Loading...
 

Volume 34, Issue 4, December 2023



Sign in to download the Issue in PDF format.

Mediterr J Rheumatol 2023;34(4):537-43
Refractory Acquired Amegakaryocytic Thrombocytopenia with Rapid Progression to Aplastic Anaemia in SLE
Authors Information

1State University of New York Downstate Health Sciences University, College of Medicine, Brooklyn, NY, USA

2State University of New York Downstate Health Sciences University, Department of Internal Medicine, Brooklyn, NY, USA

3NYC Health and Hospitals/Kings County Hospital Centre, Department of Rheumatology, NY, USA

B Hadid, A Kodza, SC Suresh, A Feoktistov

Abstract

Acquired amegakaryocytic thrombocytopenia (AAMT) is a rare cause of thrombocytopenia seen in systemic lupus erythematosus (SLE) that is frequently misdiagnosed as immune thrombocytopenic purpura (ITP). Often patients do not respond to standard ITP treatment. Prompt bone marrow biopsy and further workup should ensue as it is a diagnosis of exclusion. While no standard guidelines exist, the mainstay of treatment is immunosuppressive therapy. Some cases are refractory and should have a follow-up biopsy, typically showing worsening disease. The exact pathogenesis is unclear; multiple mechanisms may be involved, suggesting AAMT may be a syndrome of various aetiologies rather than a distinct pathology. A common complication is aplastic anaemia, and the patient may need a haematopoietic stem cell transplant (HSCT). We present a young man with severe refractory AAMT in the setting of SLE that progressed to aplastic anaemia and required an HSCT. We then discuss and interpret the literature on AAMT.

Cite this article as: Hadid B, Kodza A, Channapatna Suresh S, Feoktistov A. Refractory Acquired Amegakaryocytic Thrombocytopenia with Rapid Progression to Aplastic Anemia in SLE. Mediterr J Rheumatol 2023;34(4):537-43

Article Submitted: 09 Dec 2022; Revised Form: 15 May 2023; Article Accepted: 16 May 2023; Available Online: 11 Aug 2023

This work is licensed under a Creative Commons Attribution 4.0 International License.

©2023 The Author(s).

https://doi.org/10.31138/mjr.110823.raa