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Prelude to the perfect storm: the many triggers of secondary hemophagocytic lymphohistiocytosis.
Postgrad Med
September 2025
Department of Medicine, Summa Health, Akron, OH, USA.
Hemophagocytic lymphohistiocytosis (HLH) is an increasingly recognized disorder of immune hyperactivity that often leads to multiorgan failure and death. In adults, HLH is usually triggered by infection, malignancy, or an autoimmune/autoinflammatory disorder that precipitates a destructive cytokine storm. Treatment aims to deescalate the hyperimmunity by treating the triggers while interfering with the immune pathways that cause the morbidity.
View Article and Find Full Text PDFHemophagocytic Lymphohistiocytosis Triggered by Influenza B in a Child with ARFGEF2 mutation.
Klin Padiatr
September 2025
Pediatric intensive care, Istanbul Kanuni Sultan Suleyman Training and Research Hospital, Istanbul, Turkey.
Rare Presentation of Posttransplant Lymphoproliferative Disease in a Pediatric Liver Transplant Recipient: Plasmablastic Lymphoma Complicated With Hemophagocytic Lymphohistiocytosis.
Exp Clin Transplant
August 2025
>From the Department of Pediatric Hematology and Oncology, Baskent University Medical Faculty, Ankara, Turkey.
A 3-year-old female patient, who had received a liver transplant from her father 1 year previously to treat biliary atresia, was admitted with fever and pancytopenia. History showed Epstein-Barr virus polymerase chain reaction positivity detected in the patient 3 months earlier; the patient received reduced immunosuppression in doses of tacrolimus, and valganciclovir was administered. Physical exa-mination showed lymphadenopathies at the cervical, axillary and inguinal regions with 2 × 2 cm at diameter, splenomegaly of 5 cm, and fever of 39 °C.
View Article and Find Full Text PDFAn enigmatic tale of macrophages in bone marrow causing inflammation of the brain: A case report on CNS HLH.
Hematol Transfus Cell Ther
September 2025
Department of Medicine, Schulich School of Medicine and Dentistry, Western University, London, ON, Canada; Verspeeten Family Cancer Centre, London Health Sciences Centre, London, ON, Canada; Department of Oncology, Schulich School of Medicine and Dentistry, Western University, London, ON, Canada; Th
Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening immune disorder characterized by excessive inflammation and multiorgan involvement. Rarely, HLH can manifest with signs and symptoms isolated to the central nervous system (CNS). This case report highlights the unique clinical course of CNS-isolated HLH in a 19-year-old female who, despite a nine-year delay in diagnosis, achieved disease remission following a hematopoietic stem cell transplant (HSCT).
View Article and Find Full Text PDFBackground: Kikuchi-Fujimoto disease (KFD) is a benign, self-limited, rare disease that rarely coexists with hemophagocytic lymphohistiocytosis (HLH).
Methods: We reported a case of a young female who presented with recurrent fever accompanied by painful lymphadenopathy, joint pain, and skin rash.
Results: Excisional biopsy of lymph node indicated KFD.