Introduction <p>Systemic inflammatory and autoimmune diseases and myelodysplastic syndrome (MDS) have been linked in patients. No previous reports exist of complications with non-infectious meningeal involvement in Behçet-like disease associated with MDS.</p> Case Presentation <p>Herein, we describe a rare case of Behçet-like disease associated with MDS, with 5q deletion and monosomy 7, complicated by meningeal and pulmonary involvement. A 69-year-old man presented with intermittent fever, headache and bloody mucous stools. After hospitalisation, the patient developed oral and intestinal ulcers, peripheral blood blasts, progressive thrombocytopenia and respiratory failure. The patient was diagnosed with Behçet-like disease associated with MDS. The patient was administered steroids and antimicrobials. Fever resolved and respiratory status improved, but his ADLs declined due to exhaustion.</p> Conclusion <p>Treatment in such cases should be individualised; however, further research is required.</p>

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Behçet-like Disease Associated with Myelodysplastic Syndrome with 5q Deletion and Monosomy 7 Complicated by Meningeal and Pulmonary Involvement: A Case Report

  • Akiko Kameyama,
  • Hideyuki Nakazawa,
  • Takahito Katsuyama,
  • Michitaro Ichikawa,
  • Atsuhito Ushiki,
  • Takeshi Uehara,
  • Naoko Asano,
  • Hiroshi Imamura

摘要

Introduction

Systemic inflammatory and autoimmune diseases and myelodysplastic syndrome (MDS) have been linked in patients. No previous reports exist of complications with non-infectious meningeal involvement in Behçet-like disease associated with MDS.

Case Presentation

Herein, we describe a rare case of Behçet-like disease associated with MDS, with 5q deletion and monosomy 7, complicated by meningeal and pulmonary involvement. A 69-year-old man presented with intermittent fever, headache and bloody mucous stools. After hospitalisation, the patient developed oral and intestinal ulcers, peripheral blood blasts, progressive thrombocytopenia and respiratory failure. The patient was diagnosed with Behçet-like disease associated with MDS. The patient was administered steroids and antimicrobials. Fever resolved and respiratory status improved, but his ADLs declined due to exhaustion.

Conclusion

Treatment in such cases should be individualised; however, further research is required.