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Macrophage activation syndromes.

A Albert1, Z Azgui, J Buisine

  • 1Service d'Hématologie Normale et Pathologique, Centre Régional de Transfusion Sanguine, Strasbourg, France.

Nouvelle Revue Francaise D'Hematologie
|January 1, 1992
PubMed
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Macrophage activation syndrome (MAS) presents with fever and organ enlargement, diagnosed via bone marrow examination. While often treatable, relapses can occur, particularly with co-existing autoimmune diseases.

Area of Science:

  • Hematology
  • Immunology
  • Pathology

Background:

  • Macrophage activation syndrome (MAS) is a severe, life-threatening hyperinflammation.
  • It is characterized by widespread activation of macrophages and hemophagocytosis.
  • MAS often occurs secondary to infections, autoimmune diseases, or malignancies.

Purpose of the Study:

  • To review the clinical and laboratory features of 47 cases of MAS.
  • To identify diagnostic criteria and understand the disease's evolution and associations.
  • To explore potential pathways for understanding MAS pathophysiology.

Main Methods:

  • Retrospective review of 47 MAS cases.
  • Analysis of clinical presentations, laboratory findings, and bone marrow aspirates.

Related Experiment Videos

  • Evaluation of treatment responses and disease outcomes.

    • Main Results:

    • Common symptoms include fever, hepatosplenomegaly, and cytopenias.
    • Bone marrow examination revealed actively phagocytic macrophages as a key diagnostic sign.
    • Most patients showed regression with treatment (antibiotics, antivirals, corticoids, chemotherapy), but relapses were observed, especially with systemic lupus erythematosus.

    Conclusions:

    • MAS diagnosis is often established through bone marrow morphology.
    • While treatment can induce remission, the underlying causes and predisposition require further investigation.
    • Future research focusing on macrophage function and immune cells may elucidate MAS pathophysiology and improve therapeutic strategies.