Journal of Clinical Pathology 2007;60:717-720
CASE REPORT
Pure red cell aplasia associated with type I autoimmune polyglandular syndrome—successful response to treatment with mycophenolate mofetil: case report and review of literature
1 Institute of Hematology, Clinical Center of Serbia, Belgrade, Serbia
2 Institute for Pathophysiology, School of Medicine, University of Kragujevac, Kragujevac, Serbia
3 Institute for Molecular Genetics and Genetic Engineering, Belgrade, Serbia
4 Institute of Endocrinology, Clinical Center of Serbia, Belgrade, Serbia
5 Institute of Hematology, Clinical Center of Serbia, Belgrade, Serbia
Correspondence to:
Professor V Jurisic
University of Kragujevac, School of Medicine, PO Box 124, 34 000 Kragujevac, Serbia
Accepted 17 November 2006
| The first 150 words of the full text of this article appear below. |
Pure red cell aplasia (PRCA) is a rare haematological syndrome characterised by anaemia, reticulocytopenia and severe erythroid hypoplasia without alteration in megakaryocytic and myeloid maturation. Immune system irregularity can be mediated by the presence of autoantibodies against erythroid cells or against erythropoietin (Epo), or by hyperactivity of large granular lymphocytes with enhanced T cell or natural killer cell cytotoxicity.1–3 The association between PRCA and other autoimmune diseases such as autoimmune polyglandular syndrome (APS) I is rare.4
The second autoimmune disease, named APS I or autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), is a rare hereditary autosomal recessive disorder. It is characterised by the presence of chronic mucocutaneous candidiasis, multiple endocrinopathies (hypoparathyroidism, adrenocortical failure, hypergonadotropic hypogonadism, type I diabetes mellitus and pan-hypopituitarism), and various ectodermal manifestations (enamel dysplasia, nail dystrophy, alopecia, vitiligo and keratopathy).5,6 Clinically, APECED can be confirmed by appearances of at least two of the three features: candidiasis, hypoparathyroidism and adrenocortical failure.5–7
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