Read More

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A Case of Burnt-Out Langerhans Cell Histiocytosis Presenting as Postpartum Hypopituitarism.

AACE Clin Case Rep 2021 Jan-Feb;7(1):47-50. Epub 2020 Dec 28.

Department of Endocrinology, Townsville University Hospital, Douglas, Queensland, Australia.

Objective: To evaluate the case of a woman who presented with central hypogonadism and diabetes insipidus and further developed a persistent cough leading to an unexpected diagnosis of burnt-out Langerhans cell histiocytosis (LCH).

Methods: Clinical and laboratory endocrine evaluation, magnetic resonance imaging, high-resolution computed tomography, and open-lung biopsy results are discussed.

Results: A 28-year-old woman presented at 10 months postpartum with polydipsia, polyuria, and amenorrhea for 3 months. Read More

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December 2020

A Dose-Response Relationship to Radiotherapy for Cutaneous Lesions of Langerhans Cell Histiocytosis.

Case Rep Oncol Med 2021 24;2021:6680635. Epub 2021 Mar 24.

Department of Radiation Medicine, Roswell Park Comprehensive Cancer Center, USA.

Langerhans cell histiocytosis (LCH) is a rare disease, afflicting approximately 4.6 and 1-2 per 1 million children and adults, respectively. While LCH can involve numerous organ systems such as the lung or bone, it is uncommon for the disease to be limited to the skin. Read More

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Progressive nodular histiocytosis: an unusual disorder.

Dermatol Online J 2021 Feb 15;27(2). Epub 2021 Feb 15.

Institute of Dermatology and Dermatologic Surgery (INDERMA), Guatemala.

Progressive nodular histiocytosis (PNH) is a rare type of non-Langerhans cell histiocytosis of the xanthogranuloma group. Less than 20 cases have been reported. We report here a novel case of PNH with dermoscopic description and post-surgical outcome. Read More

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February 2021

Solitary Adult Rib Langerhans Cell Histiocytosis Evaluated by 18F-FDG PET/CT.

Clin Nucl Med 2021 Apr 1. Epub 2021 Apr 1.

From the Department of Nuclear Medicine, The Affiliated Hospital of Southwest Medical, University; Nuclear Medicine and Molecular Imaging Key Laboratory of Sichuan Province; and Academician (Expert) Workstation of Sichuan Province, Luzhou, Sichuan, People's Republic of China.

Abstract: Langerhans cell histiocytosis (LCH) is a rare proliferative histiocytic disorder. It mainly occurs in the pediatric population, whereas it is rarely reported in adults. Herein, we reported a case of a patient with isolated rib LCH, which showed elevated 18F-FDG uptake. Read More

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[Clinical characteristics of localized Langerhans cell histiocytosis in children].

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2021 Mar;35(3):242-245

Department of Otolaryngology Head and Neck Surgery,Kunming Children's Hospital,Kunming,650228,China.

To explore the clinical features of cephalic and facial limited langerhans cell histiocytosis (LCH) in children for improving its diagnosis and treatment. Clinical data of 8 children with cephalic and facial limited LCH were retrospectively analyzed, including the onset time of disease, lesion location, imaging data, clinical manifestations and treatment strategies. One case was preliminarily diagnosed as chronic inflammation with nasal back lesions, then conformed by repeated surgical pathology. Read More

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Double trouble for Langerhans cell histiocytosis.

Blood 2021 Apr;137(13):1705-1706

University of California, San Francisco.

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Eosinophilic granuloma of cranial location: report of a case and bibliographic review

Rev Fac Cien Med Univ Nac Cordoba 2021 03 12;78(1):48-51. Epub 2021 Mar 12.

Hospital Clinico Universitario Lozano Blesa. Zaragoza. España.

Introduction: Eosinophilic granuloma is an unusual benign disease that usually affects the pediatric population and young adults. It is the most benign of the diseases traditionally known as histiocytosis X that are now called Langerhans cell histiocytosis.

Clinical Case: Pediatric patient with a painful lump in the temporal region. Read More

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Spheno-orbital Lesions-A Major Review of Nonmeningioma Causes.

Ophthalmic Plast Reconstr Surg 2021 Mar 24. Epub 2021 Mar 24.

Orbital Plastic and Lacrimal Clinic, Royal Victorian Eye and Ear Hospital Centre for Eye Research Australia, University of Melbourne Department of Radiology, St Vincent's Hospital, Melbourne, Victoria, Australia Department of Surgery, Royal Melbourne Hospital, University of Melbourne, Parkville, Victoria, Australia.

Purpose: To review the imaging features of the broad range of nonmeningioma lesions of the greater wing of the sphenoid (GWS) bone and adjacent orbit to assist clinicians in differentiating these lesions from each other and from the most common lesion of the GWS and adjacent orbit, meningioma.

Methods: We reviewed 32 cases of spheno-orbital lesions involving the GWS from our own practice, as well as 109 published cases (total 141), with emphasis on available imaging features on computerized tomography (CT) and MRI. Features that might assist in differentiating meningioma from its mimics were analyzed for each lesion, including the presence of an osteoblastic or hyperostotic response, bone erosion or osteolysis, homogeneous hypo- or hyperintensity on T2-weighted MRI, leptomeningeal involvement, and the absence of a "dural tail" on contrast-enhanced MRI. Read More

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The Current Understanding on Langerhans' Cells and Its Role in Oral Lesions.

Contemp Clin Dent 2020 Jul-Sep;11(3):211-216. Epub 2020 Nov 26.

Department of Oral Pathology and Microbiology, JSS Dental College and Hospital, JSS Academy of Higher Education and Research, Mysore, Karnataka, India.

Objective: Description of Langerhans' cells (LCs) as an important antigen-presenting cells responsible for detecting the antigens, recruiting T-cells, and thereby initiating the immune response. An adequate response of the mucosal immune system is essential to protect the mucosa against pathological conditions. Hence, a detailed review was planned about this unique antigen-presenting cell. Read More

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November 2020