DOI: 10.12890/2025_005250

Rosai-Dorfman disease preceding acute myeloid leukemia: an extremely rare case and a literature review

Authors

  • Sara E. Marhoon College of Medicine, Mansoura University, Mansoura, Egypt
  • Ali H. Ali College of Medicine, Mansoura University, Mansoura, Egypt
  • Ali Husain College of Medicine, Mansoura University, Mansoura, Egypt
  • Salma Elashwah Medical Oncology Unit, Internal Medicine Department, Oncology Center, Mansoura University, Mansoura, Egypt
  • Azza AbdelAziz Department of Anatomic Pathology, Faculty of Medicine, Mansoura University, Mansoura, Egypt
  • Mariam Elias Department of Radiology, Faculty of Medicine, Mansoura University, Mansoura, Egypt

alihasanmakiali@gmail.com

Keywords

Rosai-Dorfman disease, acute myeloid leukaemia, neoplasia-associated RDD, histiocytosis

Abstract

Background: Rosai-Dorfman disease (RDD) is a histiocytic disorder that was recently reclassified as a histiocytic neoplasm. It is characterized by nodal and extranodal involvement. Leukaemia-associated RDD is a rare subtype of neoplasia-associated RDD, particularly when leukaemia precedes RDD, with only one paediatric case reported in the literature.
Case report: A 33-year-old woman presented with cervical swelling that had persisted for one month. Ultrasound examination and biopsy confirmed the diagnosis of RDD. The patient subsequently developed raccoon eyes and bilateral eyelid oedema. Laboratory investigations revealed bicytopenia and leukocytosis. Unexpectedly, bone marrow aspirate and flow cytometry confirmed the presence of acute myeloid leukaemia (AML). Following repeated complaints of headache, brain magnetic resonance imaging revealed a callosal lesion, suggestive of a space-occupying lesion. One year later, the patient relapsed and died from septicaemia.
Conclusion: This case report describes the progression of RDD to AML in an adult, highlighting the need for long-term follow-up and further research into its pathogenesis to improve outcomes.

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Published

2025-05-16

How to Cite

1.
Marhoon SE, Ali AH, Husain A, Elashwah S, AbdelAziz A, Elias M. Rosai-Dorfman disease preceding acute myeloid leukemia: an extremely rare case and a literature review. EJCRIM [Internet]. 2025 May 16 [cited 2026 Nov. 3];12(6). Available from: https://ejcrim.com/index.php/ejcrim/article/view/5250