Acquired Amegakaryocytic Thrombocytopenia and Pure Red Cell Aplasia in Thymoma

Abstract

Association of thymoma with myasthenia gravis, pure red cell aplasia, and aplastic anemia is well documented. However, thymoma complicated by acquired amegakaryocytic thrombocytopenia (AAMT) is rarely reported. Here, we present a case of a 60-year-old male with past medical history of recurrent invasive thymoma who presented with cough and blood in sputum. He was found to have severe normocytic normochromic anemia and thrombocytopenia that did not improve with intravenous steroids or multiple transfusions of red cells and platelets. Subsequent bone marrow biopsy showed severely depleted megakaryocytes and erythroid precursor cells with relative myeloid hyperplasia suggestive of amegakaryocytic thrombocytopenia and red cell aplasia. He was started on oral cyclosporine but subsequently developed leukopenia and refused any further treatment or diagnostic procedures and left the hospital against medical advice. AAMT, thus, may be a very early presentation of impending aplastic anemia, and treating physicians need to be aware of this entity.

Severe Aplastic Anemia Developed after Thymectomy: A Case Report and Literature Review

“…Studies have demonstrated that increases in cell count may require different time intervals, besides exhibiting different follow-up times to evaluate disease recurrence. Most patients had an effective response to treatment; however, in two, the response was not described [6,10]. In addition to our study, we were only able to identify one case where a patient underwent allogeneic stem cell transplantation and, in contrast to our case, longterm cure was documented [9].…”

“…Two patients were diagnosed with thymoma in the presence of signals and symptoms of myasthenia gravis [2,6], while the other cases reported no systemic abnormalities other than hematologic ones. Regarding hematological progress, in one case aplastic anemia was preceded by immune thrombocytopenic purpura [4] and in three others by the association of pure red cell aplasia and amegakaryocytic thrombocytopenia [6,9,10]. e present case was only preceded by isolated amegakaryocytic thrombocytopenia.…”

“…A literature review was conducted in the PubMed database with English language case reports of patients who developed aplastic anemia after thymectomy. After reviewing publications from 1993 to 2018, we were able to identify a total of nine publications [2][3][4][5][6][7][8][9][10] with a total of ten cases that we summarize in Table 1. Six patients were male and four females.…”

“…Although being an uncommon association, over the last years several case reports of aplastic anemia following thymecotymy have been published. However, development of such autoimmune manifestation after successful surgical resection of the tumor is rarely described in the literature [2][3][4][5][6][7][8][9][10]. We report a case of a 53-year-old man with severe aplastic anemia three years after thymectomy with no signs of disease recurrence.…”

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.…”