Response of Pure Red Cell Aplasia to Cyclophosphamide After Failure of Mycofenolate Mofetil in a Patient With Polyglandular Syndrome Type I

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.

[1]  Mark S. Anderson,et al.  Control of central and peripheral tolerance by Aire , 2011, Immunological reviews.

[2]  M. Rimbert,et al.  Large Granular Lymphocyte Leukemia with Pure Red Cell Aplasia Associated with Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy: An Unfortuitous Association? , 2010, International journal of immunopathology and pharmacology.

[3]  E. S. Kuznetsova,et al.  Autoimmune Polyglandular Syndrome Type 1 in Russian Patients: Clinical Variants and Autoimmune Regulator Mutations , 2010, Hormone Research in Paediatrics.

[4]  M. Hirokawa,et al.  Acquired pure red cell aplasia: updated review of treatment , 2008, British journal of haematology.

[5]  J. Perheentupa.  Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy. , 2006, The Journal of clinical endocrinology and metabolism.

[6]  J. Lust,et al.  Aplastic anemia and pure red cell aplasia associated with large granular lymphocyte leukemia. , 2003, Seminars in hematology.

[7]  S. Ogoshi,et al.  Pure red cell aplasia and myasthenia gravis with thymoma: a case report and review of the literature. , 1998, Japanese journal of clinical oncology.

[8]  V. Santini,et al.  Pure red cell aplasia in autoimmune polyglandular syndrome with T lymphocytosis. , 1998, Haematologica.

[9]  L. Peltonen,et al.  An autoimmune disease, APECED, caused by mutations in a novel gene featuring two PHD-type zinc-finger domains , 1997, Nature Genetics.

[10]  Amos Etzioni,et al.  Complete recovery of pure red cell aplasia by intramuscular gammaglobulin therapy in a child with hypoparathyroidism , 1986, American journal of hematology.

[11]  T. J. Myers,et al.  Pure red cell aplasia and the syndrome of multiple endocrine gland insufficiency. , 1980, The American journal of the medical sciences.