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Langerhans cell histiocytosis

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https://www.readbyqxmd.com/read/28538782/diffuse-cystic-lung-diseases-differential-diagnosis
#1
Bruno Guedes Baldi, Carlos Roberto Ribeiro Carvalho, Olívia Meira Dias, Edson Marchiori, Bruno Hochhegger
Diffuse cystic lung diseases are characterized by cysts in more than one lung lobe, the cysts originating from various mechanisms, including the expansion of the distal airspaces due to airway obstruction, necrosis of the airway walls, and parenchymal destruction. The progression of these diseases is variable. One essential tool in the evaluation of these diseases is HRCT, because it improves the characterization of pulmonary cysts (including their distribution, size, and length) and the evaluation of the regularity of the cyst wall, as well as the identification of associated pulmonary and extrapulmonary lesions...
March 2017: Jornal Brasileiro de Pneumologia: Publicaça̋o Oficial da Sociedade Brasileira de Pneumologia e Tisilogia
https://www.readbyqxmd.com/read/28533655/-18-f-fdg-pet-ct-in-localizing-additional-cns-lesion-in-a-case-of-langerhans-cell-histiocytosis-determining-accurate-extent-of-the-disease
#2
Shamim Ahmed Shamim, Sarthak Tripathy, Anirban Mukherjee, Chandrasekhar Bal, Madhavi Tripathi
Central nervous system involvement is a rare manifestation of Langerhans cell histiocytosis (LCH), with bone and skin lesions being more frequent. MR remains the investigation of choice for localizing brain lesions. However, due to poor sensitivity of MRI in detecting osseous and pulmonary lesions, it is not used routinely in staging purposes until and unless indicated. We hereby discuss a case of 6-year-old boy of LCH who was referred for 18-F-FDG PET/CT for staging and knowing the extent of the disease, but a lesion in hypothalamus was picked up incidentally on FDG PET-CT study that was confirmed by MRI...
April 2017: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
https://www.readbyqxmd.com/read/28532436/vinblastine-chemotherapy-in-adult-patients-with-langerhans-cell-histiocytosis-a-multicenter-retrospective-study
#3
Abdellatif Tazi, Gwenaël Lorillon, Julien Haroche, Antoine Neel, Stéphane Dominique, Achille Aouba, Jean-David Bouaziz, Constance de Margerie-Melon, Emmanuelle Bugnet, Vincent Cottin, Thibault Comont, Christian Lavigne, Jean-Emmanuel Kahn, Jean Donadieu, Sylvie Chevret
BACKGROUND: Vinblastine is the standard treatment for children with Langerhans cell histiocytosis (LCH). Whether this treatment could be extended to adults with LCH is questionable. This retrospective multicenter study included 35 adult patients (median age 33 years; 23 men; 80% with multisystem LCH) who were treated with vinblastine + steroids as a first-line chemotherapy and followed for a median time of 83 months. The objectives were to determine the overall response rate (based on the Histiocyte Society criteria), disease reactivation rate, toxicity, permanent consequences, and survival rate corresponding to this treatment...
May 22, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28521873/low-periostin-levels-in-adult-patients-with-langerhans-cell-histiocytosis-are-independently-associated-with-the-disease-activity
#4
Athanasios D Anastasilakis, Stergios A Polyzos, Marina Tsoli, Athanasios Papatheodorou, Panagiotis Kokkoris, Gregory Kaltsas, Evangelos Terpos, Polyzois Makras
PURPOSE: Langerhans cell histiocytosis (LCH) is a rare proliferative disease of cells of the CD1a+/CD207+ myeloid dendritic cell lineage that may infiltrate one or more organs or systems at all ages. We aimed to evaluate periostin and sclerostin serum levels in adult patients with LCH. PROCEDURES: This was a cross-sectional study comparing 38 adult patients with LCH with 38 age- and sex-matched healthy controls. Serum periostin and sclerostin levels were measured to compare between LCH patients and controls as well as between patients with active and non-active disease...
June 2017: Metabolism: Clinical and Experimental
https://www.readbyqxmd.com/read/28520453/-indeterminate-cell-histiocytosis-disappearance-of-skin-infiltration-following-electron-beam-therapy-and-an-application-of-2-chlorodeoxyadenosine-case-report
#5
Zdeněk Adam, Marta Ježová, Pavel Šlampa, Leoš Křen, Vladimír Vašků, Renata Koukalová, Zdeněk Řehák, Luděk Pour, Marta Krejčí, Zdeněk Král, Jiří Mayer
Indeterminate cell histiocytosis is a rare disease belonging to the group of malignant histiocytic diseases. The disease predominantly affects the skin. The disease appeared in the described patient at the age of 80 years. Morphs began to develop on the skin and rapidly spread over the whole body including the face. Only the hands and feet were left uncovered. The patients skin samples were taken from 2 sites for histological examination. The resulting conclusion was indeterminate cell histiocytosis. The treatment we chose was analogous to the procedures for Langerhans cell histiocytosis...
2017: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/28516403/long-term-clinical-outcome-of-spinal-langerhans-cell-histiocytosis-in-children
#6
Seong Wook Lee, Hyery Kim, Jin Kyung Suh, Kyung-Nam Koh, Ho Joon Im, Hee Mang Yoon, Jong Jin Seo
Spinal involvement of Langerhans cell histiocytosis (LCH) affects morbidity, but outcomes are not well understood. We analyzed long-term outcomes following uniform treatment at a single institution. Clinical characteristics and outcomes of spinal LCH patients were retrospectively analyzed. Height ratios were calculated using the anterior height of the involved vertebral body on magnetic resonance imaging (MRI) and the expected normal vertebral height. Twenty-two (22.4%) of 98 patients diagnosed with LCH had spinal involvement...
May 17, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28514411/-langerhans-cell-histiocytosis-skin-diseases-and-visceral-lesions
#7
V D Elkin, T G Sedova, E A Kopytova, E V Plotnikova
Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder that belongs to class I histiocytoses and is characterized by infiltration of one or many organs by Langerhans cells to form granulomas. The literature analysis could identify a lot of etiological, pathogenetic, and trigger factors and mechanisms for LCH development, which determine the diversity of the clinical picture and course of the disease. The clinical manifestations of LCH are very variable and depend on the severity of lesions and the age of patients...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28512190/hematopoietic-origin-of-langerhans-cell-histiocytosis-and-erdheim-chester-disease-in-adults
#8
Paul Milne, Venetia Bigley, Chris M Bacon, Antoine Néel, Naomi McGovern, Simon Bomken, Muzlifah Haniffa, Eli L Diamond, Benjamin H Durham, Johannes Visser, David Hunt, Harsha Gunawardena, Mac Macheta, Kenneth L McClain, Carl Allen, Omar Abdel-Wahab, Matthew Collin
Langerhans cell histiocytosis (LCH) and Erdheim Chester Disease (ECD) are rare histiocytic disorders induced by somatic mutation of MAP kinase pathway genes. BRAF(V600E) mutation is the most common mutation in both conditions and also occurs in the hematopoietic neoplasm hairy cell leukemia (HCL). It is not known if adult LCH or ECD arise from hematopoietic stem cells (HSC) nor which potential blood borne precursors lead to the formation of histiocytic lesions. In this study, BRAF V600E allele-specific PCR was used to map the neoplastic clone in 20 adults with LCH ECD and HCL...
May 16, 2017: Blood
https://www.readbyqxmd.com/read/28504206/braf-v600e-mutation-in-pediatric-intracranial-and-cranial-juvenile-xanthogranuloma
#9
Piti Techavichit, Darintr Sosothikul, Thiamjit Chaichana, Chinachote Teerapakpinyo, Paul Scott Thorner, Shanop Shuangshoti
Juvenile xanthogranuloma (JXG) is a cutaneous form of non-Langerhans cell histiocytosis (LCH), primarily affecting children. The lesion is presumed to originate from either macrophages or dermal dendritic cells. JXG can rarely present as an isolated intracranial lesion and, in contrast to the dismal outcome of patients with systemic disease, cranial JXG has been shown to carry a more favorable prognosis. Here, we report for the first time 3 pediatric cases of JXG with a BRAF V600E mutation, 2 with intracranial lesions and one with cranial lesions...
May 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/28500693/the-clinical-spectrum-of-xanthomatous-lesions-of-the-eyelids
#10
REVIEW
Can Baykal, Algun Polat Ekinci, Kurtulus D Yazganoglu, Nesimi Buyukbabani
Yellowish papules, nodules, or plaques, namely "xanthomatous" lesions, may be seen on the eyelids in the course of various disorders. The prototype is "xanthelasma palpebrarum" (XP) that is localized only to the eyelids and may be associated with hyperlipidemia. On the other hand, different types of normolipemic disorders may also cause xanthomatous eyelid lesions. Among these, Langerhans cell histiocytosis, diffuse normolipemic xanthoma, and non-Langerhans cell histiocytoses (papular xanthoma, juvenile xanthogranuloma, xanthoma disseminatum, adult-onset xanthogranuloma, adult-onset asthma and periocular xanthogranuloma, necrobiotic xanthogranuloma, Erdheim-Chester disease, Rosai-Dorfman disease, and reticulohistiocytosis) can be listed...
May 13, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28492172/fingernail-involvement-in-adult-langerhans-cell-histiocytosis
#11
Takahiro Ishikawa, Aya Nishizawa, Yuiko Nagata, Takahiro Satoh
No abstract text is available yet for this article.
May 9, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28490189/langerhans-cell-histiocytosis-eosinophilic-granuloma-of-the-skull-mimicking-nummular-headache-report-of-two-cases
#12
Carlos Silva Rosas, Heather Angus-Leppan, Melchor Bruno Lemp, Juan Pablo Rozas, Alonso Humberto Quijada
Background Nummular headache is a rare, recently described topographic headache defined by the circumscribed coin-shaped area of pain. It is classified as a primary headache. There is debate about whether it is due to a peripheral or central disturbance, and its relationship to migraine. Case reports We report two patients with presumed nummular headache secondary to Langerhans cell histiocytosis, both with resolution of their headaches after surgical resection. Conclusion Imaging in patients with clinical features of nummular headache is recommended, as this and other cases highlight that it may be symptomatic...
January 1, 2017: Cephalalgia: An International Journal of Headache
https://www.readbyqxmd.com/read/28483018/clinical-analysis-of-122-cases-of-langerhans-cell-histiocytosis
#13
Tao Wang, Beibei Su, Dawei Han, Anli Tong, Jian Li, Yuehua Liu, Hongzhong Jin, Kai Fang
Objective To investigate the features of Langerhans cell histiocytosis (LCH). <b>Method</b> Skin lesions,systemic involvement,imaging characteristics,laboratory tests,immunophenotying,treatment response,and survival of 122 LCH patients treated at our center from February 1983 to August 2013 were retrospectively analyzed. Results LCH was associated with diverse skin lesions. Lung was the most involved organ,followed by bone,skin,lymph nodes,liver,spleen,oral cavity,and thyroid. Multisystem LCH was more common than single-system LCH...
April 20, 2017: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/28482919/langerhans-cell-histiocytosis-of-the-maxillae-in-a-child-treated-only-with-chemotherapy-a-case-report
#14
Angela Pia Cazzolla, Giuseppe Troiano, Khrystyna Zhurakivska, Eugenio Maiorano, Gianfranco Favia, Maria Grazia Lacaita, Giuseppe Marzo, Franca Dicuonzo, Stefano Andresciani, Lorenzo Lo Muzio
BACKGROUND: Langerhans cell histiocytosis is a sporadic disease caused by an uncontrolled pathogenic clonal proliferation of dendritic cells that have Langerhans cell characteristics. New treatment protocols provided by the HISTSOC-LCH-III (NCT00276757) trial show an improvement in the survival of children with langerhans cell histiocytosis. CASE PRESENTATION: We report a case of Langerhans cell histiocytosis, which presented as an osteolytic lesion of the left pre-maxillae enclosing the deciduous incisor and canine in a 7-month-old white Italian boy...
May 9, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28479703/a-rare-and-unusual-case-report-of-langerhans-cell-histiocytosis
#15
Dhanu G Rao, Malay Vishnuprasad Trivedi, Raghavendra Havale, S P Shrutha
Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, is an uncommon hematological disorder affecting infants and young children. It is the condition characterized by uncontrolled stimulation and proliferation of normal antigen presenting cells, Langerhans cells. Because of its relatively low incidence, limited data are available regarding the epidemiology of LCH, with estimation of 2-5 cases per million inhabitants per year. The purpose of this report is to describe the case of LCH in the 3-year-old male child with multiple focal involvements of bones and to discuss clinical, radiological and histopathological features of LCH and role of the dental surgeon in diagnosing and managing such lesions...
January 2017: Journal of Oral and Maxillofacial Pathology: JOMFP
https://www.readbyqxmd.com/read/28478970/langerhans-cell-histiocytosis-presenting-as-a-blueberry-muffin-rash
#16
Adam R Schmitt, David A Wetter, Michael J Camilleri, Shakila P Khan, Megha M Tollefson
No abstract text is available yet for this article.
May 4, 2017: Lancet
https://www.readbyqxmd.com/read/28466548/novel-clinical-observations-on-benign-cephalic-histiocytosis-in-a-large-series
#17
Algün Polat Ekinci, Nesimi Buyukbabani, Can Baykal
BACKGROUND/OBJECTIVES: Benign cephalic histiocytosis (BCH) is a rare form of non-Langerhans cell histiocytosis in infants and young children characterized by self-healing macules and papules occurring primarily in the head and neck region. So far there have been nearly 60 reported cases in the English-language literature. METHODS: In this retrospective study, we evaluated clinical features and follow-up data of 11 patients diagnosed with BCH between 2004 and 2016 in the Department of Dermatovenereology, Istanbul Medical Faculty...
May 3, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28458889/an-unusual-case-of-adult-onset-multi-systemic-langerhans-cell-histiocytosis-with-perianal-and-incident-thyroid-involvement
#18
Ozen Oz Gul, Pinar Sisman, Soner Cander, Erdem Gozden, Meral Kurt, Ozlem Saraydaroglu, Turkay Kirdak, Canan Ersoy, Erdinc Erturk
SUMMARY: Langerhans cell histiocytosis (LCH) is a rare sporadic disease characterized by histiocytic neoplastic infiltration of various organ systems and a wide spectrum of clinical manifestations, ranging from benign and self-limiting to lethal. Herein, we report a rare case of adult-onset multi-systemic LCH in a 36-year-old male patient with an initial perianal presentation and incidental finding of subsequent thyroid gland involvement in the follow-up period. The patient with a history of perianal LCH treated with surgical excision and local radiotherapy was referred to our Endocrinology Department upon detection of hypermetabolic nodular lesions in the left lateral lobe of thyroid gland on positron emission tomography-computed tomography (PET/CT) scan in the nineth month of follow-up...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28452094/imaging-characteristics-of-liver-langerhans-cell-histiocytosis-in-children
#19
Hui Liu, Xueying Long, Xiaoyi Wang, Wenzheng Li, Yigang Pei
BACKGROUND: Hepatic involvement of Langerhans cell histiocytosis (LCH) in children is important not only for prognostic purposes, but also as a guide for therapeutic strategies. The purpose of this study was to evaluate the imaging characteristics of hepatic LCH by computed tomography (CT) and magnetic resonance imaging (MRI) in children. METHODS: Imaging features of 17 children with hepatic LCH diagnosed by physical examination, laboratory and histopathological tests were analyzed retrospectively...
April 28, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28450940/langerhans-cell-histiocytosis-of-the-temporal-bone-a-case-report
#20
Maomei Ni, Xiuhai Yang
Langerhansmporal histiocytosis (LCH) refers to a group of diseases that are characterized by the primary pathogenesis of an abnormal polyclonal proliferation of Langerhans cells that affect different structures of the human body, including the temporal bone. Thus far, the etiology of LCH remains unclear. Diagnosis of LCH is based on a synthetic analysis of the clinical presentations, as well as on features of imaging and histopathology. The clinical features, diagnosis, treatment and prognosis of LCH remain obscure, and temporal LCH is often confused with ear inflammatory lesions and malignant tumors...
March 2017: Experimental and Therapeutic Medicine
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