During the past 5 years we have investigated 45 patients with the clinical manifestations suggestive of cirrhosis. We found 40 of them having cirrhosis but 3 patients had idiopathic splenomegaly. They underwent splenctomy and after 1-5 years they have remained free of the disease.
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J Med Case Rep
January 2025
Department of Hepatic Biliary Pancreatic Medicine, First Hospital of Jilin University, 1 Xinmin Avenue, Changchun, 130021, China.
Background: Dyskeratosis congenita is a rare genetic disease due to telomere biology disorder and characterized by heterogeneous clinical manifestations and severe complications. "Porto-sinusoidal vascular disease" has been recently proposed, according to new diagnostic criteria, to replace the term "idiopathic non-cirrhotic portal hypertension." TERT plays an important role in telomeric DNA repair and replication.
View Article and Find Full Text PDFClin J Gastroenterol
December 2024
Department of Gastroenterology and Hepatology, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu, 514-8507, Japan.
J Equine Vet Sci
January 2025
Department of Functional Sciences, Equine Division, Sports Medicine Section, Faculty of Veterinary Medicine, University of Liège, Liège, Belgium. Electronic address:
Intern Med
November 2024
Department of Rheumatology, Fukushima Medical University School of Medicine, Japan.
It is difficult to distinguish idiopathic multicentric Castleman disease (iMCD) from immunoglobulin G4-related disease (IgG4-RD). A 47-year-old man was diagnosed with venous thrombosis in the right lower extremity. Multiple lymphadenopathies and splenomegaly were incidentally detected.
View Article and Find Full Text PDFEur J Haematol
December 2024
Division of Hematology, Dalhousie University, Halifax, Nova Scotia, Canada.
Background: Idiopathic multicentric Castleman disease (iMCD) is a rare inflammatory disorder mediated by excessive proinflammatory cytokine signaling, most notably by interleukin 6 (IL-6). IL-6-induced extramedullary hematopoiesis (EMH) has been reported in murine models of iMCD. Herein we present four cases of iMCD with EMH in humans.
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