title: A sustained response to low dose interferon-alpha in a case of refractory pure red cell aplasia.
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.
authors.affiliation: Division of Hematology/Oncology, Department of Medicine, Case Western Reserve University, Cleveland, OH 44106, USA.
authors.forename: Laura
authors.initials: L
authors.lastname: Nadeau
authors.forename: Howard
authors.initials: H
authors.lastname: Meyerson
authors.forename: Gregory
authors.initials: G
authors.lastname: Warren
authors.forename: Omer N
authors.initials: ON
authors.lastname: Koç
mesh_terms: D000328:Adult; D015395:Histocompatibility Antigens Class I / Q000276:immunology; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use*; D008297:Male; D012010:Red-Cell Aplasia, Pure / Q000188:drug therapy* / Q000276:immunology
chemical_list: D015395:Histocompatibility Antigens Class I; D016898:Interferon-alpha