Results 61 to 70 of about 1,394 (181)

Targeting the mTOR pathway in idiopathic multicentric Castleman disease [PDF]

Journal of Clinical Investigation, 2019
Idiopathic multicentric Castleman disease (iMCD) is a rare hematologic illness of systemic inflammation and organ dysfunction, with unknown etiology. Although therapies targeting IL-6 have been proven effective, a subset of patients with iMCD are resistant to this approach. In this issue of the JCI, Fajgenbaum et al.
Robert M. Stern, Nancy Berliner
openaire   +2 more sources

Diagnostic challenges in patients with Castleman disease, a single center experience from Hungary

Pathology and Oncology Research
Castleman disease is a rare and atypical lymphoproliferative disorder characterized by diverse clinical manifestations. It has both unicentric and multicentric forms, the latter with further subdivisions, i.e., human herpesvirus 8-associated and ...
Boglárka Brúgós, Boglárka Brúgós, Zsófia Simon, Gábor Méhes, Árpád Illés, György Pfliegler, György Pfliegler   +6 more
doaj   +1 more source

Characterizing Mortality Associated with Idiopathic Multicentric Castleman Disease

Blood, 2021
Abstract Idiopathic multicentric Castleman disease (iMCD) is a rare, life-threatening hematologic disorder involving multi-organ dysfunction driven by inflammatory cytokines, often including interleukin-6 (IL-6). The five-year overall survival rate varies considerably between cohorts, ranging from 50 to 77%. It is not well understood why
Sheila K Pierson, Karan Kanhai, Adam Bagg, Daisy Alapat, Megan S. Lim, Mary Jo Lechowicz, Gordan Srkalovic, Thomas S. Uldrick, Frits van Rhee, David C Fajgenbaum   +9 more
openaire   +1 more source

Clinical Presentation, Treatment, and Outcomes of 28 Patients With Castleman Disease: A Retrospective Analysis of an Italian Cohort

eJHaem, Volume 6, Issue 6, December 2025.
ABSTRACT Background Castleman disease (CD) encompasses a range of heterogeneous non‐clonal lymphoproliferative disorders, including unicentric (UCD), and multicentric (MCD) forms. The latter is subdivided into HHV‐8+ MCD, POEMS‐MCD, and idiopathic‐MCD, not otherwise specified (iMCD‐NOS).
Caterina Cristinelli, Michele Merli, Marco Lucioni, Manuel Gotti, Roberta Sciarra, Sara Rattotti, Federico Carpi, Gianmarco Favrin, Benedetta Bianchi, Giuseppe Neri, Marta Coscia, Marcello Gambacorta, Francesco Passamonti, Marco Paulli, Luca Arcaini   +14 more
wiley   +1 more source

Ruxolitinib for the Treatment of Refractory Idiopathic Multicentric Castleman Disease: A Case Report [PDF]

Turk J Haematol
Gao YH, Duan MH, Li J, Zhang L.
europepmc   +2 more sources

Idiopathic Plasmacytic Lymphadenopathy Forms an Independent Subtype of Idiopathic Multicentric Castleman Disease

International Journal of Molecular Sciences, 2022
Idiopathic multicentric Castleman disease (iMCD) is a type of Castleman disease that is not related to KSHV/HHV8 infection. Currently, iMCD is classified into iMCD-TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly) and iMCD-NOS (not otherwise specified).
Asami Nishikori, Midori Filiz Nishimura, Yoshito Nishimura, Fumio Otsuka, Kanna Maehama, Kumiko Ohsawa, Shuji Momose, Naoya Nakamura, Yasuharu Sato   +8 more
openaire   +3 more sources

The Clinicopathologic Spectrum of Rosai–Dorfman–Destombes Disease in Adults: An Analysis of 16 Cases

European Journal of Haematology, Volume 115, Issue 6, Page 555-564, December 2025.
ABSTRACT Rosai–Dorfman–Destombes disease (RDD) is a rare histiocytic proliferation with protean clinical manifestations, resulting from the accumulation of activated histiocytes within nodal and extra‐nodal tissues. The diagnosis can be missed, particularly when biopsies are obtained from extra‐nodal sites, where histological features may be less ...
Mark Trinder, Collin Pryma, Emily Leung, Graham W. Slack, Pedro Farinha, Sean Young, Neval Ozkaya, Mollie Carruthers, Luke Y. C. Chen   +8 more
wiley   +1 more source

Think highly of immunoglobulin G4‐related chronic rhinosinusitis as a clinical entity in immunoglobulin G4‐related disease

Eye &ENT Research, Volume 2, Issue 3, Page 151-163, September 2025.
Abstract IgG4‐related chronic rhinosinusitis (IgG4‐related CRS) is gaining recognition among various specialized physicians. As a systemic disease involving multiple organs, IgG4‐related CRS is still not widely recognized as an independent clinical entity. Given the complexity of diagnosing autoimmune‐related multisystem diseases, early recognition and
Lianqi Wan, Luo Zhang, Yingshi Piao, Yuan Zhang   +3 more
wiley   +1 more source

Case Report: VEXAS Syndrome: From Mild Symptoms to Life-Threatening Macrophage Activation Syndrome

Frontiers in Immunology, 2021
Recently, a novel disorder coined VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome was identified in patients with adult-onset inflammatory syndromes, often accompanied by myelodysplastic syndrome1.
Frederik Staels, Frederik Staels, Albrecht Betrains, Albrecht Betrains, F. J. Sherida H. Woei-A-Jin, F. J. Sherida H. Woei-A-Jin, Nancy Boeckx, Nancy Boeckx, Marielle Beckers, Marielle Beckers, An Bervoets, An Bervoets, Mathijs Willemsen, Mathijs Willemsen, Barbara Neerinckx, Barbara Neerinckx, Stephanie Humblet-Baron, Daniel Engelbert Blockmans, Daniel Engelbert Blockmans, Steven Vanderschueren, Steven Vanderschueren, Rik Schrijvers, Rik Schrijvers, Rik Schrijvers   +23 more
doaj   +1 more source

Efficacy of High‐Dose Intravenous Anakinra in Pediatric TAFRO Syndrome: Report of Two Cases and Literature Review

Pediatric Blood &Cancer, Volume 72, Issue 8, August 2025.
ABSTRACT TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly) syndrome is a rare, life‐threatening inflammatory condition linked to infections, neoplasms, and idiopathic multicentric Castleman disease. Interleukin (IL)‐6 inhibitors are the primary treatment, but refractory cases require alternatives. This
Serena Palmeri, Jacopo Ferro, Valentina Natoli, Caterina Matucci‐Cerinic, Riccardo Papa, Silvia Rosina, Stefania Sorrentino, Stefano Volpi, Valerio Gaetano Vellone, Marco Gattorno, Roberta Caorsi   +10 more
wiley   +1 more source