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

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...

Full description

Saved in:
Bibliographic Details
Main Authors: Gabrielle Elena Brown, Hani M. Babiker, Carlos L. Cantu, Andrew M. Yeager, Ravitharan Krishnadasan
Format: Article
Language:English
Published: Wiley 2014-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2014/806541
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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).
ISSN:2090-6560
2090-6579