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https://www.readbyqxmd.com/read/27908938/a-rare-case-of-extensive-cranial-langerhans-cell-histiocytosis-synchronously-presenting-as-otitis-externa-and-giant-cell-arteritis
#1
Adnan Darr, Zahir Mughal, Thomas Martin
Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder of unpredictable clinical course and varied modes of presentation. The spectrum of presentation is wide, ranging from isolated eosinophilic granulomas to multiple lesions and diffuse systemic involvement. We present the case of a 52-year-old man, who presented with an 8-week history of worsening otalgia and superficial temporal tenderness attributed to otitis externa within the community and subsequently giant cell arteritis. Computed tomography and magnetic resonance imaging were undertaken due to atypical features, which demonstrated bony destruction within the right greater wing of the sphenoid, squamous part of temporal and mastoid bone, with middle cranial fossa communication...
December 1, 2016: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/27902613/lobular-capillary-hemangioma-of-the-tracheobronchial-tree-a-case-report-and-literature-review
#2
Xiaojian Qiu, Zhiwu Dong, Jie Zhang, Jin Yu
RATIONALE: Lobular capillary hemangioma (LCH) of the tracheobronchial tree is a rare benign tumor, whose characteristics and treatments remain relatively unknown. PATIENT CONCERNS: A 39-year-old man with hemoptysis caused by neoplasm in the bronchus intermedius was admitted to our hospital. DIAGNOSES: The patient was diagnosed with LCH. INTERVENTIONS: The lesions were removed with biopsy forceps, and cryotherapy was performed...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27898473/histopathological-and-clinical-findings-in-cutaneous-manifestation-of-erdheim-chester-disease-and-langerhans-cell-histiocytosis-overlap-syndrome-associated-with-the-brafv600e-mutation
#3
Julia Liersch, J Andrew Carlson, Jörg Schaller
The overlap of Erdheim-Chester disease (ECD) and Langerhans cell histiocytosis (LCH) is more common than it was generally accepted. Both diseases seem to be linked by a mutation in oncogenic BRAFV600E, probably an early event which occurs in bone marrow progenitor cells. In this article are described the clinical and histological findings in 2 cases of ECD-LCH overlap syndrome bearing the BRAFV600E mutation in both ECD and LCH lesions in bone and skin. In one case, lesions of ECD and LCH were situated directly site-to-site in the same bone section leading to the assumption of a common myeloid precursor cell for these diseases...
November 23, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27894453/usual-and-unusual-manifestations-of-familial-hemophagocytic-lymphohistiocytosis-and-langerhans-cell-histiocytosis
#4
REVIEW
Craig Erker, Paul Harker-Murray, Julie-An Talano
Familial hemophagocytic lymphohistiocytosis (FHL) and Langerhans cell histiocytosis (LCH) are histiocytic diseases that occur most commonly in young children. Improvements in recognition and treatment have been substantial for both diseases in the past decade, although early and late morbidity continue to be major concerns. These two diagnoses behave differently, although the clinical spectra for both diseases are diverse and can lead to confusion and delays in diagnosis and treatment. This article focuses on the clinical and genetic spectrum of FHL as well as the clinical and treatment variations of LCH...
February 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/27867869/thyroid-langerhans-cell-histiocytosis-and-papillary-thyroid-carcinoma
#5
Rajab AlZahrani, Mohammed Algarni, Hadi Alhakami, Haia AlSubayea, Naif Alfattani, Mohammet Guler, Mohamed Satti
A 27-year-old female, married with two children, presented to our clinic with a 1-year history of thyroid swelling and pressure symptoms on lying backward and bilateral cervical lymphadenopathy. The patient was a known case of panhypopituitarism for 5 years. Comprehensive patient evaluation including FNAC with papillary thyroid cancer result then she underwent total thyroidectomy and bilateral neck dissection and final histologic examination confirmed papillary thyroid carcinoma in the background of lymphocytic thyroiditis, associated with Langerhans cell histiocytosis (LCH)...
October 2016: Gland Surgery
https://www.readbyqxmd.com/read/27862059/comprehensive-analysis-of-host-cell-impurities-in-monoclonal-antibodies-with-improved-sensitivity-by-capillary-zone-electrophoresis-mass-spectrometry
#6
Zhenbin Zhang, Thomas Albanetti, Travis Linkous, Christopher J Larkin, Ronald Schoner, James B McGivney, Norman J Dovichi
Four methods were compared for analysis of host-cell protein (HCP) impurities in a recombinant monoclonal antibody. First, capillary zone electrophoresis-tandem mass spectrometry (CZE-MS/MS) was used to analyze the digest of a host-cell protein sample following extraction of the monoclonal antibody with Protein A and Protein L affinity columns; 220 protein groups and 976 peptides were identified from the depleted HCP digest. Second, a nanoACQUITY UltraPerformance LCH system was also used to analyze the depleted HCP digest; 34 protein groups and 53 peptides from 50 ng of the depleted HCP digest and 290 protein groups and 1011 peptides were identified from 1 μg of the depleted HCP digest...
November 12, 2016: Electrophoresis
https://www.readbyqxmd.com/read/27846659/the-non-langerhans-cell-histiocytoses-rare-histiocytoses-clinical-aspects-and-therapeutic-approaches
#7
C F Classen, M Minkov, T Lehrnbecher
Rare histiocytoses, also called non-Langerhans cell histiocytoses, include all proliferative disorders of histiocytes, macrophages and dendritic cells that are not classified as Langerhans cell histiocytosis (LCH) and do not belong to the hemophagocytic lymphohistiocytosis (HLH) group of diseases. Thus, the term includes numerous benign or malignant, localized or systemic, adult or pediatric diseases. The classification of the histiocytic disorders has been revised several times. Here, we follow the classification recently published by Jean Francois Emile and an international expert panel, defining subgroups of histiocytoses described as L-Group, C-Group, M-Group, R-Group, and H-Group, which stands for LCH-like, cutaneous or mucocutaneous, malignant, Rosai-Dorfman-Disease like and HLH like...
November 2016: Klinische Pädiatrie
https://www.readbyqxmd.com/read/27845884/central-diabetes-insipidus-clinical-profile-that-suggests-organicity-in-peruvian-children-lima-peru-2001-2013
#8
Miguel Angel De Los Santos, Carlos Manuel Del Águila, Maria Isabel Rojas, Juan Manuel Falen, Oswaldo Nuñez, Eliana Manuela Chávez, Oscar Antonio Espinoza, Paola Marianella Pinto, Martha Rosario Calagua
BACKGROUND: Central diabetes insipidus (CDI) is a heterogeneous disease caused by arginine vasopressin deficiency; its management implies a profound understanding of the pathophysiology and the clinical spectrum. The aim of the study was to describe the clinical characteristics that indicate organicity in children and adolescents with central diabetes insipidus treated at the Department of Endocrinology from The Child Health's Institute during 2001 to 2013. METHODS: Cross-sectional, retrospective study...
December 1, 2016: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/27833773/a-fatal-case-of-congenital-langerhans-cell-histiocytosis-with-disseminated-cutaneous-lesions-in-a-premature-neonate
#9
Michio Inoue, Yoko Tomita, Tsuyoshi Egawa, Tomoaki Ioroi, Masaaki Kugo, Shinsaku Imashuku
Background. The outcome of neonates with congenital cutaneous Langerhans cell histiocytosis (LCH) is variable. Observations. We report a case of LCH in a female premature neonate born at 33-week gestation. She had disseminated cutaneous lesions, which consisted of hemorrhagic papules and vesicles, with sparse healthy skin areas, and the hands and feet were contracted with scarring and blackened. She was in respiratory failure although no apparent pulmonary or bone lesions on X-rays were noted. Skin biopsy confirmed a diagnosis of LCH due to observation of CD1a(+) Langerhans cells, which lacked expression of E-cadherin and CD56...
2016: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/27823979/high-frequency-of-clonal-ig-and-t-cell-receptor-gene-rearrangements-in-histiocytic-and-dendritic-cell-neoplasms
#10
Wenting Huang, Tian Qiu, Linshu Zeng, Bo Zheng, Jianming Ying, Xiaoli Feng
The 2008 World Health Organization (WHO) diagnostic criteria of histiocytic and dendritic cell neoplasms from hematopoietic and lymphoid tissues no longer required the absence of clonal B-cell/T-cell receptor gene rearrangements. It is true that the clonal B-cell/T-cell receptor gene rearrangements have been identified in rare cases of histiocytic and dendritic cell neoplasms, such as those with or following lymphoma/leukemia or in some sporadic histiocytic/dendritic cell sarcomas, but the clonal features of such group of tumor are still not clear...
November 3, 2016: Oncotarget
https://www.readbyqxmd.com/read/27816346/-neuro-langerhans-cell-histiocytosis
#11
Loïc Le Guennec, Nadine Martin-Duverneuil, Karima Mokhtari, Maria Santiago-Ribeiro, Eléonore Bayen, Antoine Del Cul, Daniel Delgadillo, Aurélie Kas, Carine Courtillot, Julien Haroche, Fleur Cohen, Jean Donadieu, Khê Hoang-Xuan, Ahmed Idbaih
Langerhans cell histiocytosis (LCH) is a rare multisystemic disease. LCH is characterized by proliferation of myeloid progenitors with altered differentiation program and similar phenotypic features to epidermal dendritic cells termed Langerhans cell. LCH cells express CD1a+ and langerin and exhibit BRAF V600E mutation in ∼50% of cases. Neurological involvement or neuro-LCH is observed in 5 to 10% of cases. Three subtypes of neuro-LCH are individualized. The tumor type, accounting for 45% of neuro-LCH, affect mainly young adults...
November 2, 2016: La Presse Médicale
https://www.readbyqxmd.com/read/27816342/-adult-pulmonary-langerhans-cell-histiocytosis
#12
Gwenaël Lorillon, Véronique Meignin, Abdellatif Tazi
There is a strong relationship between tobacco smoking and pulmonary Langerhans cell histiocytosis (LCH) in adults. Definitive diagnosis relies on lung histology. In certain cases, the diagnosis can be retained on a typical nodulo-cystic pattern on lung high-resolution computed tomography (HRCT) and appropriate clinical setting. The main differential diagnoses to consider vary according to nodulo-cystic or only cystic pattern on lung HRCT and the clinical context. The natural history of the disease is better known and regular evaluation of respiratory function is essential during the follow-up of the patients...
November 2, 2016: La Presse Médicale
https://www.readbyqxmd.com/read/27816338/-adult-langerhans-cell-histiocytosis
#13
Mathilde de Menthon, Véronique Meignin, Alfred Mahr, Abdellatif Tazi
Langerhans cell histiocytosis (LCH) is a rare disease affecting both genders and can occur at any age. It often evolves through successive flares, and its severity varies from benign forms that don't require treatment to life threatening disease. Some patients have important functional impairment with psychological and social consequences and prolonged disability. LCH may affect only one organ, with uni- or multifocal involvement or be multisystem disease involving multiple organs. The organs most frequently involved are bones, lung, skin and the endocrinal system...
November 2, 2016: La Presse Médicale
https://www.readbyqxmd.com/read/27804235/a-phase-iia-study-of-afuresertib-an-oral-pan-akt-inhibitor-in-patients-with-langerhans-cell-histiocytosis
#14
Robert J Arceci, Carl E Allen, Ira J Dunkel, Eric Jacobsen, James Whitlock, Robert Vassallo, Shannon R Morris, Alison Portnoy, Beth Ann Reedy, Deborah A Smith, Robert Noble, Amy Murnane, Mark Cornfeld, Carlos Rodriguez-Galindo, Mark L Heaney, Kenneth McClain, Sarah Vaiselbuh
BACKGROUND: Langerhans cell histiocytosis (LCH) is a clonal neoplasm characterized by widely varied clinical presentations, including multisystem involvement and systemic inflammatory symptoms. The AKT pathway is relevant to survival and proliferation of dendritic cells, and is also often upregulated in hematopoietic malignancies. A clinical response in an adult patient with LCH participating in the first-in-human trial of afuresertib prompted this prospective trial. PROCEDURE: The population in the current study included treatment-naïve (n = 7) and recurrent/refractory patients with LCH (n = 10), who received oral afuresertib (125 mg)...
November 2, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27785447/insights-into-the-pathogenesis-of-langerhans-cell-histiocytosis-the-development-of-targeted-therapies
#15
REVIEW
Caroline Hutter, Milen Minkov
More than a century after its first description, Langerhans cell histiocytosis (LCH) still remains an intriguing disease. Considerable progress in understanding its biology has been achieved recently. Description of the V600E BRAF mutation in samples of LCH tissue in 2010 was followed by description of additional mutations, all leading to constitutive ERK activation. Current experimental data suggest that LCH is a myeloid neoplasia with inflammatory properties, yet the exact pathophysiology remains poorly understood...
2016: ImmunoTargets and Therapy
https://www.readbyqxmd.com/read/27761291/langerhans-cell-histiocytosis-with-atypical-intervertebral-disc-and-sacroiliac-joint-involvement-mimicking-osteoarticular-tuberculosis-in-an-adult
#16
Zeynep Maraş Özdemir, Ayşegül Sağır Kahraman, Cemile Ayşe Görmeli, Reşit Sevimli, Nusret Akpolat
BACKGROUND: Langerhans cell histiocytosis (LCH), typically found in children, is a rare single or multisystem disorder with a wide range of clinical and radiological manifestations. Unusual presentations of LCH are occasionally encountered and it may be difficult to distinguish LCH from an infection or a benign or malignant tumor. RESULTS: A 35-year-old female presented with pain in her back and left buttock, malaise, and weight loss, with a duration of several months...
September 2016: Balkan Medical Journal
https://www.readbyqxmd.com/read/27759703/p53-is-a-helpful-marker-in-distinguishing-langerhans-cell-histiocytosis-from-langerhans-cell-hyperplasia
#17
Shane A Grace, Angela M Sutton, Eric S Armbrecht, Claudia I Vidal, Ilana S Rosman, Maria Y Hurley
Langerhans cell histiocytosis (LCH) is a proliferative disorder of Langerhans cells that can be challenging to distinguish histologically from Langerhans cell (LC) hyperplasia, seen in a variety of inflammatory dermatoses. Lesional cells in both entities demonstrate positive staining for CD1a and S100. Previous studies have demonstrated positive staining of fascin, CD31, and p53 in cases of LCH, but currently, no studies have compared the staining profiles of these markers between LCH and LC hyperplasia. The authors compared immunohistochemical staining profiles of LCH (n = 15) and various inflammatory dermatoses with LC hyperplasia (n = 15) using fascin, CD31, and p53...
October 18, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27759701/concomitant-cutaneous-langerhans-cell-hystiocytosis-and-leukemia-cutis
#18
Sergio Pina-Oviedo, Carlos A Torres-Cabala, Roberto N Miranda, Michael T Tetzlaff, Selina Singh, Ronald P Rapini, Victor G Prieto, Phyu P Aung
Leukemia cutis develops in <4% of all acute leukemias. Concurrent acute myeloid leukemia (AML) and Langerhans cell histiocytosis (LCH) is rare, with most cases involving lymph nodes or spleen, and no cutaneous involvement. We report the case of a 59-year-old man who presented with fever, malaise, and fatigue. The CBC showed leukocytosis (30.4 × 10/L, 9% blasts), anemia, and thrombocytopenia. Bone marrow biopsy was diagnosed with AML, not otherwise specified, with mutations of FLT3 and IDH2 (R140Q). The patient developed skin rash on the right flank with the clinical differential diagnosis of herpes simplex virus or varicella-zoster virus infection/reactivation versus leukemia cutis...
October 18, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27747126/tracheal-lobular-capillary-haemangioma-a-rare-benign-cause-of-recurrent-haemoptysis
#19
Metesh Nalin Acharya, Konstantinos Kotidis, Mahmoud Loubani
Lobular capillary haemangioma (LCH), previously known as pyogenic granuloma, is a benign vascular lesion commonly found within the oral and nasal cavity. However, it is rarely encountered within the trachea, where presenting features include recurrent haemoptysis, cough, and wheeze. We here describe a case of a 7 mm tracheal LCH in a 56-year-old woman, which was successfully resected at interventional bronchoscopy using biopsy forceps. Clinicians should be aware of tracheal LCH in the differential diagnosis for recurrent haemoptysis...
2016: Case Reports in Surgery
https://www.readbyqxmd.com/read/27729324/alternative-genetic-mechanisms-of-braf-activation-in-langerhans-cell-histiocytosis
#20
Rikhia Chakraborty, Thomas M Burke, Oliver A Hampton, Daniel J Zinn, Karen Phaik Har Lim, Harshal Abhyankar, Brooks Scull, Vijetha Kumar, Nipun Kakkar, David A Wheeler, Angshumoy Roy, Poulikos I Poulikakos, Miriam Merad, Kenneth L McClain, D Williams Parsons, Carl E Allen
Langerhans cell histiocytosis (LCH) is characterized by inflammatory lesions containing pathologic CD207+ dendritic cells with constitutively activated ERK. Mutually exclusive somatic mutations in MAPK pathway genes have been identified in approximately 75% of LCH cases, including recurrent BRAF-V600E and MAP2K1 mutations. In order to elucidate mechanisms of ERK activation in the remaining 25% of patients, we performed whole exome sequencing (WES, n=6), targeted BRAF sequencing (n=19) and/or whole transcriptome sequencing (RNA-seq, n=6) on 24 LCH patient samples lacking BRAF-V600E or MAP2K1 mutations...
October 11, 2016: Blood
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