Clinical Characteristics and Outcomes of 11 Patients with Pure Red Cell Aplasia at a Single Institution Over a 13-Year Period
Autor: | Yuri Nagahiro, Akira Ueda, Naoko Yokota-Ikeda, Shigeyoshi Makino, Noriaki Kawano, Yoshiya Shimao, Kiyoshi Yamashita, Shuro Yoshida, Daisuke Himeji, Kazuya Shimoda, Shigehiro Uezono |
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Rok vydání: | 2013 |
Předmět: |
Adult
Male medicine.medical_specialty Anemia Prednisolone medicine.medical_treatment Pure red cell aplasia Red-Cell Aplasia Pure urologic and male genital diseases Gastroenterology Parvoviridae Infections Internal medicine Internal Medicine medicine Humans Aged Retrospective Studies Aged 80 and over business.industry Standard treatment Parvovirus infection Immunoglobulins Intravenous Infant Retrospective cohort study Thymus Neoplasms General Medicine Middle Aged medicine.disease Surgery Treatment Outcome medicine.anatomical_structure Cyclosporine Kidney Failure Chronic Female Bone marrow Hemodialysis business Immunosuppressive Agents medicine.drug |
Zdroj: | Internal Medicine. 52:2025-2030 |
ISSN: | 1349-7235 0918-2918 |
Popis: | Objective Pure red cell aplasia (PRCA) is a rare clinical entity characterized by anemia due to severe suppression of erythroid precursors, where the other cell lineages in the bone marrow remain morphologically normal. A standard treatment has not yet been established for PRCA due to the rarity of this condition. Recently, however, the administration of either cyclosporine (CSP) or prednisolone (PSL) has been reported to be an effective treatment for PRCA. Methods To clarify the clinical characteristics of PRCA, 11 PRCA cases were retrospectively analyzed over a 13-year period at our institution. Since acute PRCA was found to be self-limiting, we administered the immunosuppressive treatment of CSP or PSL after providing supportive care for 4 weeks. Results The causes of PRCA were as follows: idiopathic (3), acute parvovirus infection (1), chronic parvovirus infection (3), thymic tumor (3), and end-stage renal disease with hemodialysis (1). Complete remission (CR) was achieved for 4 of the 5 patients treated with CSP, for 2 of the 3 patients with chronic parvovirus infection treated by immunoglobulin (Ig), and for all 3 patients treated with PSL. During the follow-up periods, 4 of the 11 patients relapsed. Complete remission was achieved a second time in all 4 cases by therapies that were more intensive and had longer administration periods than those provided during initial treatment. Consequently, 9 of the 11 patients were still alive (80%) after 5 years. Conclusion Depending on the cause of the PRCA, treatment with CSP, PSL, or Ig was found to be effective in most PRCA cases. |
Databáze: | OpenAIRE |
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