Thymoma Complicated by Acquired Amegakaryocytic Thrombocytopenia and Pure Red Cell Aplasia

Abstract

Although the association of pure red cell aplasia (PRCA) and aplastic anemia with thymoma is well-known, acquired amegakaryocytic thrombocytopenia (AAMT) is not a recognized paraneoplastic manifestation of thymoma. This report discusses a patient with recurrent thymoma complicated by myasthenia gravis, PRCA, and AAMT. Both PRCA and AAMT are diagnosed after a thymoma recurrence, 11 years after complete resection of the initial tumor and 9 months after chemotherapy for the relapsed disease. Both PRCA and AAMT responded to immunosuppression with cyclosporine, corticosteroid, and an abbreviated course of antithymocyte globulin, achieving a very good erythroid response and a complete remission for AAMT, suggesting that AAMT, although extremely rare, can be an immune-mediated paraneoplastic manifestation of thymoma.

Pure red cell aplasia and amegakaryocytic thrombocytopenia in thymoma: The uncharted territory

“…Between these cases, four patients were able to improve their cytopenias whereas in the remaining two cases information about follow‐up were not available and one patient left the hospital few days after therapy initiation . Notably, in three cases including ours PRCA and amegakaryocytic thrombocytopenia (AT) progressed to aplastic anemia despite immunosuppression, thus empowering the theory, previously cited in the other reports, that PRCA and AT may be early presentation of complete aplasia.…”

“…3 Our case is an example where the addition of a TPO agonist did not provide an hematological recovery, whereas in the latest years this molecule has been proven effective in the treatment of severe aplastic anemia (SAA) both as a single agent in SAA refractory to IST or in frontline combination with cyclosporine/horse ATG. 4,5 Between these cases, four patients were able to improve their cytopenias [6][7][8][9] whereas in the remaining two cases information about follow-up were not available and one patient left the hospital few days after therapy initiation. 9,10 Notably, in three cases including ours PRCA and amegakaryocytic thrombocytopenia (AT) progressed to aplastic anemia despite immunosuppression, thus empowering the theory, previously cited in the other reports, that PRCA and AT may be early presentation of complete aplasia.…”

Invasive Thymoma with Pure Red Cell Aplasia and Amegakaryocytic Thrombocytopenia

“…Limited case reports on the coexistence of thymoma, PRCA, and AAMT, a seen in our case, have been published. 3,6-8 These cases are summarized in Table 1. Unlike in our case, however, in which all conditions occurred simultaneously, in each of these previous cases, the diseases occurred separately, indicating that thymoma-related hematologic disorders can change over time.…”

Amegakaryocytic Thrombocytopenia and Subsequent Aplastic Anemia Associated with Apparent Epstein-Barr Virus Infection

“…Viral infections, toxins, drug exposure, lymphoproliferative disorders, thymoma, and rheumatologic diseases have all been described, mostly as individual case reports [2,5,6,8,10,11]. The herpesvirus EBV may have various hematologic complications such as Burkitt lymphoma, autoimmune hemolytic anemia, hemophagocytic syndrome, or thrombocytopenia [12].…”