Abstract
Background Pure red cell aplasia (PRCA) is less common blood disorder; the causes and the treatments of PRCA are varied. Methods We conducted a retrospective study during January 2010–December 2017, to explore the etiologies and to evaluate the response and treatment burden in adult patients with PRCA. Results Of 32 PRCA patients, median age was 57 years (18–90 years). Median hemoglobin level and reticulocyte count at the time of diagnosis were 5.6 g/dL (3.3–7.3 g/dL) and 0.3% (0.1–0.7%), respectively. Median time to hematologic recovery was 12 weeks (3–72 weeks), and median number of red blood cell transfusion (RBC) was 20 units (4–100 units). Causes of PRCA were erythropoiesis-stimulating agent (ESA) (47%), parvovirus B19 infection (19%), thymoma (13%), zidovudine (6%), primary autoimmune PRCA (6%), Kaposi's sarcoma (3%), systemic lupus erythematosus (3%), and ABO-mismatched stem cell transplantation (3%). Only 9 out of 24 treated patients achieved hematologic response within 8 weeks of treatment. Intravenous immunoglobulin therapy provided 100% response rate in patients with parvovirus B19-associated PRCA and primary autoimmune PRCA. Low response rate was found in patients receiving immunosuppressants and chemotherapy for the treatment of ESA and thymoma-associated PRCA, respectively. Conclusions Treatment outcome of PRCA depended upon the causes and the types of treatment, and the burden of RBC transfusion was very high in patients with ESA and thymoma-associated PRCA.
Highlights
Pure red cell aplasia (PRCA) is a rare but devastating blood disorder presenting with normocytic anemia, severe reticulocytopenia, and markedly decreased number of erythroid precursors from the bone marrow (BM)
Of 9 patients, 7 received intravenous immunoglobulin (IvIg) therapy, five had parvovius B19, and two had primary autoimmune PRCA. e remaining 2 patients diagnosed with human immunodeficiency virus (HIV) achieved hematologic response after cessation of AZT
erythropoiesis-stimulating agent (ESA)-induced PRCA was frequently seen in the study since there were many brands of erythropoietin available in our country, and the issues that the physicians should be considered before choosing the type of erythropoietin therapy were the structure of erythropoietin, pharmacokinetic, pharmacodynamics, potency, immunogenicity, safety, purity, and the coated rubber stoppers [9]
Summary
Pure red cell aplasia (PRCA) is a rare but devastating blood disorder presenting with normocytic anemia, severe reticulocytopenia, and markedly decreased number of erythroid precursors from the bone marrow (BM). Pure red cell aplasia (PRCA) is less common blood disorder; the causes and the treatments of PRCA are varied. Causes of PRCA were erythropoiesis-stimulating agent (ESA) (47%), parvovirus B19 infection (19%), thymoma (13%), zidovudine (6%), primary autoimmune PRCA (6%), Kaposi’s sarcoma (3%), systemic lupus erythematosus (3%), and ABO-mismatched stem cell transplantation (3%). Intravenous immunoglobulin therapy provided 100% response rate in patients with parvovirus B19-associated PRCA and primary autoimmune PRCA. Low response rate was found in patients receiving immunosuppressants and chemotherapy for the treatment of ESA and thymoma-associated PRCA, respectively. Treatment outcome of PRCA depended upon the causes and the types of treatment, and the burden of RBC transfusion was very high in patients with ESA and thymoma-associated PRCA
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