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Etiologies and Treatment Burden in Adult Patients with Pure Red Cell Aplasia: A Single-Center Experience and Review of Literature
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...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7102412/ https://www.ncbi.nlm.nih.gov/pubmed/32257434 http://dx.doi.org/10.1155/2020/4812759 |
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author | Niparuck, Pimjai Kanoksil, Wasana Wacharapornin, Pathawut Chantrathammachart, Pichika Boongird, Sarinya |
author_facet | Niparuck, Pimjai Kanoksil, Wasana Wacharapornin, Pathawut Chantrathammachart, Pichika Boongird, Sarinya |
author_sort | Niparuck, Pimjai |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-7102412 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Hindawi |
record_format | MEDLINE/PubMed |
spelling | pubmed-71024122020-04-01 Etiologies and Treatment Burden in Adult Patients with Pure Red Cell Aplasia: A Single-Center Experience and Review of Literature Niparuck, Pimjai Kanoksil, Wasana Wacharapornin, Pathawut Chantrathammachart, Pichika Boongird, Sarinya Anemia Clinical Study 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. Hindawi 2020-03-15 /pmc/articles/PMC7102412/ /pubmed/32257434 http://dx.doi.org/10.1155/2020/4812759 Text en Copyright © 2020 Pimjai Niparuck et al. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Clinical Study Niparuck, Pimjai Kanoksil, Wasana Wacharapornin, Pathawut Chantrathammachart, Pichika Boongird, Sarinya Etiologies and Treatment Burden in Adult Patients with Pure Red Cell Aplasia: A Single-Center Experience and Review of Literature |
title | Etiologies and Treatment Burden in Adult Patients with Pure Red Cell Aplasia: A Single-Center Experience and Review of Literature |
title_full | Etiologies and Treatment Burden in Adult Patients with Pure Red Cell Aplasia: A Single-Center Experience and Review of Literature |
title_fullStr | Etiologies and Treatment Burden in Adult Patients with Pure Red Cell Aplasia: A Single-Center Experience and Review of Literature |
title_full_unstemmed | Etiologies and Treatment Burden in Adult Patients with Pure Red Cell Aplasia: A Single-Center Experience and Review of Literature |
title_short | Etiologies and Treatment Burden in Adult Patients with Pure Red Cell Aplasia: A Single-Center Experience and Review of Literature |
title_sort | etiologies and treatment burden in adult patients with pure red cell aplasia: a single-center experience and review of literature |
topic | Clinical Study |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7102412/ https://www.ncbi.nlm.nih.gov/pubmed/32257434 http://dx.doi.org/10.1155/2020/4812759 |
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