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Acquired pure red cell aplasia (PRCA) is a rare disorder of erythropoiesis that can develop in association with a thymoma. Optimal management of this subgroup is unclear, and there have been few series reporting long-term clinical outcomes. Here, we report features of 13 patients treated for PRCA associated with thymoma over 50 years at our institution. Surgical resection of the thymoma was insufficient for normalisation of erythropoiesis in all cases. T-cell gene rearrangement studies did not routinely demonstrate a clonal process, and ciclosporin and anti-thymocyte globulin were effective adjuvant treatments. However, treatment-related morbidity was high, with frequent infectious complications.

We analysed the cell surface expression of chemokine receptors and natural killer receptors (NKRs) in addition to conventional T- and natural killer (NK)-cell markers in patients with lymphoproliferative disease of granular lymphocytes (LDGL), and compared results between NK- and T-LDGL subgroups. The subjects of this study were 15 LDGL patients: four NK-LDGL and 11 T-LDGL [six CD8(+) T-cell receptor (TCR) alphabeta(+), four CD4(+) TCRalphabeta(+) and one CD8(+) TCRgammadelta(+)] cases. Flow cytometric analysis showed that the expanding cells had a common phenotype, CD45RA(+) CD27(-) CD28(-) CCR7(-), in NK- and T-LDGL patients irrespective of differences in TCR status. There were no marked differences in the expression patterns of chemokine receptors between NK- and T-LDGL patients. Although restricted NKR subsets were expressed on both NK- and T-large granular lymphocytes (LGLs), CD94 was the most widely expressed marker. These findings may be unique to cells of LDGL cases, because normal CD56(dim) NK cells frequently express killer cell immunoglobulin-like receptors. Furthermore, analysis of NKR expression was repeated over an interval of more than 6 months, and fluctuations of NKR repertoire in the LGL clones were minimal.

A patient with acquired pure red cell aplasia and agnogenic myeloid metaplasia (AMM) was treated with rHuEPO. She became transfusion independent. Weekly injections of rHuEPO have maintained the response without side-effects or disease progression. In addition, marrow fibrosis and splenomegaly have decreased. Therefore, rHuEPO may be effective therapy for some patients with acquired PRCA and/or AMM.