Abstract
Pure red cell aplasia is a rare cause of anemia, caused by an absence of red blood cell precursors in the bone marrow. It is usually a paraneoplastic syndrome, associated most commonly with large-cell granular lymphocyte leukemia but also thymoma. For patients who present both pure red cell aplasia and thymoma, thymectomy leads to an initial remission of the aplasia in 30% of cases. However, sustained remission may require the addition of medications such as corticosteroids, cyclosporine, or cyclophosphamide. We present a case of pure red cell aplasia associated with a thymoma in an otherwise healthy 80 year-old woman.
Highlights
Pure red cell aplasia is a rare cause of anemia, caused by an absence of red blood cell precursors in the bone marrow
Surgery leads to an initial remission of the aplasia in 30% of cases, sustained remission may require the addition of medications such as corticosteroids, cyclosporine, or cyclophosphamide.[1,3]
Pure red cell aplasia (PRCA) may sometimes be congenital but it is usually acquired and in this case is most frequently associated with hematologic disorders, especially large-cell granular lymphocyte leukemia (LGL)[2] and other hematologic malignancies; it may occur in the setting of viral infections, autoimmune disorders, and the use of certain medications.[3]
Summary
Pure red cell aplasia is a rare cause of anemia, caused by an absence of red blood cell precursors in the bone marrow. It is often a paraneoplastic syndrome which may be associated with a thymoma.[1,2] Thymectomy is usually indicated in these patients. Bone marrow aspirate and biopsy revealed an abundance of myeloid cells and some megakaryocytes, but a complete absence of erythroid precursors (Figure 1A,B). This was consistent with the diagnosis of pure red cell aplasia. Repeat imaging did not show any recurrent thymoma, and remission was achieved once more with the addition of cyclosporine
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
