Acquired amegakaryocytic thrombocytopenic purpura

Abstract

Acquired amegakaryocytic thrombocytopenia is an unusual hematologic disorder characterized by thrombocytopenia in association with markedly diminished bone marrow megakaryocytes. We report a case that responded to treatment with cyclosporine but not to IL-11. The bone marrow biopsy, repeated after resolution of thrombocytopenia, showed normal number of megakaryocytes. Am.

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

“…These defects in maturation and differentiation may be idiopathic or caused by exogenous (virus, toxin, or drug exposure) [1] or immune (humoral or cellular) [1,2,4] mechanisms. Antibodies to CFU-MK (megakaryocyte progenitor) have been described mainly in association with other autoimmune diseases, such as systemic lupus erythematosus [1,4,5,6].…”

“…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].…”

“…Viral infections have been described as potential causes of this disorder [2], but, to the best of our knowledge, Epstein-Barr virus (EBV)-induced AAT has not been reported until now. Here, we present a patient with potentially life-threatening bleeding due to this disorder and review available literature on this topic.…”

Treatment of acquired amegakaryocytic thrombocytopenic purpura with romiplostim

“…Once AATP is diagnosed, a therapeutic trial of cyclosporine, with or without antithymocyte globulin (ATG), is indicated [1]. Allogeneic bone marrow transplantation (BMT) is considered to be an appropriate treatment for patients with refractory or progressive AATP, who are relatively young and have matched siblings [2].…”

“…Of the drugs that the patient had received, both aspirin and allopurinol had previously been given to patients with AATP [1,3,4]. However, since it was impossible to show that none of the other drugs were associated with AATP, all medications were discontinued [5].…”