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).
Highlights
Acquired amegakaryocytic thrombocytopenia (AAT) is a rare hematological disorder causing severe thrombocytopenia and bleeding
We report a case of AAT successfully treated with equine antithymocyte globulin (ATG) and cyclosporine (CSP)
Other etiologies of thrombocytopenia relate to increased destruction of platelets, as in idiopathic thrombocytopenia purpura (ITP), thrombocytopenic purpura (TTP), autoimmune diseases, hemolytic uremic syndrome, medications, severe bacterial infections, and pregnancy
Summary
A 40-year-old woman with a past medical history of migraine headaches presented to her primary care physician with the chief complaint of “I am almost bleeding to death” and endorsed a history of fatigue, easy bruising, and frequent nosebleeds. A complete blood count (CBC) revealed a platelet count of 12 × 109/L (normal, 150–425 × 109/L), and she was referred to a hematologist. She was initially diagnosed with idiopathic thrombocytopenia purpura (ITP) and was treated with prednisone 60 mg per day for one week, without improvement in platelet count. Figure 1: ((a)-(b)) The peripheral blood shows normal mature neutrophils and lymphocytes with normal red blood cell morphology. The patient was admitted and received a four-day course of equine ATG (40 mg/kg/day) followed by a 6-month outpatient course of CSP She received a two-week course of methylprednisolone to ameliorate symptoms of serum sickness from ATG administration. By 4 months after initiation of ATG/CSP treatment the platelet level had increased to 115, 000 × 109/L and remained stable thereafter (Figure 2)
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