keyword
https://read.qxmd.com/read/38478667/letterer-siwe-disease-presenting-with-gastrointestinal-and-cutaneous-manifestations
#1
JOURNAL ARTICLE
Antonina Sergeevna Stadnikova, Walaa Fadhil Abbas, Olga Borisovna Tamrazova, Ekaterina Andreevna Pristanskova, Irina Nikolaevna Zakharova, Irina Vladimirovna Berezhnaya, Dmitry Alexandrovich Skobeev, Lyudmila Viktorovna Goncharova
Histiocytosis is a set of distinct proliferative illnesses defined by the proliferation and infiltration of varied numbers of dendritic cells, macrophages, and monocytes in the afflicted tissues. The skin and other organs may be impacted by the inflammatory infiltration. It can occur at any age. The severity of the symptoms can range from mild to severe, depending on the degree and type of organ involvement. Although certain forms of histiocytosis can be fatal, others can be treated successfully without sequelae...
December 15, 2023: Dermatology Online Journal
https://read.qxmd.com/read/38472383/-xanthogranulomatous-adrenalitis-a%C3%A2-rare-and-difficult-differential-diagnosis-of-adrenal-gland-tumors
#2
JOURNAL ARTICLE
Wolfgang Saeger, Andreas M Luebke, S T Mekoula, Jörg-Michael Pahnke
A radiologically diagnosed tumor in a 29-year-old woman with a fever of around 39 °C was operated on under the suspicion of cholecystitis or a liver abscess. A solid tumor was found in the adrenal gland and resected. The frozen section findings did not reveal a clear diagnosis of entity and assignment. Histologically, the tumor was found to consist of densely clustered large histiocyte-like cells with expression of vimentin, CD68, and CD163 as well as negativity for keratin, langerin, and SMA...
March 12, 2024: Pathologie (Heidelb)
https://read.qxmd.com/read/38461672/isolated-rosai-dorfman-disease-of-the-spine-a-systematic-literature-review
#3
REVIEW
Maroua Slouma, Sirine Bouzid, Karima Tlili, Dahmani Yedaes, Khaled Radhwen, Imen Gharsallah
INTRODUCTION: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis involving the central nervous system in 5% of cases. Spinal location occurs in less than 1% of extranodal RDD and can be responsible for neurological manifestations. We present a systematic review of cases of isolated spinal RDD. We also report a new case of isolated spinal RDD revealed by spinal cord compression. MATERIALS AND METHODS: The systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guideline using the MEDLINE and SCOPUS databases and included case reports and case series describing isolated RDD of the spine...
February 29, 2024: Clinical Neurology and Neurosurgery
https://read.qxmd.com/read/38459977/-68-ga-ga-fapi-versus-18-f-fdg-pet-ct-in-the-evaluation-of-langerhans-cell-histiocytosis
#4
JOURNAL ARTICLE
Linlin Guo, Guohua Shen
No abstract text is available yet for this article.
March 9, 2024: European Journal of Nuclear Medicine and Molecular Imaging
https://read.qxmd.com/read/38444598/from-terminal-ileum-to-terminal-diagnosis-the-critical-role-of-terminal-ileum-intubation-in-diagnosing-langerhans-cell-histiocytosis-in-a-patient-with-tar-syndrome
#5
Mena Louis, Chad Copper, Elaine Lelli, Joseph Conway, Daniel Sarmiento, Hardeep Singh
The co-occurrence of Thrombocytopenia with Absent Radius (TAR) syndrome and Langerhans Cell Histiocytosis (LCH) is exceedingly rare, with scant documentation in existing medical literature. This case report aims to shed light on this unique intersection of conditions, emphasizing the diagnostic and therapeutic challenges it presents. A 27-year-old female with a history of TAR syndrome presented with microcytic anemia, hip pain, and gastrointestinal symptoms. Terminal ileum intubation during colonoscopy revealed superficial ulcerations, leading to a biopsy that confirmed LCH...
May 2024: Radiology Case Reports
https://read.qxmd.com/read/38440436/rosai-dorfmann-disease-a-rare-disease-presenting-as-a-unilateral-neck-swelling
#6
JOURNAL ARTICLE
Stanley John, Tanubha Goel
Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder of unknown etiology. Usually it presents with massive painless cervical lymph node enlargement. Histologically, it shows proliferation of distinctive histiocytic cells that demonstrate emperipolesis in the background of a mixed inflammatory infiltrates. Immunohistochemically, the cells are positive for markers such as CD68 and S100. A 14-year-old boy presented with painless right sided cervical lymphadenopathy without any systemic and other ear, nose, and throat manifestations...
February 2024: Indian Journal of Otolaryngology and Head and Neck Surgery
https://read.qxmd.com/read/38427961/typical-bone-scintigraphy-presentation-of-erdheim-chester-disease-in-a-patient-diagnosed-with-igg4-related-disease
#7
JOURNAL ARTICLE
Chloé François, Clémentine Rousselin, Thomas Quemeneur, Franck Demailly, Guillaume Collet, Alexandre Fagart
A 50-year-old woman presented a dry syndrome, joint pain, inflammatory syndrome, polyclonal hypergammaglobulinemia, and tubulointerstitial nephritis. Imaging studies (including FDG PET/CT) revealed infrarenal retroperitoneal fibrosis with periaortitis and hypermetabolic osteosclerotic lesions. Bone scintigraphy demonstrated intense uptake in the femoral, tibial, and radial regions, suggestive of non-Langerhans histiocytosis, specifically Erdheim-Chester disease. A bone biopsy confirmed the presence of IgG4-positive plasma cells but no histiocytes...
April 1, 2024: Clinical Nuclear Medicine
https://read.qxmd.com/read/38425778/erdheim-chester-disease-with-tendon-and-muscle-involvement-reports-of-a-rare-presentation
#8
Mahshid Golagha, Fatemeh Dehghani Firouzabadi, Corina Millo, Moozhan Nikpanah, Mark A Ahlman, Rahul H Dave, Juvianee I Estrada-Veras, Kevin O'Brien, Ashkan A Malayeri
Erdheim-Chester disease (ECD) is a rare histiocytic disease that affects multiple systems in the body. While it typically targets long bones, cardiovascular structures, the retroperitoneum, and the central nervous system, reports of tendon and skeletal muscle involvement are scarce. This review presents 2 cases: a case of ECD involving the left Achilles tendon and left abductor hallucis, as well as an unusual manifestation of ECD in the thigh musculature. In Case 1, studies involved a 39-year-old man who initially presented with bone and pituitary involvement...
May 2024: Radiology Case Reports
https://read.qxmd.com/read/38417992/imaging-of-fibro-osseous-lesions-and-other-bone-conditions-of-the-jaws
#9
REVIEW
Noura Alsufyani, Adel Alzahrani
This review directs the focus on the imaging features of various fibro-osseous lesions and other bone lesions that can be of similar presentation. Broad diagnosis of "fibrous osseous lesion" may culminate in improper treatment and management. Radiographic discriminating factors between these entities are highlighted and summarized to improve the diagnostic process when encountering these lesions.
April 2024: Dental Clinics of North America
https://read.qxmd.com/read/38415682/pediatric-neck-pain-of-a-10-year-old-child-with-cervical-spinal-tumor-evaluated-and-managed-in-direct-access-physical-therapy-a-case-report
#10
JOURNAL ARTICLE
Virginia K Henderson, Jean-Michel Brismée
INTRODUCTION: Differential diagnosis of pediatric neck pain requires age-appropriate communication and assessment tools. Recognizing these age-related nuances is critical, emphasizing the role of physical therapists in assessing and managing pediatric patients while ruling out severe pathologies. CASE DESCRIPTION: A 10-year-old male presented to physical therapy with a five-week history of increasing neck pain. A thorough history and segmental cervical examination considering the patient's age and development, led to patient referral to the emergency department...
February 28, 2024: Journal of Manual & Manipulative Therapy
https://read.qxmd.com/read/38414144/extensive-yellowish-masses-in-bilateral-orbit-and-neck
#11
Yinshan Zang, Lingxiao Xu, Ran Fan, Lu Cheng, Yan Xu
No abstract text is available yet for this article.
February 27, 2024: Journal of Dermatology
https://read.qxmd.com/read/38414140/gastrointestinal-langerhans-cell-histiocytosis-in-an-adult-with-anal-protrusion-and-multiple-colorectal-ulcers
#12
REVIEW
Q T Truong, Nhh Pham, T H Doan, V T Hoang, D T Hoang, C T Trinh, N T Tran
No abstract text is available yet for this article.
February 27, 2024: Journal of Gastroenterology and Hepatology
https://read.qxmd.com/read/38409747/clinical-characteristics-molecular-aberrations-treatments-and-outcomes-of-malignant-histiocytosis
#13
JOURNAL ARTICLE
Gordon J Ruan, Saurabh Zanwar, Aishwarya Ravindran, Susan Schram, Jithma P Abeykoon, Antonious Hazim, Jason R Young, Mithun V Shah, N Nora Bennani, Liuyan Jiang, Diana Morlote, Karen L Rech, Gaurav Goyal, Ronald S Go
Malignant histiocytosis (MH) is an extremely rare neoplasm of the macrophage-dendritic cell lineage. We report the clinical characteristics, molecular aberrations, treatments, and outcomes of patients with MH seen at two referral centers from January 2000 to May 2023. We identified 43 patients with MH, of which 26 had histiocytic sarcoma (MH-H), 9 interdigitating dendritic cell sarcoma (MH-IDC), and 8 Langerhans cell sarcoma (MH-LC). The median age at diagnosis was 61 years (range, 3-83). Thirty-three patients (77%) had multifocal disease, while 10 had unifocal involvement...
February 26, 2024: American Journal of Hematology
https://read.qxmd.com/read/38406811/case-report-exploring-the-utility-of-whole-body-bone-scintigraphy-for-pediatric-langerhans-cell-histiocytosis-insights-from-clinical-practice
#14
Wenyu Song, Fan Hu, Wei Shi, Fang Wang, Yongxue Zhang, Xiaoli Lan, Xiaotian Xia
PURPOSE: This mini-review delves into the realm of Langerhans cell histiocytosis (LCH) in children, focusing on its skeletal involvement. By synthesizing pertinent literature, we sought to provide a comprehensive understanding of LCH's clinical and radiographic spectrum. Our study then demonstrates the diagnostic prowess of whole-body 99m Tc-methyl diphosphonate (MDP) scintigraphy in LCH cases, underscoring its value in tandem with existing knowledge. METHODS: Our approach involved an extensive literature review that contextualized LCH within the current medical landscape...
2024: Frontiers in Oncology
https://read.qxmd.com/read/38406623/a-rare-case-of-cutaneous-langerhans-cell-histiocytosis-in-an-adult-patient
#15
JOURNAL ARTICLE
Aleena Boby, Nirav Shah, Ann Lin
No abstract text is available yet for this article.
March 2024: JAAD Case Reports
https://read.qxmd.com/read/38401151/founders-of-pediatric-pathology-dr-ron-jaffe-1943-2022-an-appreciation
#16
JOURNAL ARTICLE
Laura S Finn, Jennifer Picarsic, A S Knisely
No abstract text is available yet for this article.
February 24, 2024: Pediatric and Developmental Pathology
https://read.qxmd.com/read/38390771/an-unusual-case-of-metastatic-trophoblastic-neoplasm-presenting-with-diffuse-cystic-lung-disease-and-pulmonary-artery-pseudoaneurysms-in-a-teenager
#17
JOURNAL ARTICLE
Sevim Guler, Nathan C Hull, Manuel Arteta, Wendy Allen-Rhoades, Maryam Shahi, Michael B Ishitani, Nadir Demirel
Diffuse cystic lung diseases (DCLDs) are a diverse group of lung disorders characterized by the presence of multiple air filled cysts within the lung tissue. These cysts are thin walled and surrounded by normal lung tissue. In adults, DCLD can be associated with various conditions such as lymphangioleiomyomatosis (LAM), Langerhans cell histiocytosis, cancers, and more. In children, DCLD is often linked to lung developmental abnormalities, with bronchopulmonary dysplasia being a common cause. Patients with pulmonary cysts are typically asymptomatic, but some may experience mild symptoms or pneumothorax...
February 23, 2024: Pediatric Pulmonology
https://read.qxmd.com/read/38388646/phase-2-study-using-low-dose-cytarabine-for-adult-patients-with-newly-diagnosed-langerhans-cell-histiocytosis
#18
JOURNAL ARTICLE
Long Chang, Min Lang, He Lin, Hao Cai, Ming-Hui Duan, Dao-Bin Zhou, Xin-Xin Cao
Langerhans cell histiocytosis (LCH) lacks a standardized first-line therapy. This single-center, phase 2 prospective study (NCT04121819) enrolled 61 newly diagnosed adult LCH patients with multisystem or multifocal single system disease from October 2019 to June 2022. Subcutaneous cytarabine (100 mg/m2 for 5 days) was administered in 35-day cycles for 12 total cycles. The primary endpoint was event-free survival (EFS). The median age was 33 years (range 18-66). Twelve patients (19.7%) had liver involvement, of which 2 also had spleen involvement...
February 22, 2024: Leukemia
https://read.qxmd.com/read/38387093/treatment-of-congenital-langerhans-cell-histiocytosis-with-cobimetinib
#19
Ghita Benjelloun, Charlotte Roquet-Gravy, Liliane Marot, Léo-Paul Secco, Pierre-Paul Roquet-Gravy, Marie Baeck, Audrey Bulinckx
We report a case of congenital multisystem Langerhans cell histiocytosis with cutaneous and hematopoietic involvement. After the failure of first-line (vinblastine and prednisolone) and second-line (vincristine and cytarabine) therapies, treatment with cobimetinib, a mitogen-activated protein kinase (MEK) inhibitor, led to the remission of disease and a sustained response after 11 months of ongoing treatment. Protein kinase inhibitors targeting BRAF or MEK could represent a promising future therapeutic option, also in children with LCH...
February 22, 2024: Pediatric Dermatology
https://read.qxmd.com/read/38381107/-panhypopituitarism-diabetes-insipidus-and-bone-pain-is-there-a-systemic-disease-behind-it
#20
JOURNAL ARTICLE
Martina Bertschinger, Lisa Sze, Irene Bosma, Corina Dommann-Scherrer, Jeroen Goede
A 27-year-old man presented due to unilateral leg pain. He had a history of diabetes insipidus and panhypopituitarism. Laboratory analysis revealed hormonal undersupply. MRI showed a large contrast medium-absorbing mass in the pituitary gland extending into the hypothalamus. FDG-PET/CT examination revealed a hypermetabolic soft tissue lesion around the left femoral shaft. After biopsy of the lesion, a diagnosis of multisystemic Langerhans cell histiocytosis was made.
January 2024: Praxis
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