Response of pure red cell aplasia to cyclophosphamide after failure of mycofenolate mofetil in a patient with polyglandular syndrome type I.

Abstract

A 26-year-old female with the classic major and minor components of autoimmune polyglandular syndrome type 1 was diagnosed as having pure red cell aplasia. Treatment with 1.5 g/d mycofenolate mofetil for 3 months failed to restore erythroid production. Treatment with cyclosporine A produced a good partial response but led to renal toxicity and was therefore substituted with cyclophosphamide, which had a good partial effect and lasted for 18 months. The relapse of anemia was not observed during the 6-month follow-up period after the cessation of treatment.

• Authors: Elizaveta M Orlova, Maria A Kareva, Maria A Melikyan, Elena Boyakova, Valentina A Peterkova, Alexey A Maschan
• Journal: Journal of pediatric hematology/oncology (J Pediatr Hematol Oncol) Vol. 35 Issue 8 Pg. e338-40 (Nov 2013) ISSN: 1536-3678 [Electronic] United States
• PMID: 23128333 (Publication Type: Case Reports, Journal Article)

Chemical References

• APECED protein
• Immunosuppressive Agents
• Transcription Factors
• Cyclophosphamide

Topics

• Adult
• Cyclophosphamide (therapeutic use)
• Female
• Humans
• Immunosuppressive Agents (therapeutic use)
• Mutation
• Polyendocrinopathies, Autoimmune (complications, genetics, physiopathology)
• Red-Cell Aplasia, Pure (complications, drug therapy, physiopathology)
• Transcription Factors (genetics)