Invasive Thymoma with Pure Red Cell Aplasia and Amegakaryocytic Thrombocytopenia

Abstract

We here describe a case involving a 67-yearold female patient who was referred to our hospital due to severe anemia (hemoglobin, 5.0 g/dL), thrombocytopenia (platelet count, 0.6 × 104/μL), and a mediastinal shadow with calcification noted on X-ray. On admission, an anterior mediastinal tumor was detected, and bone marrow biopsy revealed few megakaryocytes and severely reduced numbers of erythroid cells. The diagnosis was thymoma with pure red cell aplasia (PRCA) and acquired amegakaryocytic thrombocytopenia (AAMT). On Day 8 of admission, the patient received immunosuppressive therapy together with cyclosporine for the 2 severe hematologic diseases, which were stabilized within 2 months. Subsequently, total thymectomy was performed. The diagnosis of the tumor invading the left lung was invasive thymoma, Masaokakoga stage III. The histological diagnosis was World Health Organization type AB. Thymoma accompanied with PRCA and AAMT is very rare, and, based on our case, immunotherapeutic therapy for the hematologic disorders should precede surgical intervention.

Acquired Amegakaryocytic Thrombocytopenia and Pure Red Cell Aplasia in Thymoma

“…Our patient with severe thrombocytopenia, however, had a bone marrow depleted of megakaryocytes and erythroid precursors with normal functioning myeloid precursors that differentiated it from aplastic anemia or immune thrombocytopenic purpura. These findings suggestive of concomitant PRCA and AAMT are rarely reported in thymoma [ 12 – 14 ].…”

“…This could represent the natural progression of the disease into aplastic anemia manifested as pancytopenia [ 14 ]. Furthermore, with our patient refusing further treatment after just nine days, the effectiveness, or lack thereof, of cyclosporine-based therapy could not be ascertained as prior reports had shown a treatment duration of 8 to 20 weeks before the hematological parameters stabilized [ 12 , 13 ].…”

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.…”

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].…”