“Almost Bleeding to Death”: The Conundrum of Acquired Amegakaryocytic Thrombocytopenia

Abstract

Acquired amegakaryocytic thrombocytopenia (AAT) is a rare hematological disorder causing severe thrombocytopenia and bleeding. Previous in vitro studies postulated both cell-mediated suppression of megakaryocytopoiesis in early megakaryocytic progenitor cells and humoral-mediated suppression by anti-thrombopoietin antibodies as possible etiologies of AAT. Patients with AAT usually present with severe bleeding and thrombocytopenia that is unresponsive to steroids and intravenous immunoglobulin (IVIG). Although standard guidelines have not been established for management of AAT, a few case reports have indicated a response to immunosuppressive treatment. The prompt recognition of this disease entity is essential in view of the substantial risk of morbidity and mortality from excessive bleeding. We report a case of AAT successfully treated with equine antithymocyte globulin (ATG) and cyclosporine (CSP).

Marked rebound thrombocytosis in response to glucocorticoids in a patient with acquired amegakaryocytic thrombocytopenia

“…As therapeutic strategies for AATP, glucocorticoid, 1 , 3 , 4 , 7 , 8 , 10 - 12 , 22 , 23 , 25 , 26 high-dose IVIg, 1 , 7 , 8 , 22 , 23 anti-thymocyte globulin, 4 , 9 cyclosporine A, 7 , 9 - 12 , 23 rituximab, 10 , 11 splenectomy, 8 allogeneic hematopoietic stem cell transplantation, 8 and agonists for c-Mpl, 10 androgens, 5 and vincristine 7 , 8 have been employed. Of these, glucocorticoid therapy, which is widely used and effective for ITP, is less effective for AATP.…”

Immune‐mediated thrombocytopenia induced with durvalumab after chemoradiotherapy in a non‐small cell lung cancer patient: A case report

“…In the present case, we measured neither serum TPO levels nor anti‐c‐mpl autoantibodies because there was no serum left before treatment. In addition, AATP could be the precursor for aplastic anemia, 11 myelodysplastic syndromes (MDS), 12 or acute leukemia 13 . Although there is no standard treatment guideline for AATP, anecdotal episodes succeeding in the treatment with cyclosporine 13 and TPO receptor agonist such as eltrombopag or romiplostim, 14 were reported.…”

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

“…The approach to AAT treatment is complex, and a variety of different therapies have been proposed, ranging from androgens [1,2,3,4,16,17,18] to immunosuppressive agents such as corticosteroids [3,7,19], azathioprine [20], and ATG, or chemotherapeutic agents (cyclophosphamide, CSA, and busulfan) [1,2,4,7,17], while IVIg, plasmapheresis and splenectomy have also been tried [1,4,5,17]. Biological therapies, e.g., rituximab or recombinant interleukin-11, were used in a few cases [2,21], while attempts to stimulate thrombopoiesis with TPO receptor agonists have also been reported [1,4,5,18].…”