Hemophagocytic lymphohistiocytosis (HLH), characterized by acute and progressive hyperinflammation, is a rare syndrome documented in a limited number of coronavirus disease 2019 (COVID-19) and human immunodeficiency virus (HIV) cases. While severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can provoke extensive immune activation and systemic inflammation, individuals with HIV, susceptible to immune dysregulation, are at heightened risk of severe complications from SARS-CoV-2. We report a case of a 24-year-old male with no significant medical history presenting with fever, weight loss, respiratory symptoms, and acute renal failure. Initial diagnosis revealed HIV with a CD4 count < 20 and concurrent COVID-19 infection leading to development of HLH. Despite aggressive management including antiretroviral therapy (ART), dexamethasone and supportive care, the patient deteriorated rapidly, leading to multiorgan failure. Coinfection with HIV and SARS-CoV-2 presents unique challenges, especially when complicated by secondary conditions such as HLH, which remains a diagnostic and therapeutic dilemma. Prompt recognition and aggressive management are crucial, necessitating a high index of suspicion and comprehensive evaluation including bone marrow biopsy to improve diagnostic accuracy and guide therapeutic interventions in such complex scenarios.
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http://dx.doi.org/10.14740/jmc4226 | DOI Listing |
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 PDFJ 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 PDFObstet Med
January 2025
Intensive Care and Obstetric Research Group (GRICIO), Universidad de Cartagena, Cartagena, Colombia.
Objective: This report details the complexities of diagnosing and treating rapid-onset multisystemic hemophagocytic lymphohistiocytosis (HLH) during pregnancy, as evidenced by a fatal case in early pregnancy with severe hematological and obstetric complications.
Case Presentation: A 20-year-old pregnant woman in her second pregnancy presented at 8 weeks of gestation with abdominal pain, fever, and rectal bleeding. Laboratory tests revealed leukopenia, thrombocytopenia, and anemia, leading to immediate transfusion and intensive care unit admission.
Front Pediatr
January 2025
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 PDFCell Mol Biol Lett
January 2025
University Cote d'Azur, Inserm, C3M, Nice, France.
Vacuolization of hematopoietic precursors cells is a common future of several otherwise non-related clinical settings such as VEXAS, Chediak-Higashi syndrome and Danon disease. Although these disorders have a priori nothing to do with one other from a clinical point of view, all share abnormal vacuolization in different cell types including cells of the erythroid/myeloid lineage that is likely the consequence of moderate to drastic dysfunctions in the ubiquitin proteasome system and/or the endo-lysosomal pathway. Indeed, the genes affected in these three diseases UBA1, LYST or LAMP2 are known to be direct or indirect regulators of lysosome trafficking and function and/or of different modes of autophagy.
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