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Related Concept Videos

Hypersensitivity Reactions: Immune-Complex Reactions01:19

Hypersensitivity Reactions: Immune-Complex Reactions

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Type III hypersensitivity reactions occur when antigen–antibody complexes form and activate the complement system. Normally, these complexes help the clearance of antigens by phagocytes and red blood cells. However, when large numbers of immune complexes are present, they can deposit in tissues—particularly in the walls of blood vessels—leading to inflammation and tissue injury. These deposits trigger complement activation and neutrophil recruitment, resulting in serum...
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[Lymphohistiocytic activation syndrome (LHAS)].

R Berrady1, W Bono1

  • 1Service de médecine interne, CHU Hassan II, route de Sidi Hrazem, Fes, Maroc.

Annales Francaises D'Anesthesie Et De Reanimation
|January 21, 2014
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Summary

Lymphohistiocytic activation syndrome (LHAS) involves overactive immune cells, presenting with fever, enlarged organs, and abnormal blood counts. Early diagnosis is crucial for managing this emergency condition, with genetic studies offering insights into its causes.

Keywords:
CIVDDICFeverFièvreMacrophagePancytopeniaPancytopénieShockÉtat de choc

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Area of Science:

  • Hematology
  • Immunology
  • Genetics

Context:

  • Lymphohistiocytic activation syndrome (LHAS) is a critical condition characterized by inappropriate macrophage activation.
  • It presents with non-specific clinical signs like fever, hepatosplenomegaly, and lymphadenopathy.
  • Associated biological markers include cytopenias, liver dysfunction, coagulopathy, and elevated LDH, ferritin, and triglycerides.

Purpose:

  • To define the clinical and biological characteristics of Lymphohistiocytic activation syndrome (LHAS).
  • To emphasize the diagnostic urgency of LHAS in febrile cytopenia cases.
  • To highlight the importance of understanding the etiology, particularly through genetic studies.

Summary:

  • LHAS involves aberrant macrophage stimulation in the bone marrow and lymphoid system.
  • Clinical manifestations are diverse, including fever, malaise, organomegaly, and lymphadenopathy.
  • Key laboratory findings encompass cytopenias, abnormal liver function, coagulopathy, and elevated inflammatory markers.

Impact:

  • Highlights the critical need for prompt diagnosis and management of LHAS.
  • Underscores the role of genetic research in elucidating the underlying mechanisms of familial LHAS.
  • Aims to improve clinical recognition and timely intervention for patients with febrile cytopenias.