Etiologies and Treatment Burden in Adult Patients with Pure Red Cell Aplasia: A Single-Center Experience and Review of Literature

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.

A 46-Year-Old Thai Woman with Secondary Acquired Pure Red Cell Aplasia Due to Treatment with Recombinant Erythropoietin While on Dialysis for End-Stage Renal Disease Who Recovered Following ABO-Incompatible Kidney Transplantation

“…In this case, our suspicions were based on her underlying autoimmune disease and past exposure to 2 types of rHuEPO (continuous erythropoietin receptor activator [CERA] and biosimilar erythropoietin alpha). PRCA is rare in patients with SLE [ 16 , 17 ]. A retrospective study [ 17 ] we performed January 2010 to December 2017 in our center showed that nearly 50% of the etiology was ESA-related PRCA.…”

“…PRCA is rare in patients with SLE [ 16 , 17 ]. A retrospective study [ 17 ] we performed January 2010 to December 2017 in our center showed that nearly 50% of the etiology was ESA-related PRCA. Only 3% were related to SLE, which responded poorly to immunosuppressive agents.…”

Pure red cell aplasia secondary to rheumatoid arthritis: a case report

“…The clinical outcome of PRCA is variable from complete remission to fatal, and it depends upon the cause and the types of treatment [ 5 , 17 , 22 – 24 ]. Some patients with PRCA show no response to treatment [ 5 ].…”

Pure Red Cell Aplasia Secondary to Rheumatoid Arthritis

“…The clinical outcome of PRCA is variable from complete remission to fatal, and it depends upon the cause and the types of treatment [11,18,23]. Some patients with PRCA show no response to treatment [11].…”

“…Naparuck et al has reported favourable treatment response in groups of parvovirus B19 infection, drug-(non-erythropoiesisstimulating agent (ESA)-) induced PRCA and idiopathic PRCA. The outcome has been unfavourable in groups of ESA and thymoma-induced PRCA, even they were receiving immunosuppressants or chemotherapy, respectively [23]. However, Hirokawa et al has reported cyclosporine producing excellent responses in patients with thymoma-associated PRCA, although limited information suggests that patients with thymoma-associated PRCA have poor prognoses [24].…”