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    10439 results match your criteria Langerhans Cell Histiocytosis

    1 OF 209

    The outcome of eosinophilic granuloma involving unilateral atlantoaxial joint: A case report and literature review.
    Medicine (Baltimore) 2017 Jun;96(25):e7197
    aDepartment of Orthopedics, Union Hospital, Tongji Medical College of Huazhong University of Science and Technology, Wuhan, Hubei bDepartment of Ophthalmology, Shengjing Hospital of China Medical University, Shenyang, Liaoning cDepartment of Pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
    Rationale: Solitary eosinophilic granuloma (EG), the most benign, common form of Langerhans cell histiocytosis, has a self-limiting process and is associated with a good prognosis. Immobilization is recommended as the first treatment strategy for solitary EG, although the treatment protocols are still controversial. Radiotherapy and surgery are secondary treatment choices. Read More

    A rare case of oral multisystem Langerhans cell histiocytosis.
    J Clin Exp Dent 2017 Jun 1;9(6):e820-e824. Epub 2017 Jun 1.
    Oral Surgery Unit of George Eastman Hospital, Umberto I Teaching Hospital, Rome, Italy.
    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by high proliferation of Langerhans dendritic cells. LCH is a solitary or multifocal disease that primarily involves bone tissue and often affects children and young men. A 29 years-old Caucasian man was referred to the Oral Surgery Unit of George Eastman Hospital - Umberto I teaching hospital, with third degree mobility of teeth belonging to second, third and fourth quadrant. Read More

    Chronic Recurrent Multifocal Osteomyelitis: A Case Report with Atypical Presentation.
    J Orthop Case Rep 2017 Jan-Feb;7(1):75-78
    Department of Orthopaedics, Hospital Prof. Doutor Fernando Fonseca, Estrada IC19, 2720-276 Amadora, Portugal.
    Introduction: Chronic recurrent multifocal osteomyelitis (CRMO) is a rare autoinflammatory condition. The clinical picture consists of sterile osteomyelitis, typically with multiple-site lesions in the metaphysis of long bones and not uncommonly, symmetrical bone involvement. It is a poorly understood entity, whose prognosis, etiology and ideal treatment are still controversial. Read More

    Langerhans Cell Sarcoma: A Case Report Demonstrating Morphological and Immunophenotypical Variability within a Single Lesion.
    Case Rep Pathol 2017 24;2017:9842605. Epub 2017 May 24.
    Department of Cellular Pathology, Royal Devon and Exeter NHS Foundation Trust, Old Pathology Building, Church Lane, Exeter EX2 5DW, UK.
    Langerhans cells are antigen presenting dendritic cells and tumours arising from these are rare. The tumours arising from these dendritic cells are divided into two categories according to a WHO classification: Langerhans cell histiocytosis and Langerhans cell sarcoma. It is the degree of atypia and clinical aggressiveness that distinguishes the two subtypes. Read More

    Association between Ischemic Stroke and Erdheim-Chester Disease: A Case Report and Review of Literature.
    J Stroke Cerebrovasc Dis 2017 Jun 13. Epub 2017 Jun 13.
    Neurology Department, Meaux General Hospital, Meaux, France.
    Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis characterized by a xanthogranulomatous infiltration of tissues by spumous histiocytes. Neurological involvement is frequent, but ischemic strokes have been exceptionally described. We report the case of a 68-year-old woman who presented with an acute ischemic stroke associated with a multisystemic disorder including insipidus diabetes, infiltration of the aorta and the carotid arteries, perirenal infiltration, aortitis, and lytic bone lesions. Read More

    Cyclin D1 is Expressed in Neoplastic Cells of Langerhans Cell Histiocytosis but Not Reactive Langerhans Cell Proliferations.
    Am J Surg Pathol 2017 Jun 15. Epub 2017 Jun 15.
    Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA.
    Langerhans cell histiocytosis (LCH) is characterized by frequent activating mutations involving the mitogen-activated protein kinase (MAPK) pathway. Therefore, downstream markers of MAPK pathway activation such as cyclin D1 may be useful as novel diagnostic markers of neoplasia in LCH. The goal of this study was to investigate cyclin D1 expression in LCH and reactive Langerhans cell accumulations using immunohistochemistry on archival tissue. Read More

    A consensus review on malignancy-associated hemophagocytic lymphohistiocytosis in adults.
    Cancer 2017 Jun 16. Epub 2017 Jun 16.
    Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, Texas.
    Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation and dysregulation resulting in extreme and often life-threatening inflammation. HLH has been well recognized in pediatric populations, and most current diagnostic and therapeutic guidelines are based on pediatric HLH. Recently there has been recognition of HLH in adults, especially secondary to immune deregulation by an underlying rheumatologic, infectious, or malignant condition. Read More

    Erdheim-Chester Disease presenting with histiocytic colitis and cytokine storm.
    J Gastrointestin Liver Dis 2017 Jun;26(2):183-187
    Division of Gastroenterology, Department of Medicine Washington University School of Medicine, St. Louis, MO, USA.
    Background: Non-Langerhans histiocytosis is a group of inflammatory lymphoproliferative disorders originating from non-clonal expansion of hematopoietic stem cells into cytokine-secreting dendritic cells or macrophages. Erdheim-Chester Disease (ECD) is a rare type of non-Langerhans cell histiocytosis characterized by tissue inflammation and injury caused by macrophage infiltration and histologic findings of foamy histiocytes. Often ECD involves the skeleton, retroperitoneum and the orbits. Read More

    Metastatic pancreatic carcinoma masquerading as cystic lung disease: a rare presentation.
    Respirol Case Rep 2017 Sep 11;5(5):e00246. Epub 2017 Jun 11.
    Department of Respiratory MedicineSir Charles Gairdner HospitalPerthAustralia.
    This 52-year-old male ex-smoker presented with a six-month history of progressive breathlessness and weight loss. He deteriorated acutely, and was admitted with severe type 1 respiratory failure. Apart from diffuse coarse crackles on chest auscultation, physical examination was unremarkable. Read More

    A Case of Pulmonary Langerhans Cell Sarcoma Simultaneously Diagnosed with Cutaneous Langerhans Cell Histiocytosis Studied by Whole-Exome Sequencing.
    Acta Haematol 2017 Jun 15;138(1):24-30. Epub 2017 Jun 15.
    Department of Thoracic and Cardiovascular Surgery, Chungbuk National University College of Medicine, Cheongju, Republic of Korea.
    Langerhans cell histiocytosis (LCH) and Langerhans cell sarcoma (LCS) are clonal proliferations of Langerhans-type cells. Unlike in LCH, the pathophysiology and clinical course of LCS are unclear due to its rarity. Here, we report the case of a 73-year-old male patient who was diagnosed with cutaneous LCH and pulmonary LCS at the same time. Read More

    Congenital Syphilis of Bone: A Potential Mimicker of Childhood Histiocytoses.
    Am J Surg Pathol 2017 Jun 13. Epub 2017 Jun 13.
    *Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH †Pitts Radiology ‡Pediatric Associates, PA, Columbia, SC.
    Involvement of the skeletal system by congenital syphilis is well documented in the literature, chiefly in the form of radiologic studies, including periostitis, osteitis, and osteochondritis. Because congenital syphilis is generally recognized clinically, tissue biopsy is virtually never performed. Therefore, the histopathologic findings are less well documented and mostly exist in the older literature. Read More

    Erdheim-Chester Disease Presenting with Secondary Hypertension as a Result of Bilateral, Proximal Renal Artery Stenosis: A Case Report.
    Case Rep Nephrol Dial 2017 May-Aug;7(2):55-62. Epub 2017 May 23.
    University of California, Los Angeles, California, USA.
    Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis presenting most commonly with bone and central nervous system symptoms, including but not limited to bone pain and diabetes insipidus. We present a known case of ECD, which was referred for secondary hypertension workup and diagnosed with severe, proximal, bilateral renal artery stenosis. Read More

    [Clinical features and prognosis of Langerhans cell histiocytosis in children: an analysis of 34 cases].
    Zhongguo Dang Dai Er Ke Za Zhi 2017 Jun;19(6):627-631
    Department of Pediatric Hematology and Oncology, Chengdu Women and Children's Central Hospital, Chengdu 610091, China.
    Objective: To investigate the clinical features and prognosis of children with Langerhans cell histiocytosis (LCH).

    Methods: A retrospective analysis was performed for the clinical data of 34 children with newly diagnosed LCH.

    Results: The 34 children had a median age of 14. Read More

    PET/MR in the Assessment of Pediatric Histiocytoses: A Comparison to PET/CT.
    Clin Nucl Med 2017 Jun 9. Epub 2017 Jun 9.
    From the *Department of Pediatric Radiology, Texas Children's Hospital, Houston; and †Section of Hematology/Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, TX.
    Purpose: The aim of this study was to analyze the feasibility and diagnostic performance of F-FDG PET/MR compared with F-FDG PET/CT in a cohort of pediatric histiocytosis patients with regard to image quality, lesion detection, and FDG quantification.

    Methods: Children with a diagnosis of Langerhans cell histiocytosis or Rosai-Dorfman disease were prospectively recruited. Seventeen PET/CT and PET/MR examinations were performed on 9 patients (mean age, 6. Read More

    Langerhans Cell Histiocytosis (LCH) of the Tonsil in Adult Patient: An Uncommon Disease at an Uncommon Site.
    Indian J Hematol Blood Transfus 2017 Jun 2;33(2):276-277. Epub 2016 Nov 2.
    Department of Medical Oncology, Basavatarakam Indo-American Cancer Hospital and Research Institute, Hyderabad, India.
    Langerhans cell histiocytosis (LCH) is a rare disease characterized by clonal neoplastic proliferation of normal antigen presenting cell (APC), the Langerhans cell. Most cases occur in childhood and the disease is rare in adults. LCH can involve solitary organ or can present as a multi-system disease in children. Read More

    Spontaneous pneumothorax in diffuse cystic lung diseases.
    Curr Opin Pulm Med 2017 Jul;23(4):323-333
    aDepartment of Internal Medicine, University of Cincinnati, Cincinnati, Ohio, USAbPleural Medicine Unit, Institute for Respiratory HealthcDepartment of Respiratory Medicine, Sir Charles Gairdner HospitaldSchool of Medicine and Pharmacology, University of Western Australia, Perth, Western Australia, AustraliaeDivision of Pulmonary, Critical Care and Sleep Medicine, University of CincinnatifMedical Service, Veterans Affairs Medical Center, Cincinnati, Ohio, USA.
    Purpose Of Review: Diffuse cystic lung diseases (DCLDs) are a heterogeneous group of disorders with varying pathophysiologic mechanisms that are characterized by the presence of air-filled lung cysts. These cysts are prone to rupture, leading to the development of recurrent spontaneous pneumothoraces. In this article, we review the epidemiology, clinical features, and management DCLD-associated spontaneous pneumothorax, with a focus on lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, and pulmonary Langerhans cell histiocytosis. Read More

    Looks Can Be Deceiving: A Case Report on Multicentric Reticulohistiocytosis Successfully Treated with Rituximab.
    Cureus 2017 May 3;9(5):e1220. Epub 2017 May 3.
    Rheumatology, Orlando VA Medical Center.
    Multicentric reticulohistiocytosis (MRH) is an idiopathic multisystemic inflammatory disease characterized by symmetric erosive polyarthritis and typical papulonodular skin lesions. MRH can be associated with autoimmune diseases, malignancy, mycobacterial infections, and hyperlipidemia, and it is important to consider appropriate screening in this population. There is no specific diagnostic laboratory test for MRH. Read More

    MRI features of growth hormone deficiency in children with short stature caused by pituitary lesions.
    Exp Ther Med 2017 Jun 24;13(6):3474-3478. Epub 2017 Apr 24.
    Department of Radiology, Xuzhou Children's Hospital, Xuzhou, Jiangsu 221002, P.R. China.
    We verified the advantages of using magnetic resonance imaging (MRI) for improving the diagnostic quality of growth hormone deficiency (GHD) in children with short stature caused by pituitary lesions. Clinical data obtained from 577 GHD patients with short stature caused by pituitary lesions were retrospectively analyzed. There were 354 cases (61. Read More

    Dendritic Cell Trafficking and Function in Rare Lung Diseases.
    Am J Respir Cell Mol Biol 2017 Jun 6. Epub 2017 Jun 6.
    University of Cincinnati, Cincinnati, Ohio, United States ;
    Dendritic cells (DCs) are highly specialized immune cells that capture antigens and then migrate to lymphoid tissue and present antigen to T cells. This critical function of DCs is well defined and recent studies further demonstrate that DCs are also key regulators of several innate immune responses. Studies focused on the roles of DCs in the pathogenesis of common lung diseases such as asthma, infection and cancer have traditionally driven our mechanistic understanding of pulmonary DC biology. Read More

    [A lumbar vertebral eosinophilic granuloma with spinal instability in an adolescent].
    Arch Pediatr 2017 Jul 3;24(7):618-621. Epub 2017 Jun 3.
    Service de chirurgie pédiatrique, CHU Réunion, site Saint-Pierre, avenue Francois-Miterrand, 97410 Saint-Pierre, Réunion.
    This is a case report of a rare isolated eosinophilic granuloma of vertebra L3, in a 15-year-old adolescent. Vertebral instability, due to aggravation of the osteolysis, occurred without neurological symptoms despite orthopedic treatment. Surgery was necessary to correct and stabilize the spinal deformation. Read More

    Benign Bone Conditions That May Be FDG-avid and Mimic Malignancy.
    Semin Nucl Med 2017 Jul 12;47(4):322-351. Epub 2017 Apr 12.
    Department of Radiology and Nuclear Medicine, Deventer Ziekenhuis, Deventer, The Netherlands.
    Positron emission tomography with the radiotracer (18)F-fluoro-2-deoxy-d-glucose (FDG) plays an important role in the evaluation of bone pathology. However, FDG is not a cancer-specific agent, and knowledge of the differential diagnosis of benign FDG-avid bone alterations that may resemble malignancy is important for correct patient management, including the avoidance of unnecessary additional invasive tests such as bone biopsy. This review summarizes and illustrates the spectrum of benign bone conditions that may be FDG-avid and mimic malignancy, including osteomyelitis, bone lesions due to benign systemic diseases (Brown tumor, Erdheim-Chester disease, Gaucher disease, gout and other types of arthritis, Langerhans cell histiocytosis, and sarcoidosis), benign primary bone lesions (bone cysts, chondroblastoma, chondromyxoid fibroma, desmoplastic fibroma, enchondroma, giant cell tumor and granuloma, hemangioma, nonossifying fibroma, and osteoid osteoma and osteoblastoma), and a group of miscellaneous benign bone conditions (post bone marrow biopsy or harvest status, bone marrow hyperplasia, fibrous dysplasia, fractures, osteonecrosis, Paget disease of bone, particle disease, and Schmorl nodes). Read More

    Oral Juvenile Xanthogranuloma: Report of Two Cases.
    Pediatr Dent 2017 May;39(3):238-240
    Associate professor of Oral Pathology, in the School of Dentistry, Rio de Janeiro State University, Rio de Janeiro, Brazil;, Email:
    Juvenile xanthogranuloma is a rare non-Langerhans cell histiocytosis that usually occurs in the skin of children. Extracutaneous involvement is rare, and few cases affecting the oral cavity have been reported. The purpose of the present study was to report two cases of oral juvenile xanthogranuloma affecting the lower lip of a 14-year-old girl and the soft palate of a second 14-month-old girl, both present as yellowish papules. Read More

    Inflammatory serum cytokines and chemokines increase associated with the disease extent in pediatric Langerhans cell histiocytosis.
    Cytokine 2017 Jun 2;97:73-79. Epub 2017 Jun 2.
    Division of Laboratory Medicine, Uji-Tokushukai Medical Center, Uji, Japan.
    Objective: Langerhans cell histiocytosis (LCH) is characterized by immature dendritic cell proliferation, infiltration of LCH lesions by various inflammatory cells, and a lesional cytokine storm. It is classified into three groups on the basis of disease extent, namely, multisystem with risk-organ involvement (MS+), multisystem without risk-organ involvement (MS-), and single-system (SS) disease. We comprehensively analyzed whether serum levels of cytokines/chemokines reflect the disease extent. Read More

    Functional evidence for derivation of systemic histiocytic neoplasms from hematopoietic stem/progenitor cells.
    Blood 2017 May 31. Epub 2017 May 31.
    EA4340, Versailles University, Paris-Saclay university, Boulogne, France;
    Langerhans Cell Histiocytosis (LCH) and the non-LCH neoplasm Erdheim-Chester Disease (ECD) are heterogeneous neoplastic disorders marked by infiltration of pathologic macrophage-, dendritic cell-, or monocyte-derived cells in tissues driven by recurrent mutations activating MAP kinase signaling. Although recent data indicate that at least a proportion of LCH and ECD patients have detectable activating kinase mutations in circulating hematopoietic cells and bone marrow-based hematopoietic progenitors, functional evidence of the cell-of-origin of histiocytosis from actual patient materials has long been elusive. Here we provide evidence for mutations in MAP kinase signaling intermediates in CD34(+) cells from patients with ECD and LCH/ECD, including detection of shared origin of LCH and acute myelomonocytic leukemia driven by TET2-mutant CD34(+) cell progenitors in one patient. Read More

    Langerhans' Cell Histiocytosis Mimicking a Pott Puffy Tumor.
    J Pediatr Hematol Oncol 2017 May 29. Epub 2017 May 29.
    *NESMOS Department, Sant'Andrea Hospital, University La Sapienza †Imaging Department, Neuroradiology Unit Departments of ‡Hematology and Oncology §Neurosciences and Neurorehabilitation, Neurosurgery Unit ∥Pathology, Bambino Gesù Children's Hospital, Rome, Italy.
    Langherans' cell histiocytosis (LCH) is a rare disease mostly affecting children in the first decade of life. As clinical presentation is extremely heterogenous, a prompt diagnosis may be challenging, sometimes leading to a diagnostic delay, especially when the disease involves a single site. Herein, we report a case of a child with an unusual presentation of (LCH) mimicking a Pott puffy tumor with extracranial and epidural abscesses, surgically treated. Read More

    A rare 'incidentaloma' found on low-dose CT screening for lung cancer: 'scanner beware'.
    Postgrad Med 2017 Jun 5:1-4. Epub 2017 Jun 5.
    a VA Connecticut Healthcare System , West Haven , CT , USA.
    Screening for lung cancer with low-dose computed tomography (LDCT) has been shown to reduce mortality and has been recommended by the U.S. Preventive Services Task Force for adults 55 to 80 years of age with a 30 pack-year smoking history who are either current smokers or those that quit within 15 years. Read More

    Cerebellar Disease Mimicking Cerebrotendinous Xanthomatosis: Langerhans Cell Histiocytosis.
    Pediatr Neurol 2017 Apr 18. Epub 2017 Apr 18.
    Department of Neurology, Canisius Wilhelmina Hospital, Nijmegen, The Netherlands.
    Background: This report highlights the differential diagnosis of predominant cerebellar white matter abnormalities with dentate nuclei involvement.

    Patient Description: We describe two individuals with Langerhans cell histiocytosis in whom the diagnosis of cerebrotendinous xanthomatosis was initially considered. The clinical picture consisted of a progressive cerebellar syndrome with typical magnetic resonance imaging abnormalities. Read More

    The clinical spectrum of Erdheim-Chester disease: an observational cohort study.
    Blood Adv 2017 Feb;1(6):357-366
    Section on Human Biochemical Genetics, Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health. 10 Center Dr, Bldg 10, Room 3-2551, Bethesda, Maryland, USA, 20892. Telephone: 301-594-2952.
    Erdheim-Chester Disease (ECD) is a rare, potentially fatal, multi-organ myeloid neoplasm occurring mainly in adults. The diagnosis is established by clinical, radiologic, and histologic findings; ECD tumors contain foamy macrophages that are CD68+, CD163+, CD1a-, and frequently S100-. The purpose of this report is to describe the clinical and molecular variability of ECD. Read More

    A case of congenital Langerhans cell histiocytosis with disseminated skin and pulmonary involvement masquerading as multiple infantile hemangiomas.
    Pediatr Neonatol 2017 May 9. Epub 2017 May 9.
    Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University, College of Medicine, Taiwan. Electronic address:

    Erdheim-Chester Disease: Comprehensive Review of Molecular Profiling and Therapeutic Advances.
    Anticancer Res 2017 06;37(6):2777-2783
    Division of Hematology and Oncology, Department of Medicine, George Washington University Medical Center, Washington, DC, U.S.A.
    The revised 2016 World Health Organization classification introduced Erdheim-Chester disease (ECD) as a provisional entity within the histiocytic and dendritic cell neoplasms separate from the juvenile xanthogranuloma family based on distinct molecular features. However, evolving knowledge regarding the molecular and genetic aberrations in addition to common clinical features of ECD support the classification of ECD together with Langerhans cell histiocytosis (LCH). Accordingly, ECD can be thought of as an inflammatory myeloid clonal disorder based on the detection of various activating mutations along the mitogen activated protein kinase-extracellular signal regulated kinase (MAPK-ERK) pathway with most notable variant being a valine to a glutamic acid substitution at amino acid 600 in the B-rapidly accelerated fibrosarcoma protein (BRAFV600E). Read More

    KRAS(G12D) expression in lung-resident myeloid cells promotes pulmonary LCH-like neoplasm sensitive to statin treatment.
    Blood 2017 May 26. Epub 2017 May 26.
    Department of Cancer Studies, University of Leicester, United Kingdom.
    Langerhans cell histiocytosis (LCH) is a rare histiocytic neoplasm associated with somatic mutations in the genes involved in the RAF/MEK/ERK signaling pathway. Recently, oncogenic mutations in NRAS/KRAS, upstream regulators of the RAF/MEK/ERK pathway, have been reported in pulmonary, but not in non-pulmonary, LCH cases, suggesting organ-specific contribution of oncogenic RAS to LCH pathogenesis. Using a mouse model expressing KRAS(G12D) in the lung by nasal delivery of adenoviral Cre, here we show that KRAS(G12D) expression in lung-resident myeloid cells induces pulmonary LCH-like neoplasms comprised of pathogenic CD11c(high)F4/80+CD207+ cells. Read More

    Diffuse cystic lung diseases: differential diagnosis.
    J Bras Pneumol 2017 Mar-Apr;43(2):140-149
    . Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre (RS) Brasil.
    Diffuse cystic lung diseases are characterized by cysts in more than one lung lobe, the cysts originating from various mechanisms, including the expansion of the distal airspaces due to airway obstruction, necrosis of the airway walls, and parenchymal destruction. The progression of these diseases is variable. One essential tool in the evaluation of these diseases is HRCT, because it improves the characterization of pulmonary cysts (including their distribution, size, and length) and the evaluation of the regularity of the cyst wall, as well as the identification of associated pulmonary and extrapulmonary lesions. Read More

    (18-)F-FDG PET/CT in Localizing Additional CNS Lesion in a Case of Langerhans Cell Histiocytosis: Determining Accurate Extent of the Disease.
    Indian J Nucl Med 2017 Apr-Jun;32(2):162-163
    Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India.
    Central nervous system involvement is a rare manifestation of Langerhans cell histiocytosis (LCH), with bone and skin lesions being more frequent. MR remains the investigation of choice for localizing brain lesions. However, due to poor sensitivity of MRI in detecting osseous and pulmonary lesions, it is not used routinely in staging purposes until and unless indicated. Read More

    Vinblastine chemotherapy in adult patients with langerhans cell histiocytosis: a multicenter retrospective study.
    Orphanet J Rare Dis 2017 May 22;12(1):95. Epub 2017 May 22.
    Université Paris Diderot, Sorbonne Paris Cité, Inserm UMR-1153 (CRESS), Biostatistics and Clinical Epidemiology research team (ECSTRA), Paris, France.
    Background: Vinblastine is the standard treatment for children with Langerhans cell histiocytosis (LCH). Whether this treatment could be extended to adults with LCH is questionable. This retrospective multicenter study included 35 adult patients (median age 33 years; 23 men; 80% with multisystem LCH) who were treated with vinblastine + steroids as a first-line chemotherapy and followed for a median time of 83 months. Read More

    Low periostin levels in adult patients with Langerhans cell histiocytosis are independently associated with the disease activity.
    Metabolism 2017 Jun 4;71:198-201. Epub 2017 Apr 4.
    Department of Endocrinology and Diabetes, 251 Hellenic Air Force & VA General Hospital, Athens, Greece. Electronic address:
    Purpose: Langerhans cell histiocytosis (LCH) is a rare proliferative disease of cells of the CD1a+/CD207+ myeloid dendritic cell lineage that may infiltrate one or more organs or systems at all ages. We aimed to evaluate periostin and sclerostin serum levels in adult patients with LCH.

    Procedures: This was a cross-sectional study comparing 38 adult patients with LCH with 38 age- and sex-matched healthy controls. Read More

    [Indeterminate cell histiocytosis - disappearance of skin infiltration following electron beam therapy and an application of 2-chlorodeoxyadenosine: case report].
    Vnitr Lek 2017 ;63(4):284-288
    Indeterminate cell histiocytosis is a rare disease belonging to the group of malignant histiocytic diseases. The disease predominantly affects the skin. The disease appeared in the described patient at the age of 80 years. Read More

    Long-term clinical outcome of spinal Langerhans cell histiocytosis in children.
    Int J Hematol 2017 May 17. Epub 2017 May 17.
    Division of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea.
    Spinal involvement of Langerhans cell histiocytosis (LCH) affects morbidity, but outcomes are not well understood. We analyzed long-term outcomes following uniform treatment at a single institution. Clinical characteristics and outcomes of spinal LCH patients were retrospectively analyzed. Read More

    [Langerhans cell histiocytosis: Skin diseases and visceral lesions].
    Ter Arkh 2017 ;89(4):115-120
    Acad. E.A. Vagner Perm State Medical Academy, Ministry of Health of Russia, Perm, Russia.
    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. Read More

    Hematopoietic origin of Langerhans cell histiocytosis and Erdheim Chester disease in adults.
    Blood 2017 May 16. Epub 2017 May 16.
    Institute of Cellular Medicine, Newcastle University, United Kingdom;
    Langerhans cell histiocytosis (LCH) and Erdheim Chester Disease (ECD) are rare histiocytic disorders induced by somatic mutation of MAP kinase pathway genes. BRAF(V600E) mutation is the most common mutation in both conditions and also occurs in the hematopoietic neoplasm hairy cell leukemia (HCL). It is not known if adult LCH or ECD arise from hematopoietic stem cells (HSC) nor which potential blood borne precursors lead to the formation of histiocytic lesions. Read More

    BRAF V600E Mutation in Pediatric Intracranial and Cranial Juvenile Xanthogranuloma.
    Hum Pathol 2017 May 10. Epub 2017 May 10.
    Department of Pathology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand; Chulolongkorn GenePRO Center, Research Affairs, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand. Electronic address:
    Juvenile xanthogranuloma (JXG) is a cutaneous form of non-Langerhans cell histiocytosis (LCH), primarily affecting children. The lesion is presumed to originate from either macrophages or dermal dendritic cells. JXG can rarely present as an isolated intracranial lesion and, in contrast to the dismal outcome of patients with systemic disease, cranial JXG has been shown to carry a more favorable prognosis. Read More

    The clinical spectrum of xanthomatous lesions of the eyelids.
    Int J Dermatol 2017 May 13. Epub 2017 May 13.
    Pathology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.
    Yellowish papules, nodules, or plaques, namely "xanthomatous" lesions, may be seen on the eyelids in the course of various disorders. The prototype is "xanthelasma palpebrarum" (XP) that is localized only to the eyelids and may be associated with hyperlipidemia. On the other hand, different types of normolipemic disorders may also cause xanthomatous eyelid lesions. Read More

    Langerhans cell histiocytosis (eosinophilic granuloma) of the skull mimicking nummular headache. Report of two cases.
    Cephalalgia 2017 Jan 1:333102417708772. Epub 2017 Jan 1.
    1 Department of Neurology & Neurosurgery, Clinical Hospital of University of Chile, Santiago de Chile, Chile.
    Background Nummular headache is a rare, recently described topographic headache defined by the circumscribed coin-shaped area of pain. It is classified as a primary headache. There is debate about whether it is due to a peripheral or central disturbance, and its relationship to migraine. Read More

    Clinical Analysis of 122 Cases of Langerhans Cell Histiocytosis.
    Zhongguo Yi Xue Ke Xue Yuan Xue Bao 2017 Apr;39(2):206-210
    Department of Dermatology,PUMC Hospital,CAMS and PUMC,Beijing 100730,China.
    Objective To investigate the features of Langerhans cell histiocytosis (LCH). Method Skin lesions,systemic involvement,imaging characteristics,laboratory tests,immunophenotying,treatment response,and survival of 122 LCH patients treated at our center from February 1983 to August 2013 were retrospectively analyzed. Results LCH was associated with diverse skin lesions. Read More

    Langerhans cell histiocytosis of the maxillae in a child treated only with chemotherapy: a case report.
    J Med Case Rep 2017 May 9;11(1):130. Epub 2017 May 9.
    Department of Clinical and Experimental Medicine, University of Foggia, Foggia, Italy.
    Background: Langerhans cell histiocytosis is a sporadic disease caused by an uncontrolled pathogenic clonal proliferation of dendritic cells that have Langerhans cell characteristics. New treatment protocols provided by the HISTSOC-LCH-III (NCT00276757) trial show an improvement in the survival of children with langerhans cell histiocytosis.

    Case Presentation: We report a case of Langerhans cell histiocytosis, which presented as an osteolytic lesion of the left pre-maxillae enclosing the deciduous incisor and canine in a 7-month-old white Italian boy. Read More

    A rare and unusual case report of Langerhans cell histiocytosis.
    J Oral Maxillofac Pathol 2017 Jan-Apr;21(1):140-144
    Department of Pedodontics and Preventive Dentistry, AME Dental College and Hospital, Raichur, Karnataka, India.
    Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, is an uncommon hematological disorder affecting infants and young children. It is the condition characterized by uncontrolled stimulation and proliferation of normal antigen presenting cells, Langerhans cells. Because of its relatively low incidence, limited data are available regarding the epidemiology of LCH, with estimation of 2-5 cases per million inhabitants per year. Read More

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