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Langerhans cell histiocytosis

Zachary E Holcomb, Sherry H Yu, Tyler D Menge, Rosalynn M Nazarian, Chad J Jessup
Langerhans cell histiocytosis is a rare and clinically heterogeneous group of dendritic histiocytic disorders with typical onset in the neonatal period or infancy, although it can present at any age. Histiocytes accumulate in one or more organs, leading to a variable clinical presentation of disease. We report a case of biopsy-proven Langerhans cell histiocytosis in a newborn and discuss the workup and management of this disease, along with reviewing its clinical variants.
July 2019: Dermatopathology (Basel, Switzerland)
Baha Obaidat, Dina Yazdani, Kathryn A Wikenheiser-Brokamp, Nishant Gupta
Diffuse cystic lung diseases (DCLDs) are a group of diverse pulmonary disorders with varying pathophysiology that are characterized by the presence of thin-walled, air-filled spaces within lung parenchyma. High-resolution computed tomography plays a crucial role in the evaluation of DCLDs, and cyst characteristics such as morphology, distribution, and the presence of other associated radiologic findings can help distinguish between different DCLDs. DCLDs can be classified according to their underlying pathophysiology as neoplastic, genetic, lymphoproliferative, infectious, associated with other forms of interstitial lung disease, or related to smoking...
October 15, 2019: Respiratory Care
Sophie Jessop, Donna Crudgington, Kevin London, Stewart Kellie, Robert Howman-Giles
OBJECTIVE: Langerhans cell histiocytosis (LCH) in pediatric patients presents with single-system or multisystem disease. Accurate staging is essential for selecting the most appropriate therapy ranging from local surgery to chemotherapy. METHODS: A retrospective review was undertaken of reported fludeoxyglucose (FDG) positron emission tomography - computed tomography (PET-CT) scans performed in children with LCH from June 2006 to February 2017. Findings were compared with a reference standard of biopsy or informed clinical follow-up...
October 10, 2019: Pediatric Blood & Cancer
Kazuko Kudo
Whether Langerhans cell histiocytosis (LCH) is an inflammatory disorder or a neoplasm is an ongoing debate. On the basis of the identification of the BRAF V600E mutation in 2010, LCH should be defined as an inflammatory myeloid neoplasia. Mutually exclusive BRAF V600E (50-60%) and MAP2K1 (12-25%) mutation renders the ERK activation. BRAF V600E mutations were detected in approximately 50-60% of patients with Erdheim-Chester disease (ECD), and these patients are being treated with vemurafenib, a selective BRAF V600 kinase inhibitor, approved by the US Food and Drug Administration...
2019: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Laura Wake, Liqiang Xi, Mark Raffeld, Elaine S Jaffe
No abstract text is available yet for this article.
September 2019: Human Pathology (New York)
Hussein Algahtani, Bader Shirah, Mohammed Bajunaid, Ahmad Subahi, Hatim Al-Maghraby
Langerhans cell histiocytosis (LCH) is a rare neoplasm that is caused by an uncontrolled proliferation of Langerhans cells. The clinical presentation of LCH is heterogeneous and can manifest as single or multiple osteolytic lesions, skin ulcerations, and involvement of single or multiple systems. Central nervous system (CNS) involvement is reported in 3.4-57% of patients with multisystem disease. In this article, we present the case of a young man with single system involvement (bone) of LCH who presented with seizures, headache, papilledema, and tinnitus...
September 2019: Gulf Journal of Oncology
Saeed Nezafati, Javad Yazdani, Shahriar Shahi, Mahsa Mehryari, Emran Hajmohammadi
Langerhans cell histiocytosis (LCH) is characterized by the congregation of proliferating langerhans cells (LC). Langerhans cells are a part of dendritic cell system of primary immune response that is responsible for presenting antigen to lymphocytes. Being a rare disease, the total incidence of LCH is reported to be 1 in 2 million people. LCH mainly affects children and young adults, with a slight male predilection. LCH is clinically divided into three groups namely Letter-Siwe disease (multiple multi organ affecting LCH at very young age), Hand-Schuler-Christian disease (LCH of bone involvement exophthalmos and diabetes insipidus), and Eosinophilic granuloma (LCH of bone, solitary or multiple)...
September 2019: Journal of Dentistry
Tanja A Gruber
In this issue of Cancer Discovery , Halbritter and colleagues utilize single-cell RNA sequencing to dissect the cellular hierarchy in Langerhans cell histiocytosis. They identified a remarkably consistent composition of 14 cellular subsets across all patients with a range of clinical spectrums consistent with a shared developmental hierarchy driven by key transcriptional regulators. See related article by Halbritter et al., p. 1406 .
October 2019: Cancer Discovery
Abhishek Singla, Elizabeth J Kopras, Nishant Gupta
BACKGROUND: The optimal approach for management of spontaneous pneumothoraces (SPs) and the safety of air travel in patients with pulmonary Langerhans cell histiocytosis (PLCH) are not well established. METHODS: Patients with PLCH were recruited from the Rare Lung Diseases Clinic Network and the Histiocytosis Association, and surveyed about disease manifestations and safety of air travel. RESULTS: A total of 94 patients completed the survey...
September 25, 2019: Respiratory Investigation
M Omar Iqbal, Ashirwad Merve, Nathalie Galea, Kristian Aquilina
Tumors of the CNS represent the largest group of solid tumors found in the pediatric patient population. Langerhans cell histiocytosis (LCH) is an inflammatory lesion that may present in bone and/or soft tissue, including the CNS. Management depends on the extent of multisystem involvement, which determines resection with or without systemic chemotherapy. The authors report on the case of a child who underwent an open craniotomy for biopsy of a pituitary stalk lesion followed by neuropathological assessment, procedures used to diagnose LCH...
September 27, 2019: Journal of Neurosurgery. Pediatrics
Nobuhiro Kanaji, Yoshimasa Tokunaga, Ryou Ishikawa, Naoki Watanabe, Norimitsu Kadowaki
BACKGROUND: The common computed tomography findings of pulmonary Langerhans cell histiocytosis (PLCH) are multiple cysts and micronodules predominantly in middle to upper lung lobes. Non-cystic nodules and large nodules are atypical findings of PLCH. CASE SUMMARY: The patient was a 48-year-old Japanese man with a smoking history (20 cigarettes/d, 28 years) and no symptoms. Multiple nodules existed in all lung lobes, predominantly in the right lower lobe. Some nodules seemed to be distributed randomly, and others were adjacent to bronchus...
September 6, 2019: World Journal of Clinical Cases
Adrian A Ong, Mattie Rosi-Schumacher, Michael Pizzuto
This is a case of a pediatric male who presented with a postauricular mass and pain. Computed tomography and magnetic resonance imaging suggested a diagnosis of Langerhans cell histiocytosis. Characteristic imaging findings and initial workup for Langerhans cell histiocytosis are described.
September 26, 2019: Ear, Nose, & Throat Journal
Edward Bellamy, Stefano Di Palma, Lorenzo Ressel, Elisabet Domínguez, Yordan Fernández
Case summary: An 11-year-old female spayed domestic shorthair cat was referred with a 2-month history of ptyalism, hyporexia and weight loss. Physical examination revealed reduced body condition score (2/9) and decreased skin turgor. Laboratory abnormalities included mild erythrocytosis, elevated creatine kinase, hypercobalaminaemia and hypofolataemia. CT of the head and abdominal ultrasonography were within normal limits. Gastroesophagoscopy revealed mucosal ulceration and possible stenosis of the distal oesophagus...
July 2019: JFMS open reports
Shanice McKenzie, Natalia Vecerek, Yuna Kang, Byron Knowles, Marcia Hogeling
Langerhans cell histiocytosis (LCH) is a rare, clinically heterogeneous disease that most commonly occurs in pediatric populations. Congenital self-limited LCH is a benign variant of LCH. It most commonly presents as a diffuse eruption and reports of single lesion cases are infrequent in the literature. Even in the case of congenital self-limited LCH, there is potential for future multisystem relapse, making long-term follow-up important. We present a case of single lesion self-limited LCH in a full-term male infant with interesting morphology...
August 15, 2019: Dermatology Online Journal
Wassim Baassiri, Charbel K Moussalem, Elie Massaad, Youssef H Zeidan, Houssein Darwish
Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, a rare non-Langerhans cell reactive histiocytic disorder that rarely occurs in the central nervous system (CNS). Extranodal RDD most frequently involves the skin, upper respiratory tract, soft tissue, gastrointestinal tracts, bones, breast, and CNS. RDD of the CNS infiltrates most commonly the dura of the sella, cavernous sinus, and the periclivial regions. It is usually clinically and radiologically mistaken for meningioma because of its focal dural based aspect...
September 21, 2019: World Neurosurgery
Ishwor Gurung, Yan Gao, Kai Han, Xue-Biao Peng
Langerhans cell histiocytosis (LCH) is a proliferative disease commonly seen in the pediatric population but rarely encountered in the adult population. The exact etiology remains unclear. It has various clinical features and is very likely to be misdiagnosed. Histopathology and immunohistochemistry are very important for the diagnosis of LCH. Treatment protocols remain controversial. Herein, we report a rare adult onset LCH, which is confined to the skin. A 50-year-old Chinese man presented with a nodule with itchy rashes on the left lower leg, which gradually grew in size for the last 6 months...
September 2019: Indian Journal of Dermatology
E Boyuk, I Bulur, F Canaz, O Cilingir
No abstract text is available yet for this article.
September 14, 2019: Actas Dermo-sifiliográficas
David S Nelson, Ryan L Marano, Yechaan Joo, Sara Y Tian, Bhumi Patel, Daniel H Kaplan, Mark J Shlomchik, Kristen Stevenson, Roderick T Bronson, Barrett J Rollins
Langerhans cell histiocytosis (LCH) is characterized by the accumulation of Langerin (CD207)-expressing histiocytes. Mutational activation of mitogen-activated protein kinase pathway genes, in particular BRAF, drives most cases. To test whether activated BRAF is sufficient for the development of LCH, we engineered mice to express BRAF V600E under the control of the human Langerin promoter. These mice have shortened survivals, smaller lymphoid organs, absent Leydig cells, and fewer epidermal LCs than controls, but do not accumulate histiocytes...
2019: PloS One
Meghana Prabhu, Shobhana Raju, Dhritiman Chakraborty, Saurabh Arora, Rakesh Kumar
Bilateral lung parenchymal involvement is seen in infective as well as noninfective conditions, appearing as focal or diffuse lung disease. PET/CT with FDG helps in characterization (increased glucose utilization is seen by both inflammatory and neoplastic cells). In this article, we describe the spectrum of patterns of FDG uptake and associated CT changes involving bilateral lung parenchyma. Benign conditions described are aspiration pneumonia; pulmonary toxicity by bleomycin; infections, namely, sarcoidosis, miliary pulmonary tuberculosis, and pulmonary nocardiosis; and inflammatory conditions such as pulmonary Langerhans cell histiocytosis and pulmonary alveolar proteinosis...
September 12, 2019: Clinical Nuclear Medicine
Jean Donadieu, Islam Amine Larabi, Mathilde Tardieu, Johannes Visser, Caroline Hutter, Elena Sieni, Nabil Kabbara, Mohamed Barkaoui, Jean Miron, François Chalard, Paul Milne, Julien Haroche, Fleur Cohen, Zofia Hélias-Rodzewicz, Nicolas Simon, Mathilde Jehanne, Alexandra Kolenova, Anne Pagnier, Nathalie Aladjidi, Pascale Schneider, Geneviève Plat, Anne Lutun, Anne Sonntagbauer, Thomas Lehrnbecher, Alina Ferster, Viktoria Efremova, Martina Ahlmann, Laurence Blanc, James Nicholson, Anne Lambilliote, Houda Boudiaf, Andrej Lissat, Karel Svojgr, Fanette Bernard, Sarah Elitzur, Michal Golan, Dmitriy Evseev, Michael Maschan, Ahmed Idbaih, Olga Slater, Milen Minkov, Valerie Taly, Matthew Collin, Jean-Claude Alvarez, Jean-François Emile, Sébastien Héritier
PURPOSE: Off-label use of vemurafenib (VMF) to treat BRAFV600E mutation-positive, refractory, childhood Langerhans cell histiocytosis (LCH) was evaluated. PATIENTS AND METHODS: Fifty-four patients from 12 countries took VMF 20 mg/kg/d. They were classified according to risk organ involvement: liver, spleen, and/or blood cytopenia. The main evaluation criteria were adverse events (Common Terminology Criteria for Adverse Events [version 4.3]) and therapeutic responses according to Disease Activity Score...
September 12, 2019: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
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