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A sustained response to low dose interferon‐α in a case of refractory pure red cell aplasia
Author(s) -
Nadeau Laura,
Meyerson Howard,
Warren Gregory,
Koç Omer N.
Publication year - 2004
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.1600-0609.2004.00291.x
Subject(s) - refractory (planetary science) , pure red cell aplasia , medicine , aplasia , bone marrow aplasia , interferon , immunology , biology , bone marrow , astrobiology
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.