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A sustained response to low dose interferon-α in a case of refractory pure red cell aplasia

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Nadeau L, Meyerson H, Warren G, Koç ON. A sustained response to low dose interferon-α in a case of refractory pure red cell aplasia.

Eur J Haematol 2004: 73: 300–303. © Blackwell Munksgard 2004. 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-α (INF-α) increases HLA class I expression on hematopoietic precursor cells. Therefore, we initiated a trial of INF-α 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-α should be used in a controlled trial in patients with PRCA to determine its activity and mechanism of action.
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Keywords: PRCA; red cell aplasia; refractory anemia

Document Type: Research Article

Publication date: October 1, 2004

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