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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
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
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
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
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
Rodrigo S Raposo, M Catarina M D de Almeida, M da Conceição M A de Oliveira, M Manuela da Fonseca, M Teresa Cesário
Efficient production of poly-3-hydroxybutyrate (P(3HB)) based on glucose-xylose mixtures simulating different types of lignocellulosic hydrolysate (LCH) was addressed using Burkholderia sacchari, a wild strain capable of metabolizing both sugars and producing P(3HB). Carbon catabolite repression was avoided by maintaining glucose concentration below 10g/L. Xylose concentrations above 30g/L were inhibitory for growth and production. In fed-batch cultivations, pulse size and feed addition rate were controlled in order to reach high productivities and efficient sugar consumptions...
October 5, 2016: New Biotechnology
Sarangarajan Ranganathan
The study of Histiocytic lesions has been a passion of Pepper Dehner over the years. He has contributed several case series and reviews on various categories of these diseases for over 4 decades, with his earliest articles in the 1970s. He has written on all aspects of the disease including seminal articles on Langerhans cell histiocytosis (LCH) and their prognostic features, his experiences with regressing atypical histiocytosis, his encounters with malignant histiocytosis, and classic articles on juvenile xanthogranuloma...
September 1, 2016: Seminars in Diagnostic Pathology
Dalal Varsha, Manveen Kaur, Neena Chaudhary, Fouzia Siraj
Langerhans cell histiocytosis (LCH) is a relatively rare and unique disease characterized by an abnormal proliferation of immature dendritic cells. It is predominantly seen in children with adults showing less than ten times the incidence compared to childhood. The clinical presentation and organ involvement is highly variable. Oral manifestations generally consist of mucosal ulceration associated with lesions of the underlying bone. Lesions limited to the oral mucosa are rare. We present a case of a 45-year-old male who presented with an ulcer on the hard palate showing histopathologic features of LCH...
2016: German Medical Science: GMS E-journal
B H Lok, M R Chelius, J Yahalom
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
Julie Obert, Laetitia Vercellino, Axel Van Der Gucht, Constance de Margerie-Mellon, Emmanuelle Bugnet, Sylvie Chevret, Gwenaël Lorillon, Abdellatif Tazi
PURPOSE: The standard evaluation of multisystem Langerhans cell histiocytosis (LCH) includes a clinical evaluation, laboratory tests and a skeleton/skull X-ray survey, with chest high-resolution computed tomography (HRCT) in the case of pulmonary involvement. Preliminary reports suggest that (18)F-fluorodeoxyglucose positron emission tomography-computed tomography ((18)F-FDG PET-CT) may be useful for evaluating patients with LCH. METHODS: Fourteen consecutive adult patients with multisystem LCH were included in this retrospective study, and were evaluated using standard procedures and (18)F-FDG PET-CT...
September 20, 2016: European Journal of Nuclear Medicine and Molecular Imaging
Willemijn T Quispel, Eline C Steenwijk, Vincent van Unen, Susy J Santos, Lianne Koens, Reina Mebius, R Maarten Egeler, Astrid G S van Halteren
Langerhans cell histiocytosis (LCH) is a neoplastic myeloid disorder with a thus far poorly understood immune component. Tertiary lymphoid structures (TLS) are lymph node-like entities which create an immune-promoting microenvironment at tumor sites. We analyzed the presence and clinical relevance of TLS in n = 104 H&E-stained, therapy-naive LCH lesions of non-lymphoid origin and applied immunohistochemistry to a smaller series. Lymphoid-follicular aggregates were detected in 34/104 (33%) lesions. In line with the lymphocyte recruitment capacity of MECA-79(+) high endothelial venules (HEVs), MECA-79(+)-expressing-LCH lesions (37/77, 48%) contained the most CD3(+) T-lymphocytes (p = 0...
August 2016: Oncoimmunology
Kaixuan Zeng, Zhe Wang, Koichi Ohshima, Yixiong Liu, Weichen Zhang, Lu Wang, Linni Fan, Mingyang Li, Xia Li, Yingmei Wang, Zhou Yu, Qingguo Yan, Shuangping Guo, Jie Wei, Ying Guo
Langerhans cell histiocytosis (LCH) is a neoplasm of myeloid origin characterized by a clonal proliferation of CD1a(+)/CD207(+) dendritic cells. Recurrent BRAF V600E mutation has been reported in LCH. In the present report, we confirm the feasibility of the high-specificity monoclonal antibody VE1 for detecting BRAF V600E mutation in 36/97 (37.1%) retrospectively enrolled patients with LCH; concordant immunohistochemistry and Sanger sequencing results were seen in 94.8% of cases. We then assessed the tumor immune microenvironment status in LCH, and found that the GATA binding protein 3 (GATA3)(+)/T-bet(+) ratio could distinguish between clinical multi-system/single-system (SS) multifocal and SS unifocal LCH...
July 2016: Oncoimmunology
Elisabetta Ferrero, Angelo Corti, Julien Haroche, Daniela Belloni, Barbara Colombo, Alvise Berti, Giulio Cavalli, Corrado Campochiaro, Antonello Villa, Fleur Cohen-Aubart, Zahir Amoura, Claudio Doglioni, Lorenzo Dagna, Marina Ferrarini
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis (LCH) characterized by tissue infiltration with CD68(+) foamy histiocytes. TNF-related chronic inflammation and mutations in the MAP kinase signaling pathway in histiocytes are recognized as the two major pathogenic events. Among pleomorphic clinical manifestations, cardiovascular involvement is frequent and prognostically relevant. Evaluation of ECD clinical course and response to treatment is, however, still challenging. Taking advantage of the two largest cohorts of ECD patients worldwide, we investigated the relevance and the potential of circulating Chromogranin A (CgA), a pro-hormone involved in cardiovascular homeostasis and inflammation, as a biomarker of response to therapy in ECD...
July 2016: Oncoimmunology
Narges Gholami
INTRODUCTION: Langerhans cell histiocytosis (LCH) is a proliferative disorder of histiocytes in multiple organs. Langerhans cell histiocytosis involves bones, skin, lung and other organs. CASE PRESENTATION: This study describes a seven-month-old Iranian girl who presented with skin rash and cervical lymphadenopathy. Langerhans cell histiocytosis was suspected when it was associated with anemia, splenomegaly and lytic bone lesions. A skin biopsy confirmed the diagnosis of Langerhans cell histiocytosis...
June 2016: Iranian Red Crescent Medical Journal
Elzbieta Skowronska-Jozwiak, Stanislaw Sporny, Joanna Szymanska-Duda, Dominika Baranowska, Andrzej Lewinski
Langerhans cell histiocytosis (LCH) in adults is a rare disorder of unknown etiology characterized by monoclonal proliferation of Langerhans cells. It belongs to dendritic cell disorders and occurs in 1-2 adults per million. The most common endocrine manifestation of classical LCH is associated with the posterior pituitary, with clinical symptoms of diabetes insipidus. Less than 80 reported cases of LCH involving the thyroid gland have been published so far. We present the case of a 39 years old woman with 10 years history of diabetes insipidus and secondary amenorrhoea, which appeared after second delivery...
July 12, 2016: Neuro Endocrinology Letters
Bobby Wirawan Hassan, Bong Ju Moon, Young-Jin Kim, Sang-Deok Kim, Ki-Young Choi, Jung-Kil Lee
INTRODUCTION: Langerhans cell histiocytosis (LCH) occurs rarely in the spine of adults. The radiological findings usually resemble vertebral tumors. Etiology of LCH has not been clearly established yet. Therapeutic approaches are still controversial. We describe a case of LCH in an adult spine. CASE DESCRIPTION: A patient who presented with low back pain had an osteolytic lesion in the L1 vertebral body without neurological deficits, and fluoroscopy-guided needle biopsy of the L1 vertebral body was performed...
2016: SpringerPlus
Kaixuan Zeng, Koichi Ohshima, Yixiong Liu, Weichen Zhang, Lu Wang, Linni Fan, Mingyang Li, Xia Li, Zhe Wang, Shuangping Guo, Qingguo Yan, Ying Guo
Langerhans cell histiocytosis (LCH) is a proliferative disease of CD1a(+) /CD207(+) dendritic cells. Recurrent BRAFV600E and MAP2K1 mutations have been reported in LCH. To investigate the relationship among the mutation, clinical findings, and differentiation status of LCH, respectively, we studied 97 cases of LCH by using Sanger sequencing and immunohistochemistry. The mutually exclusive BRAFV600E and MAP2K1 mutation rates were 32% and 17.5%, respectively. All MAP2K1 mutations were missense mutations without any in-frame deletions; 2 new recurrent missense mutations (ie, p...
September 6, 2016: Hematological Oncology
Ramya Uppuluri, Sreejith Ramachandrakurup, Divya Subburaj, Atish Bakane, Revathi Raj
Refractory/relapsed Langerhans cell histiocytosis (LCH) has a difficult course with a guarded prognosis. We used a novel protocol including six cycles of pulse dexamethasone and lenalidomide in four children with LCH refractory to first-line agents and courses of cladribine and cytarabine or single-agent cladribine. All four children completed the protocol without any significant adverse effects and remain in complete and durable remission 15-18 months posttreatment. The novel protocol we propose for relapsed/refractory LCH is cost-effective and outpatient-based with durable remission and minimal toxicity...
August 24, 2016: Pediatric Blood & Cancer
Jun-Bin Huang, Li Jiang, Chun Chen, Hong-Man Xue
OBJECTIVE: To analyze and compare the clinical features, treatment and prognosis of 31 children with Langerhans cell histiocytosis(LCH) treated with modified DAL-HX83/90 or JLSG-96 protocol. METHODS: The clinical features, treatment and prognosis of 31 children with Langerhans cells admitted in our hospital from January 2005 to December 2014 were analyzed retrospectively. The outcome of patients treated with modified DAL-HX83/90 or JLSG-96 protocols were compared by using the Kaplan-Meier survival curve...
August 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
Sudha Sharma, Pranab Dey
Isolated pulmonary Langerhans cell histiocytosis (LCH) is rare in childhood, and few cases have been diagnosed on bronchoalveolar lavage (BAL) smears. We hereby report a child with cystic lesions in both lungs diagnosed as LCH on BAL smears and compare the cytomorphologic findings with BAL smears of five patients with various pathologies. The BAL smears of the index case showed numerous macrophages with many atypical histiocytes showing coffee bean-shaped nuclei, nuclear grooving along with few giant cells...
August 16, 2016: Diagnostic Cytopathology
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