A sustained response to low dose interferon-alpha in a case of refractory pure red cell aplasia |
| |
Authors: | Nadeau Laura Meyerson Howard Warren Gregory Koç Omer N |
| |
Affiliation: | Division of Hematology/Oncology, Department of Medicine, Case Western Reserve University, Cleveland, OH 44106, USA. |
| |
Abstract: | Acquired pure red cell aplasia (PRCA) may be the result of a cellular or humoral autoimmune process. One proposed mechanism is the destruction of erythroid progenitors by self-reactive, cytotoxic T cells or natural killer (NK) cells. These cells normally express MHC class I receptors (KIR) which inhibit cytotoxicity when the target cell expresses the HLA class I antigen(s) they bind. Therefore, loss of these antigens on maturing erythroid progenitors may render them susceptible to destruction by the pathogenic cells. Interferon-alpha (INF-alpha) increases HLA class I expression on hematopoietic precursor cells. Therefore, we initiated a trial of INF-alpha in a patient with refractory PRCA. Following treatment, he developed transfusion independence, and a sustained normal hematocrit. Analysis of bone marrow erythroid cells revealed an increase in expression of HLA class I molecules. INF-alpha should be used in a controlled trial in patients with PRCA to determine its activity and mechanism of action. |
| |
Keywords: | red cell aplasia PRCA refractory anemia |
本文献已被 PubMed 等数据库收录! |
|