Marginal Zone Lymphoma Manifesting as Macrophage Activation Syndrome: A Case Report

Niloufar Ebrahimi, Sahibzadi Mahrukh Noor, Shahram Kordasti, Mojtaba Akhtari, Sayna Norouzi, Mehrbod Vakhshoori, Amir Abdipour

Research output: Contribution to journalArticlepeer-review

Abstract

Macrophage activation syndrome (MAS) is a form of secondary hemophagocytic lymphohistiocytosis (HLH) when it occurs in the context of rheumatologic disorders. HLH is a rare and potentially life-threatening syndrome characterized by excessive immune system activation. It is mainly seen in children and can be genetic based or related to infections, malignancies, rheumatologic disorders, or immunodeficiency syndromes. MAS can present with nonspecific symptoms, leading to a delay in diagnosis. This report describes a case of a 64-year-old female with marginal zone lymphoma and systemic lupus erythematosus who presented with a purpuric rash and acute kidney injury. She underwent a kidney biopsy and was diagnosed with MAS. This case highlights the importance of promptly recognizing MAS’s symptoms and signs, allowing timely diagnosis and early therapeutic intervention. This potentially fatal condition tends to respond well to rapid treatment initiation with corticosteroids and to address the underlying condition.

Original languageEnglish
Article number23247096241244732
JournalJournal of Investigative Medicine High Impact Case Reports
Volume12
DOIs
Publication statusE-pub ahead of print - 5 Apr 2024

Keywords

  • Child
  • Female
  • Humans
  • Middle Aged
  • Macrophage Activation Syndrome/diagnosis
  • Lymphohistiocytosis, Hemophagocytic/diagnosis
  • Adrenal Cortex Hormones/therapeutic use
  • Lymphoma, B-Cell, Marginal Zone/complications
  • Arthritis, Rheumatoid/complications

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