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Langerhans cell histiocytosis

Julieta Ruiz Beguerie, Julia Fernández, Matías F Stringa, Javier Anaya
No abstract text is available yet for this article.
October 25, 2016: International Journal of Dermatology
M Cascarino, Y Caron, C Butnaru, F Rongioletti, S Fraitag
BACKGROUND: Progressive mucinous histiocytosis is a very rare, benign, non-Langerhans' cell histiocytosis limited to the skin. This disorder has been observed solely in women, with the exception of three cases in male patients, and most cases are hereditary. The hereditary forms begin in childhood, with sporadic cases occurring later, and it is characterized by numerous papules of slow progression. The aetiology and mode of genetic transmission remain unclear. We report one sporadic case of progressive mucinous histiocytosis...
October 17, 2016: Annales de Dermatologie et de Vénéréologie
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
Zhen Huo, Tao Lu, Zhiyong Liang, Fan Ping, Jie Shen, Jingjing Lu, Wenbing Ma, Dachun Zhao, Dingrong Zhong
BACKGROUND: Isolated hypothalamic-pituitary Langerhans cell histiocytosis (HPLCH) is very rare. We investigated the clinicopathological characteristics, endocrine function changes, BRAF(V600E) mutations and treatments of isolated HPLCH. METHODS: We identified seven patients with isolated HPLCH by reviewing the clinical and pathological files in our hospital from 2007 to 2015. The clinical characteristics of the seven patients were retrospectively reviewed, especially the endocrine function changes...
October 19, 2016: Diagnostic Pathology
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
Mohammad Adawi, Bishara Bisharat, Abdalla Bowirrat
BACKGROUND: Erdheim-Chester disease (ECD) is an uncommon aggressive, multisystem form of non-Langerhans' cell histocytosis, which was firstly reported by Jakob Erdheim and William chester in 1930. The disease pathological features encompass an aberrant multiplication, overproduction and accumulation of white blood cells called histiocytes within multiple tissues and organs. Herein, we present a case of ECD owing to the rarity of this disease (roughly 550 cases have been described in the literature to date)...
October 2016: Medicine (Baltimore)
Atman A Dave, Susan E Gutschow, Christopher M Walker
Erdheim-Chester disease (ECD) is a rare multisystemic non-Langerhans cell histiocytosis that may be clonal and inflammatory in origin. The hallmark of the disease is infiltration of various organ systems by CD68+/CD1a- histiocytes containing foamy lipid-laden inclusions. The manifestations and course of the disease are variable and depend on the organ systems that are affected. Patients may be asymptomatic or may develop life-threatening complications, including myocardial infarction. The most common clinical manifestation is lower extremity bone pain...
September 13, 2016: Curēus
Marie-Paule Guillaume, François Dubos, François Godart
We report the case of a 2-year-old boy with severe Langerhans cell histiocytosis who had tricuspid endocarditis caused by Staphylococcus lugdunensis and required surgery despite appropriate antimicrobial therapy. Through this case and literature review of endocarditis caused by S. lugdunensis in children, we highlight pitfalls and mistakes to be avoided in the management of this rare but serious infection.
October 17, 2016: Cardiology in the Young
Nishant Gupta, Dale Langenderfer, Francis X McCormack, Daniel P Schauer, Mark H Eckman
RATIONALE: Patients without a known history of lung disease presenting with a spontaneous pneumothorax are generally diagnosed as primary spontaneous pneumothorax. However, occult diffuse cystic lung diseases such as Birt-Hogg-Dubé syndrome (BHD), lymphangioleiomyomatosis (LAM), and pulmonary Langerhans cell histiocytosis (PLCH) can also first present with a spontaneous pneumothorax, and their early identification by high-resolution computed tomographic (HRCT) imaging of chest has implications for subsequent management...
October 13, 2016: Annals of the American Thoracic Society
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
S Nirmala
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
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
Seth Kligerman, Teri J Franks, Jeffrey R Galvin
The direct toxicity of cigarette smoke and the body's subsequent response to this lung injury leads to a wide array of pathologic manifestations and disease states that lead to both reversible and irreversible injury to the large airways, small airways, alveolar walls, and alveolar spaces. These include emphysema, bronchitis, bronchiolitis, acute eosinophilic pneumonia, pulmonary Langerhans cell histiocytosis, respiratory bronchiolitis, desquamative interstitial pneumonia, and pulmonary fibrosis. Although these various forms of injury have different pathologic and imaging manifestations, they are all part of the spectrum of smoking-related diffuse parenchymal lung disease...
November 2016: Radiologic Clinics of North America
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
Nina Korsaga-Somé, Élodie Miquelestorena-Standley, Antoine Finon, Marion Yvert, Aurélie Sallot, Annabel Maruani
No abstract text is available yet for this article.
September 23, 2016: European Journal of Dermatology: EJD
Marek Lommatzsch, Kai Bratke, Paul Stoll, Norbert Mülleneisen, Friedrich Prall, Andrea Bier, J Christian Virchow
BACKGROUND: The histologic diagnosis of Pulmonary Langerhans cell histiocytosis (PLCH) is invasive and can cause complications. To confirm the diagnosis of PLCH, guidelines therefore recommend measuring CD1a-positive bronchoalveolar lavage fluid (BALF) cells despite its poor sensitivity and specificity. Thus, an improved diagnostic accuracy of BALF cell analysis would be desirable. METHODS: Using four-colour flow cytometry, plasmacytoid and myeloid dendritic cells (DCs) were analysed in BALF of 10 newly diagnosed, untreated, smoking patients with PLCH, and compared with BALF DCs from 40 asymptomatic smokers and 21 never-smokers...
October 2016: Respiratory Medicine
Daniel J Zinn, Amanda B Grimes, Howard Lin, Olive Eckstein, Carl E Allen, Kenneth L McClain
No abstract text is available yet for this article.
September 29, 2016: Blood
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
Abid Hussain, Ashwani Tandon, Aruna K Prayaga, Tara Roshni Paul, Anukonda M V R Narendra
OBJECTIVES: Rosai-Dorfman disease (RDD) is an uncommon, benign histiocytic disorder of unknown etiology, typically presenting in young adulthood. We highlight the cytomorphology of RDD and correlate it with the histopathology. STUDY DESIGN: All cases diagnosed as RDD on fine-needle aspiration cytology between January 2001 and June 2015 were included. Clinical details were obtained from medical records. The cytology smears were reviewed along with the histopathology and immunohistochemistry, wherever available...
September 28, 2016: Acta Cytologica
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