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Although hemophagocytic syndrome is a rare clinical condition, it is associated with high mortality and the number of cases described in the literature has progressively increased. The diagnosis of hemophagocytic syndrome is made on the basis of a finding of hemophagocytosis. Sarcoidosis is a highly prevalent disease whose course and prognosis might correlate with the initial clinical presentation and the extent of the disease. We report the case of a patient with long-standing sarcoidosis who presented with intermittent fever and fatigue. The diagnosis of hemophagocytic syndrome was made by bone marrow aspiration, and specific treatment was ineffective. This is the third case of sarcoidosis-related hemophagocytic syndrome reported in the literature and the first reported in Latin America. All three cases had unfavorable outcomes.
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http://dx.doi.org/10.1590/s1806-37132012000500017 | DOI Listing |
Eur J Haematol
March 2025
Hematopoietic Stem Cell Transplantation and Cellular Therapy Program, Division of Hematology/Oncology, Department of Medicine, University of California Irvine, Irvine, California, USA.
Background: Data on bone marrow (BM) findings in secondary hemophagocytic lymphohistiocytosis (sHLH) and their association with overall survival (OS) are limited.
Objectives: This study aimed to develop a prognostic model incorporating BM findings and clinico-laboratory factors affecting OS.
Methods: We retrospectively evaluated 50 adults with sHLH and developed a clinicopathological prognostic model based on survival-associated factors.
Haemophagocytic lymphohistiocytosis (HLH) is a condition of uncontrolled immune activation caused by genetic defects or triggered by infections, malignancies, autoimmune diseases, drugs, pregnancy or post-transplant immunosuppression. This case describes a young man presenting with clinical jaundice and abnormal blood tests, including raised inflammatory markers, abnormal liver function, low haemoglobin (65 g/L) and a low white blood cell count (3.08×10/L).
View Article and Find Full Text PDFAm J Med
March 2025
University of Florida College of Medicine - Jacksonville, Department of Medicine, 653-1 West 8th Street, L20, Jacksonville, FL, 32209, United States of America.
BMJ Case Rep
March 2025
Norwich Medical School, University of East Anglia, Norwich, UK
Haemophagocytic lymphohistiocytosis (HLH) is a severe systemic inflammatory syndrome. Over activation of histiocytes and lymphocytes can lead to multiorgan failure and death. The three key features are fever, raised ferritin and falling counts, that is, cytopenia (3Fs).
View Article and Find Full Text PDFBMJ Case Rep
March 2025
Allergy Immunology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
Haemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disorder of uncontrolled immune activation by macrophages, cytotoxic T cells and natural killer (NK) cells, marked by severe inflammation. It is often categorised as primary (genetic) or secondary (acquired). Here, a case of a young male with a history of stage IA seminoma in remission, who presented with persistent fevers, night sweats, weight loss and fatigue, is reported.
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