Results 71 to 80 of about 6,609 (224)

VEXAS syndrome: Focus on dermatological manifestations and their histopathological correlate

JEADV Clinical Practice, Volume 3, Issue 4, Page 1035-1048, September 2024.
Abstract Background VEXAS ‘Vacuoles, E1 Enzyme, X‐linked, Autoinflammatory, Somatic syndrome’ is a rare autoinflammatory syndrome, first described in October 2020 by Beck et al. It is caused by somatic mutations in the UBA1 gene, coding for the E1 enzyme, responsible for ubiquitination.
Sofie Engelen, Anne‐Catherine Dens, Frederik Staels, Rik Schrijvers, Daniel Blockmans, Steven Vanderschueren, Albrecht Betrains, F. J. Sherida H. Woei‐A‐Jin, Arno Vanstapel, Franscesca Bosisio, Petra De Haes   +10 more
wiley   +1 more source

Emperipolesis in a Case of Adult T Cell Lymphoblastic Lymphoma (Mediastinal type)-Detected at FNAC and Imprint Cytology [PDF]

, 2011
Emperipolesis is a condition in which viable hematopoetic cells are seen intact in the cytoplasm of host cell without damage. This phenomenon is seen in many physiologic and pathologic conditions, its presence in Rosai Dorfman disease (RDD) is ...
Abhishekh, MG, Amita, K, Geethalakshmi, U, Vijay Shankar, S   +3 more
core  

Review and a case of rare Rosai-Dorfman disease

Biotechnology & Biotechnological Equipment
Rosai-Dorfman (RDD) is a rare histiocytic syndrome with variable clinical characteristics and extranodal involvement in more than 43% of the patients. This mini-review presents an overview of the literature on Rosai-Dorfman disease.
Dragomira Nikolova, Aleksandar Yordanov, Aleksandra Maslarova, Radka Hristoskova, Vera Damyanova, Savina Hadjidekova, Atanas Radinov   +6 more
doaj   +1 more source

Mixed histiocytic neoplasms: A multicentre series revealing diverse somatic mutations and responses to targeted therapy

British Journal of Haematology, Volume 205, Issue 1, Page 127-137, July 2024.
Novel somatic mutations were identified in mixed histiocytic neoplasms, including KRAS, MAP2K2, MAPK3, non‐V600‐BRAF, RAF1 and BICD2‐BRAF fusion. Targeted treatment was more likely to result in complete response and compared to other treatment modalities. Summary Histiocytic neoplasms are diverse clonal haematopoietic disorders, and clinical disease is
Joshua S. Friedman, Benjamin H. Durham, Anne S. Reiner, Mariko Yabe, Kseniya Petrova‐Drus, Ahmet Dogan, Melissa Pulitzer, Klaus J. Busam, Jasmine H. Francis, Raajit K. Rampal, Gary A. Ulaner, Ryan Reddy, Randy Yeh, Vaios Hatzoglou, Mario E. Lacouture, Veronica Rotemberg, Roei D. Mazor, Oshrat Hershkovitz‐Rokah, Ofer Shpilberg, Gaurav Goyal, Ronald S. Go, Jithma P. Abeykoon, Karen Rech, Diana Morlote, Shiraz Fidai, Vedavyas Gannamani, Maryam Zia, Omar Abdel‐Wahab, Katherine S. Panageas, Marc K. Rosenblum, Eli L. Diamond   +30 more
wiley   +1 more source

A Case Report Diagnosed with Rosai-Dorfman Disease by Voice Change [PDF]

, 2014
Rosai-Dorfman disease is a rare disorder of unknown of etiology and is usually associated with benign proliferation of hematopoietic and fibrous tissue that often manifest in the head and neck region.
최홍식
core  

Intraosseous Rosai-Dorfman disease diagnosed by touch imprint cytology evaluation: A case series [PDF]

, 2018
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.
Alvarez Alegret, Das, Demicco, Deshpande, Deshpande, Destombes, Eisen, Foucar, Gattuso, Goel, Hummel, Ioachim, Ioannidis, Jing, Kismet, Kumar, Kushwaha, Layfield, Li, Lopez, Majumdar, Malur, McAlister, Nawroz, Raslan, Rosai, Rosai, Schein, Trautman, Vujhini   +29 more
core   +2 more sources

Cutaneous crystal storing histiocytosis: A case series with review of literature

Journal of Cutaneous Pathology, Volume 51, Issue 7, Page 506-512, July 2024.
Abstract Crystal‐storing histiocytosis (CSH) is a rare condition in which crystals accumulate in the cytoplasm of histiocytes and is usually associated with a lymphoplasmacytic neoplasm. Cutaneous CSH is extraordinarily rare and limited to case reports in the literature. We report two cases of this disease with cutaneous involvement.
Haya A. Homsi, Calvin Knapp III, Shruti Agrawal, Shweta Bhavsar, Jennifer S. Ko, Steven D. Billings, Shira Ronen   +6 more
wiley   +1 more source

Diagnostic challenges in Rosai-Dorfman-Destombes disease (case description and literature review) [PDF]

, 2018
The described clinical case presents 39-year-old patient with no clinically significant accompanying diseases. Since the beginning of October 2015, the patient has complained of fever, abdominal pain and generalized enlarged lymph nodes.
Gercheva, Liana, Spasova, Snejana, Varbanov, Hinko, Yuzeir, Sheniz   +3 more
core   +2 more sources

Extranodal Rosai-Dorfman Disease presenting as an isolated epibulbar mass

Indian Journal of Ophthalmology, 2008
Rosai-Dorfman disease is a rare idiopathic disorder characterized by painless lymphadenopathy with cervical involvement in more than 80% cases. We report a case of Rosai-Dorfman disease presenting as an isolated epibulbar mass in a healthy young ...
Maheshwari Rajat, Shekde Satish
doaj  

Radiotherapeutic outcomes of Rosai–Dorfman disease with falx cerebri and superior sagittal sinus involvement: A rare case report with long‐term follow‐up

Clinical Case Reports, Volume 12, Issue 6, June 2024.
Key Clinical Message Intracranial RDD is rare medical event mimicking different diagnoses. Although the surgical resection is the best treatment option, but radiation therapy can also achieves long‐term suboptimal outcomes. Abstract An 83‐year‐old male with a history of tension‐type headaches was evaluated.
Hamid Nasrollahi, Susan Andalibi, Mansour Ansari, Maral Mokhtari, Ehsan Mohammad Hosseini, Mina Foroughi, Arman Sourani   +6 more
wiley   +1 more source