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Related Experiment Videos

[Reactive macrophage activation syndrome].

Szilvia Szamosi1, Gabriella Szúcs, Margit Zeher

  • 1Debreceni Egyetem, Orvos- és Egészségtudományi Centrum Altalános Orvostudományi Kar, III. Belgyógyászati Klinika, Klinikai Immunológia Tanszék.

Orvosi Hetilap
|November 5, 2003
PubMed
Summary
This summary is machine-generated.

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Reactive macrophage activation syndrome, also known as haemophagocytic lymphohistiocytosis, is a severe immune disorder. It involves excessive immune cell activation and cytokine release, leading to organ damage and often requiring intensive treatment.

Area of Science:

  • Immunology
  • Hematology
  • Pathology

Context:

  • A life-threatening immunoregulatory disorder characterized by excessive immune activation.
  • Often presents in children, with familial and sporadic forms.
  • Associated with infections, malignancies, and autoimmune conditions.

Purpose:

  • To describe the pathogenesis, clinical presentation, and management of haemophagocytic lymphohistiocytosis.
  • To highlight the role of uncontrolled T-cell activation and cytokine storm.
  • To outline therapeutic strategies including immunosuppression and bone marrow transplantation.

Summary:

  • Characterized by mononuclear phagocytic system activation and haemophagocytosis in multiple organs.
  • Clinical features include fever, hepatosplenomegaly, cytopenias, and coagulopathy.

Related Experiment Videos

  • Pathogenesis involves excessive pro-inflammatory cytokine secretion and histiocytic infiltrates.

    • Impact:

    • Understanding the pathophysiology aids in early diagnosis and intervention.
    • Effective treatments can induce remission in a significant number of patients.
    • Allogeneic bone marrow transplantation offers a curative option for refractory cases.