11,249 results match your criteria Langerhans Cell Histiocytosis


Erdheim-Chester Disease: An Unusual Etiology of Recurrent Massive Pericardial Effusion.

Can J Cardiol 2020 May 8. Epub 2020 May 8.

Department of Cardiology. Electronic address:

Erdheim-Chester disease (ECD), a rare non-Langerhans cell histiocytosis, involves multiple systems and may display pericardial effusion. Herein, we report a 60-year-old man who presented with refractory pericardial effusion of "unknown" etiology. As in this patient, ECD should be considered when rapidly recurrent pericardial effusion is accompanied by a pseudotumoral lesion of the right atrium and other systemic manifestations. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.cjca.2020.04.040DOI Listing

Prevalence, Epidemiology, Etiology, and Sensitivity of Invasive Bacterial Infections in Pediatric Patients Undergoing Oncological Treatment: A Multicenter Nationwide Study.

Microb Drug Resist 2020 May 20. Epub 2020 May 20.

Department of Pediatric Hematology and Oncology, Collegium Medicum, Nicolaus Copernicus University Torun, Bydgoszcz, Poland.

Infectious complications (IC) caused by bacterial strains often impede anticancer therapy. The study aimed to retrospectively analyze bacterial IC that could help predict the risk and optimize the empirical treatment for bacterial infections in pediatric cancer patients. Over a 72-month period, all-in 5,599 children with cancer: 2,441 patients with hematological malignancy (HM including acute leukemias, Hodgkin and non-Hodgkin lymphomas [NHLs], and Langerhans cell histiocytosis) and 3,158 with solid tumors (STs including central nervous system tumors, neuroblastoma, Wilms' tumor, soft tissue sarcoma, germ cell tumors, Ewing sarcoma, osteosarcoma, hepatoblastoma, and others) were enrolled into the study. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1089/mdr.2019.0393DOI Listing

The combination of methotrexate and cytosine arabinoside in newly diagnosed adult Langerhans cell histiocytosis: a prospective phase II interventional clinical trial.

BMC Cancer 2020 May 18;20(1):433. Epub 2020 May 18.

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.1 Shuaifuyuan, Beijing, People's Republic of China.

Background: Langerhans Cell Histiocytosis (LCH) is a rare disease puzzling both children and adults, however outcome of adult patients is unfavorable. This prospective interventional trial aims to test the efficacy and safety of the combination of methotrexate and cytosine arabinoside in adult LCH patients.

Method: A total of 36 patients enrolled diagnosed with LCH and treated in our center from 1st Jan, 2014 to 30th Jun, 2016. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12885-020-06872-8DOI Listing

Bone marrow involvement in pediatric malignancies: a comparison study of Positron emission tomographycomputed tomography and bone marrow biopsy.

Turk J Pediatr 2020 ;62(2):182-190

Departments of Pediatrics, Mersin University Faculty of Medicine, Mersin, Turkey.

Background And Objectives: The comparison of Positron emission tomography- computed tomography (PETCT) and bone marrow biopsy (BMB) modalities in detecting bone marrow disease is an up to date research topic. In this study, we aimed to compare the results of PET-CT and BMB procedures in detecting bone marrow involvement in pediatric malignancies.

Method: At the time of diagnosis, PET-CT imaging and BMB performed patients` data were evaluated, retrospectively. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.24953/turkjped.2020.02.003DOI Listing
January 2020

Agminated flexural melanocytic nevus associated with Langerhans cell histiocytosis.

J Dermatol 2020 May 12. Epub 2020 May 12.

Department of Dermatology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Toyama, Japan.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/1346-8138.15391DOI Listing

[Adult Langerhans cell histiocytosis treated by cladribine: a case report].

Zhonghua Xue Ye Xue Za Zhi 2019 Jul;40(7):611

Department of Hematology, Lanzhou General Hospital, Lanzhou Command, Lanzhou, 730050, China.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2019.07.016DOI Listing

Methotrexate and cytarabine for adult patients with newly diagnosed Langerhans cell histiocytosis: A single arm, single center, prospective phase 2 study.

Am J Hematol 2020 May 12. Epub 2020 May 12.

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/ajh.25864DOI Listing

Long-term outcomes of children with extracutaneous juvenile xanthogranulomas in Japan.

Pediatr Blood Cancer 2020 May 8:e28381. Epub 2020 May 8.

Department of Pediatrics, Ehime University Graduate School of Medicine, Toon, Japan.

Background: Juvenile xanthogranuloma (JXG) is the most common non-Langerhans cell histiocytosis in children. The mortality and morbidity of JXG with extracutaneous lesions remain unclear.

Methods: Data of patients aged < 18 years who were diagnosed with JXG between 2001 and 2010 were retrospectively collected through a nationwide survey. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/pbc.28381DOI Listing

Smoking-associated interstitial lung disease: update and review.

Expert Rev Respir Med 2020 May 22:1-10. Epub 2020 May 22.

Department of Pulmonary and Critical Care Medicine, Renown Health , Reno, USA.

Introduction: Smoking-associated interstitial lung disease manifests as several heterogeneous disorders involving the airways, pleura, and lung parenchyma with various radiological patterns. The clinical history, radiologic, and pathologic findings are important to distinguish these more uncommon diseases. A multidisciplinary approach is recommended for diagnosis and to manage these conditions appropriately. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/17476348.2020.1766971DOI Listing

Frequent KIT mutations in skin lesions of patients with BRAF wild-type Langerhans cell histiocytosis.

Virchows Arch 2020 May 5. Epub 2020 May 5.

2nd Department of Pathology, Semmelweis University, Budapest, Hungary.

Langerhans cell histiocytosis (LCH) is characterized by mutations of the RAS-RAF-MAPK signaling pathway. We analyzed MAP2K1, NRAS and KIT mutation incidence in skin lesions of BRAF wild-type (wt) LCH patients. We evaluated the occurrence of MAP2K1, NRAS and KIT mutations in seven LCH and one indeterminate cell histiocytosis (ICH) patients. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00428-020-02820-wDOI Listing

Bone Marrow Langerhans Cell Histiocytosis in Association with Kasabach-Merritt Syndrome: The Difficulty of a Differential Diagnosis.

Case Rep Oncol 2020 Jan-Apr;13(1):408-413. Epub 2020 Apr 14.

Department of Internal Medicine II, Professor Doutor Fernando Fonseca Hospital, Amadora, Portugal.

Langerhans cell histiocytosis is a rare haematological disorder with variable clinical findings and a high mortality rate. On the other hand, Kasabach-Merritt syndrome is of rare onset at adult age, requiring the simultaneous presentation of vascular lesion, thrombocytopenia, and consumptive coagulopathy. We present the first reported case of both diseases in a single patient and highlight the difficulties of diagnostic. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1159/000506539DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7184841PMC

[Langerhans cell histiocytosis in the pelvis].

An Pediatr (Barc) 2020 Apr 27. Epub 2020 Apr 27.

Unidad de Gestión Clínica de Medicina Nuclear, Hospital Universitario Virgen Macarena, Sevilla, España.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.anpedi.2019.05.003DOI Listing

Pediatric dermatology emergencies.

Cutis 2020 03;105(3):132-136

Division of Dermatology, Loyola University Medical Center, Maywood, Illinois, USA.

Many pediatric skin conditions can be safely monitored with minimal intervention, but certain skin conditions are emergent and require immediate attention and proper assessment of the neonate, infant, or child. We review the following pediatric dermatology emergencies so that clinicians can detect and accurately diagnose these conditions to avoid delayed treatment and considerable morbidity and mortality if missed: staphylococcal scalded skin syndrome (SSSS), impetigo, eczema herpeticum (EH), Langerhans cell histiocytosis (LCH), infantile hemangioma (IH), and IgA vasculitis. Read More

View Article

Download full-text PDF

Source

Rosai-Dorfman Disease-Utility of 18F-FDG PET/CT for Initial Evaluation and Follow-up.

Clin Nucl Med 2020 Jun;45(6):e260-e266

Departments of Radiology.

Background: The role of imaging in the management of Rosai-Dorfman disease (RDD), a rare non-Langerhans cell histiocytosis, is not clearly defined. We present an analysis of FDG PET/CT findings obtained for initial disease characterization, follow-up evaluation, and treatment planning for this disease.

Methods: From an institutional pathology database (2001-2018), we identified RDD patients who underwent FDG PET/CT scans either as part of clinical care or when done as part of clinical trials. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/RLU.0000000000003014DOI Listing

Dermal fillers may induce late-onset adverse skin reactions in patients under braf-inhibitors.

J Eur Acad Dermatol Venereol 2020 Apr 28. Epub 2020 Apr 28.

Dermatology Department, Hospital Clínic, University of Barcelona, Barcelona, Spain.

Histiocytoses are disorders characterised by inflammation and the accumulation of cells derived from the monocyte and macrophage lineages, which results in tissue damage. Considerable advances in the understanding of their genetics have led to an increased clinical recognition of these conditions, and fuelled futher insights into their pathogenesis(1). Almost 70% of patients affected by Erdheim-Chester disease (ECD), a rare non-Langerhans cell histiocytosis, have the somatic V600E mutation of the BRAF gene(2). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/jdv.16550DOI Listing

Langerhans cell sarcoma of the vulva: Case report and review of the literature.

Gynecol Oncol Rep 2020 May 15;32:100570. Epub 2020 Apr 15.

University of Florida, Division of Gynecologic Oncology, 1600 SW Archer RD, PO Box 100294, Gainesville, FL 32610, United States.

Langerhans cell sarcoma (LCS) is a rare, malignant neoplastic disorder of Langerhans cells thought to arise from antecedent Langerhans cell histiocytosis (LCH) or . There are less than 70 cases of LCS reported in the medical literature, with this case being the first report of primary vulvar LCS. We present the case of a 73-year-old female with a painful 2 cm ulcerated lesion of the right labia majora. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.gore.2020.100570DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7182718PMC

Bilateral pulmonary metastases of papillary thyroid carcinoma in a 12-year-old child-A case report and review of the literature.

Radiol Case Rep 2020 Jun 21;15(6):799-802. Epub 2020 Apr 21.

Ameripath Lubbock Pathology, Ameripath, Lubbock, TX 79407, USA.

Metastatic disease of the lung has been extensively documented in the adult patient population. The most common primary sources for pulmonary metastases include breast, colon gastrointestinal including pancreas and urinary bladder. Malignant lung tumors in pediatric population is extremely rare. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.radcr.2020.03.022DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7178319PMC

Erdheim-Chester disease: A challenging diagnosis for an effective therapy.

Clin Neurol Neurosurg 2020 Apr 15;194:105841. Epub 2020 Apr 15.

Division of Human Anatomy and I Division of Neurology and Neurophysiopathology, University of Campania "Luigi Vanvitelli", Naples, Italy. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.clineuro.2020.105841DOI Listing

Langerhan's cell Histiocytosis of Sphenoid Sinus causing Vision Loss: A Case Report.

JNMA J Nepal Med Assoc 2019 Jul-Aug;57(218):281-284

Department of ENT, Christian Medical College, Vellore, India.

Langerhan's cell histiocytosis is an uncontrolled proliferation of dendritic cells. The involvement of skull base is rare. Variable clinical presentation and multi organ involvement often warrant a multidisciplinary approach for a successful diagnosis. Read More

View Article

Download full-text PDF

Source

[Mandibular Langerhans cell histiocytosis: a case report].

Hua Xi Kou Qiang Yi Xue Za Zhi 2020 Apr;38(2):224-227

State Key Laboratory of Oral Diseases & National Clinical Research Center for Oral Diseases & Dept. of Pathology, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China.

Langerhans cell histiocytosis is commonly found in cranial bones and rarely found in the mandible. This article presents a case of mandibular Langerhans cell histiocytosis and discusses its pathogeny, clinical features, diagnosis, and treatment. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.7518/hxkq.2020.02.019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7184281PMC

Cutaneous manifestations of Langerhans cell histiocytosis in children: a retrospective cohort study of 43 patients.

J Eur Acad Dermatol Venereol 2020 Apr 20. Epub 2020 Apr 20.

Department of Dermatology & Dermatological Surgery, Medical University of South Carolina, Charleston, SC, USA.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/jdv.16509DOI Listing

Serum KL-6 levels in pulmonary Langerhans' cell histiocytosis.

Eur J Clin Invest 2020 Apr 20:e13242. Epub 2020 Apr 20.

Respiratory Diseases and Lung Transplantation, Department of Medical and Surgical Sciences & Neurosciences, Siena University Hospital, Siena, Italy.

Background: Serum Krebs von den Lungen-6 (sKL-6) is an high-molecular-weight (200 kDa) glycoprotein predominantly expressed by damaged alveolar type II cells, and it has been proposed as a potential biomarker of different ILD. This is a prognostic biomarker for chronic hypersensitivity pneumonitis (cHP) and idiopathic pulmonary fibrosis (IPF), two diseases that share several clinical and radiological features. Little data are available on the potential role of KL-6 in granulomatous and cystic interstitial lung diseases, including the orphan disease known as pulmonary Langerhans cell histiocytosis (PLCH). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/eci.13242DOI Listing

Erdheim-Chester disease: a case treated with IFN-α monitored using plasma and urine cell-free DNA.

Immunotherapy 2020 Apr 14;12(6):379-387. Epub 2020 Apr 14.

Department of Nephrology and National Clinical Research Center for Geriatrics, West China Hospital, Sichuan University, Chengdu City, Sichuan Province, China, 610041.

Erdheim-Chester disease is a rare form of non-Langerhans histiocytosis. A 40-year-old woman was diagnosed as Erdheim-Chester disease based on typical bone scintigraphy, symmetric osteosclerosis and findings of foamy, non-Langerhans histiocytes in bone marrow. mutation was detected in a bone biopsy. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.2217/imt-2019-0150DOI Listing
April 2020
2.440 Impact Factor

Foxp3 Tregs from Langerhans cell histiocytosis lesions co-express CD56 and have a definitively regulatory capacity.

Clin Immunol 2020 Apr 10;215:108418. Epub 2020 Apr 10.

Fiona Elsey Cancer Research Institute, Ballarat, Australia; Federation University Australia, Ballarat, Australia. Electronic address:

Langerhans cell histiocytosis (LCH) lesions contain myeloid lineage 'LCH' cells. Regulatory T cells (Tregs) are also enriched within lesions, although their role in LCH pathogenesis is unknown. LCH cells are thought to produce the transforming growth factor beta (TGF-β) within lesions, however whether Tregs contribute is unestablished. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.clim.2020.108418DOI Listing

Pulmonary Langerhans Cell Histiocytosis.

Semin Respir Crit Care Med 2020 Apr 12;41(2):269-279. Epub 2020 Apr 12.

Medical Service, Veterans Affairs Medical Center, Cincinnati, Ohio.

Pulmonary Langerhans cell histiocytosis (PLCH) is a diffuse cystic lung disease that is strongly associated with exposure to cigarette smoke. Recently, activating pathogenic mutations in the mitogen-activated protein kinase pathway have been described in the dendritic cells in patients with PLCH and have firmly established PLCH to be an inflammatory myeloid neoplasm. Disease course and prognosis in PLCH are highly variable among individual patients, ranging from spontaneous resolution to development of pulmonary hypertension and progression to terminal respiratory failure. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1055/s-0039-1700996DOI Listing

Solitary adult xanthogranuloma in external auditory canal: Cyto-histopathological correlation of an uncommon entity at an uncommon site.

Diagn Cytopathol 2020 Apr 10. Epub 2020 Apr 10.

Department of Pathology, Dr Baba Saheb Ambedkar Hospital and Medical College, Rohini, Delhi, India.

Juvenile xanthogranulomas (JXG) are the most common type of self-limiting non-Langerhans cell histiocytosis (LCH) usually presenting in infancy and early childhood. Clinically, they present as solitary to multiple rounded cutaneous nodules which resolve over time. Adult type xanthogranulomas (AXG), though histologically similar to JXG, are usually single and do not regress spontaneously. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/dc.24430DOI Listing

Adult Langerhans cell histiocytosis with recurrent vulvar ulcers.

Dermatol Ther 2020 Apr 10:e13387. Epub 2020 Apr 10.

Bezmialem Vakif University, Dermatology, Fatih, Istanbul, Turkey.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/dth.13387DOI Listing

Langerhans Cell Histiocytosis of the Thyroid Leading to the Diagnosis of a Disseminated Form.

Case Rep Endocrinol 2020 17;2020:6284764. Epub 2020 Mar 17.

Department of Endocrinology, Charles Nicolle Hospital, Tunis, Tunisia.

Langerhans cell histiocytosis (LCH) is a rare sporadic proliferative disorder of Langerhans cells. LCH rarely involves the thyroid gland. We report herein a case of a disseminated chronic form of LCH with a diagnosis established by histological examination of the thyroid gland. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1155/2020/6284764DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7103030PMC

Erdheim-Chester Disease With Extensive Pericardial Involvement: A Case Report and Systematic Review.

Cardiol Res 2020 Apr 10;11(2):118-128. Epub 2020 Mar 10.

Department of Medicine, Advocate Illinois Masonic Medical Center, Chicago, IL, USA.

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by systemic xanthogranulomatous infiltration. We described the case of a female adult presenting with pericardial effusion. Pericardial infiltration is the most frequent cardiac manifestation of ECD and is the one discussed in this article. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.14740/cr1025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7092773PMC

Uncommon cutaneous non-Langerhans cell histiocytosis arising from dermal dendritic cells in adults: a clinicopathological study of five cases.

Eur J Dermatol 2020 Feb;30(1):32-40

Department of Pathology, Peking University First Hospital, Beijing 100034, China.

Background: Non-Langerhans cell histiocytosis (non-LCH) is a collective term that encompasses a long list of rare "histiocytosis" that do not meet the criteria for Langerhans cell histiocytosis (LCH). Among cutaneous non-LCH, the xanthogranuloma (XG) family represents a distinct group of disorders derived from dermal dendritic cells (DDCs) at different stages of differentiation.

Objectives: To investigate the clinicopathological characteristics of the XG family in adults and review the relevant literature. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1684/ejd.2019.3679DOI Listing
February 2020

CSF1R Is Required for Differentiation and Migration of Langerhans Cells and Langerhans Cell Histiocytosis.

Cancer Immunol Res 2020 Apr 1. Epub 2020 Apr 1.

Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy.

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by tissue accumulation of CD1aCD207 LCH cells. In LCH, somatic mutations of the gene have been detected in tissue LCH cells, bone marrow CD34 hematopoietic stem cells, circulating CD14 monocytes, and BDCA1 myeloid dendritic cells (DC). Targeting in clonal Langerhans cells (LC) and their precursors is a potential treatment option for patients whose tumors have the mutation. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1158/2326-6066.CIR-19-0232DOI Listing

Langerhans cell histiocytosis of an intra-mammary lymph node in an 18-year-old woman.

Pathologica 2020 Mar;112(1):50-55

Department of Pathology, ASST Cremona, Italy.

We describe an 18-year-old woman with several month's history of a 12 x 7 mm palpable mammary nodule, that was hypoechoic, with regular margins and vascularization areas by ultrasound. A fibroadenoma was hypothesized (American College of Radiology BI-RADS 3). A 14 G needle biopsy was performed, showing a LC proliferation suspected for LCH of a lymph node, with florid dermatopathic lymphadenopathy in differential diagnosis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.32074/1591-951X-27-19DOI Listing

Long-term efficacy and safety of 2CdA (cladribine) in extra-pulmonary adult-onset Langerhans cell histiocytosis: analysis of 23 cases from the French Histiocytosis Group and systematic literature review.

Br J Haematol 2020 Mar 19. Epub 2020 Mar 19.

Service de Pneumologie, Assistance Publique-Hôpitaux de Paris, Centre National de Référence des Histiocytoses, Hôpital Saint-Louis, Paris, France.

Langerhans cell histiocytosis (LCH) is a rare protean disease that usually affects children. Few data are available for management of adult-onset cases. A complete picture of the efficacy and safety of 2CdA (2-chlorodeoxyadenosine, cladribine) is lacking. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/bjh.16449DOI Listing

Langerhans cell histiocytosis: A case report with oral manifestations and the role of pediatric dentists in the diagnosis.

Clin Case Rep 2020 Mar 15;8(3):545-549. Epub 2020 Feb 15.

Jordanian Royal Medical Services Amman Jordan.

Langerhans cell histiocytosis (LCH) is a benign disease that behaves malignantly. Early recognition and treatment of oral manifestation of LCH by pediatric dentist and other medical specialties is important to prevent further organ damage. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/ccr3.2726DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7069888PMC

Intra-Lesional Medicaments for the Management of Intra-Osseous Lesions of Maxilla and Mandible - Systematic Review.

J Int Soc Prev Community Dent 2020 Jan-Feb;10(1):36-45. Epub 2020 Jan 24.

Oral and Maxillofacial Surgery, Boston University, Boston, MA, USA.

Aims And Objectives: This study aimed to review the success or remission of intralesional medicaments in the management of intraosseous lesions in the oral cavity.

Materials And Methods: A comprehensive search was performed in two databases (PubMed and Scopus). Research articles, case reports, case series, and clinical trials were included. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4103/jispcd.JISPCD_295_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7055339PMC
January 2020

Adult Langerhans cell histiocytosis: An unusual cause of referred knee pain.

Malays Fam Physician 2019 31;14(3):60-64. Epub 2019 Dec 31.

Seremban Primary Health Care Clinic Jalan Rasah, 70300 Seremban Malaysia.

Langerhans cell histiocytosis (LCH) in adults is rare and regarded as an 'orphan disease.' The systemic symptoms of LCH can mimic many other undifferentiated diseases seen at the primary care level. Failure to diagnose and delays in referral are common pitfalls in the management of this disease. Read More

View Article

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7067507PMC
December 2019

BRAF V600E mutation in childhood Langerhans cell histiocytosis correlates with multisystem disease and poor survival.

Blood Cells Mol Dis 2020 05 30;82:102356. Epub 2019 Aug 30.

Department of Paediatrics, India. Electronic address:

Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia of children with systemic involvement and poor outcome. The altered RAS-RAF-MEK-ERK cell signalling pathway due to somatic mutation of BRAF V600E is the most common genetic abnormality associated with the disease. In the current study, we highlight the frequency of BRAF V600E in our cohort of LCH cases (n = 31) and its relation with clinical outcome. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.bcmd.2019.102356DOI Listing
May 2020
2.646 Impact Factor

Juvenile Xanthogranuloma: Case Report and Literature Review.

Int Med Case Rep J 2020 25;13:65-69. Epub 2020 Feb 25.

Cutaneous Leishmaniasis Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

Juvenile Xanthogranuloma (JXG) is a relatively uncommon non-Langerhans cell histiocytosis, which often occurs at an early age and is usually asymptomatic. Herein, we present the case of a 17-year-old man with numerous asymptomatic yellow-brown papulonodular lesions with a symmetric distribution on upper and lower extremities, face, and trunk, developed over the past 4 years. In the histopathologic examination, histiocytes with a Touton-like appearance were observed in favor of xanthogranuloma. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.2147/IMCRJ.S240115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7049274PMC
February 2020

Clinical Characteristics of Pediatric Patients With Sellar and Suprasellar Lesions Who Initially Present With Central Diabetes Insipidus: A Retrospective Study of 55 Cases From a Large Pituitary Center in China.

Front Endocrinol (Lausanne) 2020 20;11:76. Epub 2020 Feb 20.

Department of Neurosurgery, Chinese Academy of Medical Sciences and Peking Union Medical College, Peking Union Medical College Hospital, Beijing, China.

To increase knowledge for the early differential diagnosis and accurate therapeutic strategies for pediatric patients with sellar or suprasellar region (SSR) lesions who initially present with central diabetes insipidus (CDI). This is a retrospective review of 55 pediatric patients (≤14 years old) with identified lesions in the SSR who initially presented with CDI at a large pituitary center between 2012 and 2018. The following data were summarized: demographic, clinical, endocrine, and neuroimaging data, intraoperative findings, histopathological findings, treatments, and prognosis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3389/fendo.2020.00076DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7044264PMC
February 2020

[A case of Langerhans cell histiocytosis with hyperosmolar hyperglycemic syndrome].

Zhonghua Nei Ke Za Zhi 2020 Mar;59(3):232-234

Department of Endocrinology, Hainan General Hospital, Haikou 570311, China.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3760/cma.j.issn.0578-1426.2020.03.014DOI Listing

Diffuse normolipemic plane xanthoma and hepatitis C: chance?

An Bras Dermatol 2020 Mar - Apr;95(2):261-262. Epub 2020 Feb 17.

Department of Dermatology, Santa Casa de Misericórdia de Porto Alegre, Porto Alegre, RS, Brazil.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.abd.2019.08.026DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7175400PMC

A bite of truth through the teeth.

Gastroenterology 2020 Mar 3. Epub 2020 Mar 3.

Department of Pathology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1053/j.gastro.2020.02.050DOI Listing

A case report.

Zhong Nan Da Xue Xue Bao Yi Xue Ban 2020 Jan;45(1):96-101

Department of Respiratory and Critical Care Medicine, Second Xiangya Hospital, Central South University, Changsha 410011, China.

Langerhans cell histiocytosis (LCH) is a rare and unexplained disease that can involve in any organ or system in the body and displays a variety of clinical manifestations. A 31-year-old man, who had a more than 10-year smoke history, initially presented dry cough, polydipsia and diuresis, with recurrent spontaneous pneumothorax. Pulmonary high-resolution computed tomography showed diffuse cystic and nodular lesions. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.11817/j.issn.1672-7347.2020.180625DOI Listing
January 2020

Langerhans cell histiocytosis.

Blood 2020 Apr;135(16):1319-1331

Division of Pediatric Hematology-Oncology, Department of Pediatrics, Texas Children's Cancer Center and Baylor College of Medicine, Houston, TX.

Langerhans cell histiocytosis (LCH) is caused by clonal expansion of myeloid precursors that differentiate into CD1a+/CD207+ cells in lesions that leads to a spectrum of organ involvement and dysfunction. The pathogenic cells are defined by constitutive activation of the MAPK signaling pathway. Treatment of LCH is risk-adapted: patients with single lesions may respond well to local treatment, whereas patients with multisystem disease require systemic therapy. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1182/blood.2019000934DOI Listing