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

Abstract

Association between thymoma and pure red cell aplasia is already well‐documented in literature whereas acquired amegakaryocytic thrombocytopenia is rarely reported. In this case, even with the addition of eltrombopag to standard immunosuppression, the cytopenias did not improve, probably due to the lack of surgical resection of the tumor.

Radiation‑induced pure red cell aplasia combined with acquired amegakaryocytic thrombocytopenia in a thymoma after rapid response to radiotherapy: A case report and literature review

“…Thymoma has a strong correlation with paraneoplastic immune disorders, and PRCA is the most common hematologic paraneoplastic manifestation of thymoma (12). Thymoma is rarely associated with AAMT, with only 8 cases reported in the literature (4)(5)(6)(7)(8)(9)(10)(11). From the available reported literature, thymoma in combination with AAMT was always accompanied by PRCA, and in three of these cases the patients eventually developed aplastic anemia (AA) despite immunotherapy (6,9,10).…”

“…Thymoma is rarely associated with AAMT, with only 8 cases reported in the literature (4)(5)(6)(7)(8)(9)(10)(11). From the available reported literature, thymoma in combination with AAMT was always accompanied by PRCA, and in three of these cases the patients eventually developed aplastic anemia (AA) despite immunotherapy (6,9,10). Therefore, we support the theory that PRCA and AAMT may be early manifestations of aplastic anemia (10), and early intervention is needed for patients with combined PRCA and AAMT to avoid further progression to AA.…”

“…Patients with AAMT in combination with PRCA may present with anemia, such as weakness, fatigue, pallor or dyspnea, or with bleeding due to thrombocytopenia, such as mucocutaneous or skin bleeding (9). The CBC test showed markedly reduced hemoglobin and platelets, as well as markedly reduced reticulocytes; bone marrow biopsy shows severe depletion of megakaryocytes and red lineage precursor cells, while myeloid cells are normal (4)(5)(6)(7)(8)(9)(10)(11)18). In this case, the patient was presented with weakness, bleeding gums and bleeding spots on the skin, while her CBC tests and bone marrow aspiration were consistent with previous reports.…”

“…It is known that approximately 2-5% of patients with thymoma develop pure red cell aplasia (PRCA) (2,3), but the concurrent combination of acquired amegakaryocytic thrombocytopenia (AAMT) is rarely reported. The most common treatment used in PRCA complicated with AAMT included cyclosporine, alone or in combination with corticosteroid therapy (4)(5)(6)(7)(8)(9). The other salvage treatment strategies include antithymocyte globulin, bone marrow transplantation, erythropoietin, iterative transfusions, platelet transfusion and eltrombopag (4)(5)(6)(7)(9)(10)(11).…”

“…The most common treatment used in PRCA complicated with AAMT included cyclosporine, alone or in combination with corticosteroid therapy ( 4-9 ). The other salvage treatment strategies include antithymocyte globulin, bone marrow transplantation, erythropoietin, iterative transfusions, platelet transfusion and eltrombopag ( 4-7 , 9-11 ). Here, this novel report details the course of our clinical treatment of PRCA and AAMT induced by thymoma radiotherapy.…”