Pure red cell aplasia associated with thymoma: clinical insights from a 50-year single-institution experience.
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.
Thompson
Carrie A
CA
Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA. thompson.carrie@mayo.edu
Steensma
David P
DP
D000276:Adjuvants, Immunologic / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000961:Antilymphocyte Serum / Q000627:therapeutic use; D003131:Combined Modality Therapy / Q000379:methods; D016572:Cyclosporine / Q000627:therapeutic use; D004920:Erythropoiesis / Q000502:physiology; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte / Q000235:genetics; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications; D012010:Red-Cell Aplasia, Pure / Q000150:complications* / Q000276:immunology / Q000601:surgery; D013945:Thymoma / Q000150:complications* / Q000276:immunology / Q000601:surgery; D013953:Thymus Neoplasms / Q000150:complications* / Q000276:immunology / Q000601:surgery; D016896:Treatment Outcome
D000276:Adjuvants, Immunologic; D000961:Antilymphocyte Serum; D007166:Immunosuppressive Agents; D016572:Cyclosporine