Successful Treatment of Amegakaryocytic Thrombocytopenia with Azathioprine

Sharma

Case Reports in Hematology

et al. 2018

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.

Section: Discussionmentioning

confidence: 99%

Acquired Amegakaryocytic Thrombocytopenia and Pure Red Cell Aplasia in Thymoma

Sharma

Case Reports in Hematology

et al. 2018

“…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].…”

Section: Discussionmentioning

confidence: 99%

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

Levy¹,

Laor²,

Jiries³

et al. 2018

Acta Haematol

Acquired amegakaryocytic thrombocytopenia (AAT), a rare entity characterized by severe thrombocytopenia and the absence of megakaryocytes in the bone marrow, may mimic or precede the diagnosis of aplastic anemia (AA). Here, we describe a patient who presented with apparent Epstein-Barr virus (EBV)-associated immune thrombocytopenia resistant to several lines of therapies, which was in fact a form of AAT with some features of AA. He eventually responded to therapy with eltrombopag, cyclosporine A (CSA), and antithymocyte globulin (ATG) and recovered completely. EBV infection is known to cause a variety of benign and malignant hematologic disorders, including bone marrow failure. However, to the best of our knowledge, this is the first case report of EBV-associated AAT. Treatment options for AAT are still not well defined, and even response to eltrombopag together with CSA and ATG does not always imply successful therapy. The natural history of EBV infection may well be sufficient to explain unexpected eventual recovery.

Case Reports in Hematology

“…Patients can achieve a durable remission, develop a long relapsing and remitting course, progress to aplastic anemia, or require a bone marrow transplantation to achieve remission [8–10]. AAT may be associated with other hematologic and rheumatologic conditions, including MDS, aplastic anemia, acute myeloid leukemia, and systemic lupus erythematosus [11, 12]. The exact mechanism has not been elucidated; however, the presence of antithrombopoietin IgG antibodies in patients with AAT suggests a dysregulated humoral immunity [13].…”

Section: Discussionmentioning

confidence: 99%

“…Other therapies for AAT have included rituximab [11], danazol [20], azathioprine [12], and bone marrow transplant [10], with variable success. Treatment with rituximab resulted in a rapid platelet response in one case report, although the patient ultimately relapsed [11].…”

Section: Managementmentioning

confidence: 99%

“…Danazol was used in a case of cyclic AAT, with complete remission achieved for at least 10 months [20]. Azathioprine treatment resulted in resolution of clinical symptoms at 4 weeks and complete remission at 6 weeks [12]. A more aggressive approach with myeloablative chemotherapy followed by a fully HLA-matched allogeneic bone marrow transplant has also been reported to be successful [10].…”

Section: Managementmentioning

confidence: 99%

See 1 more Smart Citation

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

Brown

Babiker

Cantu

et al. 2014

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).