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Langerhans histiocytosis

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https://www.readbyqxmd.com/read/28944988/evaluation-and-treatment-of-langerhans-cell-histiocytosis-patients-with-central-nervous-system-abnormalities-current-views-and-new-vistas
#1
REVIEW
E Ann Yeh, Jay Greenberg, Oussama Abla, Giulia Longoni, Eli Diamond, Michelle Hermiston, Brandon Tran, Carlos Rodriguez-Galindo, Carl E Allen, Kenneth L McClain
Central nervous system (CNS) involvement in Langerhans cell histiocytosis (LCH) can include mass lesions of the hypothalamic pituitary axis, choroid plexus, cerebrum, and cerebellum or magnetic resonance imaging (MRI) signal abnormalities of the cerebellum, pons, and basal ganglia. The term neurodegenerative (ND) CNS-LCH has been given to the MRI signal abnormalities and neurologic dysfunction, although initially patients may have no clinical symptoms. Standardized evaluations to better understand the natural history and response to therapy are needed...
September 25, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28942734/adult-langerhans-cell-histiocytosis-with-pulmonary-and-colorectoanal-involvement-a-case-report
#2
Mohamad Jihad Mansour, Elias Mokbel, Eddy Fares, Janah Maddah, Fadi Nasr
BACKGROUND: Langerhans cell histiocytosis is a rare systemic disease characterized by the abnormal overproduction of histiocytes that tend to infiltrate single or multiple organ systems leading to significant tissue damage. It mainly affects - by order of decreasing frequency - the bone, the skin, the lymph nodes, the liver, and lungs. Gastrointestinal tract involvement is extremely rare in adults. CASE PRESENTATION: We describe the case of a 32-year-old Middle Eastern man with Langerhans cell histiocytosis involving his lungs and the colorectoanal part of his gastrointestinal tract, with complete resolution of gastrointestinal tract lesions following a non-standardized chemotherapy regimen...
September 25, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28940244/demodicosis-in-two-patients-with-a-previous-history-of-langerhans-cell-histiocytosis
#3
Marcial Álvarez-Salafranca, Asunción Vicente, Carolina Prat Torres, Andrea Combalia, Manuel Monsonís, Veronica Paola Celis-Passini, María Antonia González-Enseñat
Demodex mites are commensal organisms rarely found in healthy children. Human demodicosis can be classified as a primary or a secondary form. The secondary form in children usually affects severely immunodepressed children. To our knowledge, this is the first report of human demodicosis associated with Langerhans cell histiocytosis. These cases show that this skin disorder can occur months after completing chemotherapy, without recurrence of the systemic disease.
September 21, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28937439/langerhans-cell-histiocytosis-with-clinical-and-histologic-features-of-hidradenitis-suppurativa-brief-report-and-review
#4
Jessica E Kalen, Divya Shokeen, Mona Mislankar, Michael Wangia, Kiran Motaparthi
Langerhans cell histiocytosis (LCH) is an uncommon histiocytic disorder in adults. Clinically, this rare entity can mimic other dermatologic conditions, including hidradenitis suppurativa. A case of LCH is reported with clinical and histologic features of hidradenitis suppurativa, along with a review of these unusual findings. Clinical dermatologists and dermatopathologists benefit from awareness of this unique presentation, which may prompt earlier identification and diagnosis of adult patients with LCH.
September 11, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28932806/langerhans-cell-histiocytosis-limited-to-the-female-genital-tract-a-review-of-literature-with-three-additional-cases
#5
Rebekah Wieland, Jenna Flanagan, Elise Everett, Sharon Mount
•LCH of the female reproductive tract has four patterns of involvement.•A comprehensive literature review revealed 35 cases of pure genital LCH.•We report two new cases of pure LCH lesions of the vulva and one of the cervix.•Treatment of LCH varies and there is no standard for pure genital involvement.•Prognosis of LCH confined to the gynecologic tract appears to be favorable.
November 2017: Gynecologic Oncology Reports
https://www.readbyqxmd.com/read/28927957/clinical-course-of-the-bony-lesion-of-single-system-single-site-langerhans-cell-histiocytosis-is-appropriate-follow-up-sufficient-treatment
#6
Hiromi Sasaki, Satoshi Nagano, Hirofumi Shimada, Shunsuke Nakamura, Takao Setoguchi, Setsuro Komiya
BACKGROUND: Langerhans cell histiocytosis (LCH) is categorized into three types, which include single-system single-site (SS-s), single-system multiple-site (SS-m) and multisystem (MS). The most commonly affected site in LCH is bone, and the bony lesion of SS-s LCH has a good prognosis. The bony lesion of SS-s LCH has been thought to regress spontaneously. Although treatments such as curettage, direct injection of corticosteroids, and chemotherapy have been performed, regular follow-up is the first line of treatment for the bony lesion of SS-s LCH...
September 16, 2017: Journal of Orthopaedic Science: Official Journal of the Japanese Orthopaedic Association
https://www.readbyqxmd.com/read/28903440/evaluation-of-pituitary-uptake-incidentally-identified-on-18-f-fdg-pet-ct-scan
#7
Huijun Ju, Jinxin Zhou, Yu Pan, Jing Lv, Yifan Zhang
The clinical significance of pituitary uptake on routine whole body (18)F-fluorodeoxyglucose (FDG) positron emission tomography/computer tomography (PET/CT) is not completely characterized. We seek to assess the potential differential diagnosis/underlying etiology of pituitary FDG uptake incidentally identified on routine PET/CT scans. A total of 24,007 PET/CT whole body scans in recent 5 years were retrospectively reviewed. Patients with maximum standardized uptake value (SUVmax) > 4.1 in the pituitary glands were identified...
August 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28880370/langerhans-cell-histiocytosis-in-an-adult-female-presenting-with-widespread-confluent-crusted-papules-and-review-of-adult-cases-confined-to-skin
#8
Shiyu Zhang, Yuehua Liu
No abstract text is available yet for this article.
September 7, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28877978/how-i-manage-pulmonary-langerhans-cell-histiocytosis
#9
Gwenaël Lorillon, Abdellatif Tazi
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare sporadic cystic lung disease of unknown aetiology that is characterised by the infiltration and destruction of the wall of distal bronchioles by CD1a(+) Langerhans-like cells. In adults, PLCH is frequently isolated and affects young smokers of both sexes. Recent multicentre studies have led to the more standardised management of patients in clinical practice. Smoking cessation is essential and is occasionally the only suitable intervention. Serial lung function testing is important because a significant proportion of patients may experience an early decline in forced expiratory volume in 1 s and develop airflow obstruction...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28868307/atlanto-axial-langerhans-cell-histiocytosis-in-a-child-presented-as-torticollis
#10
Miniar Tfifha, Mehdi Gaha, Nadia Mama, Mohamed Taher Yacoubi, Saoussen Abroug, Hela Jemni
Langerhans cell histiocytosis (LCH) is a rare condition mostly seen in children and adolescents. Eosinophilic granuloma (EG) is one of its three clinical entities and is considered as a benign osteolytic lesion. Many reports of patients with spine histiocytosis are well documented in the literature but it is not the case of atlantoaxial localization. We report here a new observation of atlantoaxial LCH in a 4-year-old boy revealed by persistent torticollis. He was successfully treated with systemic chemotherapy and surgery...
August 16, 2017: World Journal of Clinical Cases
https://www.readbyqxmd.com/read/28868020/erdheim-chester-disease-the-importance-of-information-integration
#11
Anna Nikonova, Khashayar Esfahani, Guillaume Chausse, Stephan Probst, Tina Petrogiannis-Haliotis, Hans Knecht, Genevieve Gyger
BACKGROUND: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis disorder that utilizes the RAS-RAF-MEK-ERK pathway. It has a highly variable clinical presentation, where virtually any organ can be involved, thus having the potential of posing a great diagnostic challenge. Over half of the reported cases have the BRAF V600E mutation and have shown a remarkable response to vemurafenib. CASE PRESENTATION: We describe herein a patient with a history of stroke-like symptoms and retroperitoneal fibrosis that on initial pathology raised the possibility of IgG4-related disease...
May 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28864216/langerhans-cell-histiocytosis-mimicking-periapical-pathology-in-a-39-year-old-man
#12
Scott M Peters, Julie Pastagia, Angela J Yoon, Elizabeth M Philipone
Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation of Langerhans-type dendritic cells, with more than 50% of cases of LCH seen in children younger than 15 years of age. The most common clinical presentation of LCH is solitary or multiple bony lesions. The jaws are affected in approximately 10%-20% of cases, with a strong predilection for the mandible. The maxilla is involved in only 1% of head and neck cases. When the jaws are involved, lesions of LCH may mimic periapical pathology as seen in patients requiring endodontic therapy or bone loss as seen in periodontal disease...
August 29, 2017: Journal of Endodontics
https://www.readbyqxmd.com/read/28859038/refractory-lch-with-secondary-intracranial-pnet-a-case-report-and-review-of-literature
#13
Varsha Chayapathi, Amita Mahajan, Manas Kalra
The occurrence of second malignant neoplasms in patients with Langerhans cell histiocytosis is infrequent but has been reported. Here we report the case of a child with refractory Langerhans cell histiocytosis who was treated with cladribine and later developed a secondary intracranial primitive neuroectodermal tumor. The possible association of cladribine with second neoplasm is further discussed.
August 30, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28858125/langerhans-cell-histiocytosis-of-the-thyroid-complicated-by-papillary-thyroid-carcinoma-a-case-report-and-brief-literature-review
#14
REVIEW
Xin Wu, Shi Chen, Li-Yang Zhang, Ya-Ping Luo, Ying Jiang, Rui-E Feng
RATIONALE: Langerhans cell histiocytosis (LCH) involves mainly the skin and bone and rarely the thyroid. Meanwhile, papillary thyroid carcinoma (PTC) is the most common subtype of thyroid cancer. Both LCH and PTC could make the thyroid enlarged and hypermetabolic. The coincidence of these 2 events in a patient is rare, and this paper aimed to report such case. PATIENT CONCERNS: A 40-year-old man presented with polyuria and polydipsia for 5 years. The symptoms had been relieved well by drug therapy for >4 years, until the drugs could not control the symptoms anymore and an extensively enlarged thyroid gland was noticed...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28836893/systemic-juvenile-xanthogranuloma-involving-the-bone-marrow-multiple-bones-and-the-skin-that-developed-during-treatment-of-acute-lymphoblastic-leukemia-in-remission-state
#15
Eunjae Cheon, Saemi Yang, Jae Ho Han, Kwang Chul Lee, Jun Eun Park
Juvenile xanthogranuloma (JXG) is a rare benign disorder classified as non-Langerhans cell histiocytosis, with unclear etiology and pathogenesis. JXG is generally characterized by solitary or multiple cutaneous nodules that resolve spontaneously over a few years. JXG rarely presents as extracutaneous lesions that progress to a symptomatic systemic disorder through multiple organ involvement. We encountered a systemic JXG case involving the bone marrow, multiple bones, and the skin during acute lymphoblastic leukemia treatment...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28834636/intraosseous-rosai-dorfman-disease-diagnosed-by-touch-imprint-cytology-evaluation-a-case-series
#16
Avani A Pendse, Sara E Wobker, Kevin G Greene, Scott V Smith, Robert J Esther, Leslie G Dodd
Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD) is a rare benign disorder that primarily affects the lymph nodes. Localized lymphadenopathy is the most common clinical manifestation of this disorder. However, RDD has been described in several extra-nodal sites including the head and neck region, soft tissue, skin, upper respiratory tract, gastro-intestinal tract and central nervous system (CNS). Involvement of the bone is considered very rare, occurring in less than 10% patients...
August 23, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/28825606/cutaneous-indeterminate-cell-histiocytosis-of-donor-origin-after-allogeneic-hematopoietic-stem-cell-transplantation
#17
Angel Santos-Briz, Concepción Román, Rocío Corral, Alvaro de Dios, Lourdes Vázquez, María D Ludeña
Allogeneic hematopoietic stem-cell transplantation and solid-organ transplantation are associated with an increased risk of secondary neoplasms. Indeterminate cell histiocytosis (ICH) is a rare disease composed of so-called indeterminate cells, an alleged cutaneous dendritic cell subset displaying histological and some ultrastructural and immunophenotypic features of Langerhans cells but lacking Birbeck granules. We report a case of cutaneous ICH occurring after allogeneic hematopoietic stem-cell transplantation for a myelodysplastic syndrome in a 56-year-old man...
September 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28802389/cholesteatoma-as-a-complication-of-langerhans-cell-histiocytosis-of-the-temporal-bone-a-nationwide-cross-sectional-analysis
#18
Jonathan C Simmonds, Mark Vecchiotti
OBJECTIVE: To determine if patients with Langerhans Cell Histiocytosis (LCH) of the temporal bone have a higher risk of developing cholesteatoma. METHODS: Review of literature and cross-sectional weighted analysis of patients under 19 with a diagnosis of LCH from the National Inpatient Sample (NIS) and Kids' Inpatient Database (KID) from 2000 to 2013. ICD-9 codes and demographics were analyzed; pairwise comparisons and multivariate analyses were performed. RESULTS: Only seven cases of cholesteatoma after the treatment for LCH of the temporal bone have been documented in the literature...
September 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28797508/hematopoietic-tumors-primarily-presenting-in-bone
#19
REVIEW
Arjen H G Cleven, Pancras C W Hogendoorn
Hematologic neoplasms that primarily present in bone are rare; this article describes the most common examples of hematologic tumors primarily presenting in bone, including plasma cell myeloma, solitary plasmacytoma of bone, primary non-Hodgkin lymphoma of bone, acute lymphoblastic leukemia/lymphoma, and Langerhans cell histiocytosis. The macroscopic and microscopic features, differential diagnosis, diagnostic workup, and prognosis of all these different entities are discussed, with special emphasis on common differential diagnosis...
September 2017: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/28794143/differential-diagnosis-of-granulomatous-lung-disease-clues-and-pitfalls-number-4-in-the-series-pathology-for-the-clinician-edited-by-peter-dorfm%C3%A3-ller-and-alberto-cavazza
#20
Shinichiro Ohshimo, Josune Guzman, Ulrich Costabel, Francesco Bonella
Granulomatous lung diseases are a heterogeneous group of disorders that have a wide spectrum of pathologies with variable clinical manifestations and outcomes. Precise clinical evaluation, laboratory testing, pulmonary function testing, radiological imaging including high-resolution computed tomography and often histopathological assessment contribute to make a confident diagnosis of granulomatous lung diseases. Differential diagnosis is challenging, and includes both infectious (mycobacteria and fungi) and noninfectious lung diseases (sarcoidosis, necrotising sarcoid granulomatosis, hypersensitivity pneumonitis, hot tub lung, berylliosis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, rheumatoid nodules, talc granulomatosis, Langerhans cell histiocytosis and bronchocentric granulomatosis)...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
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