Hemophagocytic syndrome (HPS) is a life-threatening hematological disorder in immunocompromised patients. Reactive HPS is observed in patients with systemic infection, neoplasia or auto-immune diseases. It is a rare hematological disorder after renal transplantation and must be suspected when fever and pancytopenia are seen in association with viral infections. HPS is usually associated with infection with the Cytomegalovirus and Epstein-Barr viruses. We report here a case of BK-virus-associated HPS.
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Risk Factors of Hemophagocytic Lymphohistiocytosis in Adults with Fever of Unknown Origin: A Retrospective Study.
Int J Gen Med
January 2025
Department of Infectious Diseases, Tongji Hospital Affiliated to Tongji Medical College, Huazhong University of Science and Technology, Wuhan, People's Republic of China.
Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a critical syndrome with a high mortality rate. In clinical practice, some patients with fever of unknown origin (FUO) can develop HLH, further complicating the diagnosis and treatment. However, studies on HLH in adults with FUO are limited.
View Article and Find Full Text PDFMulticenter validation of secondary hemophagocytic lymphohistiocytosis diagnostic criteria.
J Intern Med
January 2025
Department of Anesthesiology and Intensive Care Medicine (CCM, CVK), Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
Background: Five fulfilled hemophagocytic lymphohistiocytosis (HLH)-2004 criteria, and the HScore are widely used and recommended by international expert consensus to diagnose secondary HLH. Both diagnostic scores have never been validated in heterogeneous patient cohorts of secondary HLH patients. We aimed to systematically optimize and validate diagnostic criteria of secondary HLH using a multicenter approach.
View Article and Find Full Text PDFHemophagocytic lymphohistiocytosis in early pregnancy: A rare and fatal diagnostic challenge.
Obstet Med
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Intensive Care and Obstetric Research Group (GRICIO), Universidad de Cartagena, Cartagena, Colombia.
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Cardiovascular complications in chronic active Epstein-Barr virus disease: a case report and literature review.
Front Pediatr
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Key Laboratory of Birth Defects and Related Diseases of Women and Children of Ministry of Education (MOE), West China Institute of Women and Children's Health, Key Laboratory of Development and Diseases of Women and Children of Sichuan Province, Department of Pediatrics, Department of Pediatric Cardiology, West China Second University Hospital, Sichuan University, Chengdu, Sichuan, China.
Background: Cardiovascular involvement is a rare but severe complication of Epstein-Barr virus (EBV) infections. Patients with chronic active EBV (CAEBV) are at increased risk of developing cardiovascular complications and have a poor prognosis. Here, we report the rare case of a pediatric patient with CAEBV and EBV- hemophagocytic lymphohistiocytosis (HLH) complicated with a giant coronary artery aneurysm (CAA) and thrombosis, a giant Valsalva sinus aneurysm, and ascending aorta dilation seven years after the disease onset.
View Article and Find Full Text PDFMixed histiocytic sarcoma in a Bernese Mountain Dog.
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An 8-y-old, spayed female Bernese Mountain Dog was presented to a referral center for evaluation of right thoracic limb lameness and previously suspected Evans syndrome that had been poorly responsive to immunosuppressive therapy. Based on review of examination findings and laboratory data, Evans syndrome was deemed unlikely and hemophagocytic histiocytic sarcoma (HHS) was strongly suspected. On blood smear evaluation, atypical, histiocytic cells were noted, some of which exhibited siderophagia.
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