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A Curious Case of Hemolytic Anemia with Pseudoreticulopenia

Herein, we present a unique case of a Coombs-negative, steroid-refractory autoimmune hemolytic anemia (AIHA) complicated by pseudoreticulopenia, describe its clinical presentation, histopathologic findings, and management, and review the salient literature. Coombs-negative, steroid-refractory AIHAs...

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Autores principales: Mayer, Scott, Srinivasan, Nikhilesh, Nguyen, Jenny, Spilman, Ryan, Scherbak, Dmitriy
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9050323/
https://www.ncbi.nlm.nih.gov/pubmed/35495180
http://dx.doi.org/10.1155/2022/6423143
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author Mayer, Scott
Srinivasan, Nikhilesh
Nguyen, Jenny
Spilman, Ryan
Scherbak, Dmitriy
author_facet Mayer, Scott
Srinivasan, Nikhilesh
Nguyen, Jenny
Spilman, Ryan
Scherbak, Dmitriy
author_sort Mayer, Scott
collection PubMed
description Herein, we present a unique case of a Coombs-negative, steroid-refractory autoimmune hemolytic anemia (AIHA) complicated by pseudoreticulopenia, describe its clinical presentation, histopathologic findings, and management, and review the salient literature. Coombs-negative, steroid-refractory AIHAs represent fewer than 1% of all AIHAs. Diagnosis of the disease is difficult and often delayed due to the pursuit of alternate diagnoses following a negative Coombs test. However, when suspicion remains high for an autoimmune process, the super-Coombs test may be utilized for the diagnosis of AIHA that the traditional Coombs test fails to detect. A majority of cases respond to rituximab as the indicated second-line therapy, but delays in diagnosis and subsequent treatment may increase morbidity. Reticulopenia may be associated with AIHAs secondary to bone marrow dysfunction, but this patient had a normal function marrow confirmed on biopsy. Indeed, reticulopenia in this case was a diagnostic conundrum that further obscured the diagnosis and delayed treatment. Ultimately, reticulopenia was determined to be pseudoreticulopenia secondary to an alteration in the maturation of the erythroid lineage due to an independent, newly diagnosed pernicious anemia. The interaction of these multiple coexisting disease processes is not previously described in the literature. Increased physician awareness of steroid-refractory, Coombs-negative AIHA, and the development of pseudoreticulopenia as a laboratory finding in pernicious anemia may help to improve patient outcomes.
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spelling pubmed-90503232022-04-29 A Curious Case of Hemolytic Anemia with Pseudoreticulopenia Mayer, Scott Srinivasan, Nikhilesh Nguyen, Jenny Spilman, Ryan Scherbak, Dmitriy Case Rep Hematol Case Report Herein, we present a unique case of a Coombs-negative, steroid-refractory autoimmune hemolytic anemia (AIHA) complicated by pseudoreticulopenia, describe its clinical presentation, histopathologic findings, and management, and review the salient literature. Coombs-negative, steroid-refractory AIHAs represent fewer than 1% of all AIHAs. Diagnosis of the disease is difficult and often delayed due to the pursuit of alternate diagnoses following a negative Coombs test. However, when suspicion remains high for an autoimmune process, the super-Coombs test may be utilized for the diagnosis of AIHA that the traditional Coombs test fails to detect. A majority of cases respond to rituximab as the indicated second-line therapy, but delays in diagnosis and subsequent treatment may increase morbidity. Reticulopenia may be associated with AIHAs secondary to bone marrow dysfunction, but this patient had a normal function marrow confirmed on biopsy. Indeed, reticulopenia in this case was a diagnostic conundrum that further obscured the diagnosis and delayed treatment. Ultimately, reticulopenia was determined to be pseudoreticulopenia secondary to an alteration in the maturation of the erythroid lineage due to an independent, newly diagnosed pernicious anemia. The interaction of these multiple coexisting disease processes is not previously described in the literature. Increased physician awareness of steroid-refractory, Coombs-negative AIHA, and the development of pseudoreticulopenia as a laboratory finding in pernicious anemia may help to improve patient outcomes. Hindawi 2022-04-21 /pmc/articles/PMC9050323/ /pubmed/35495180 http://dx.doi.org/10.1155/2022/6423143 Text en Copyright © 2022 Scott Mayer et al. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Mayer, Scott
Srinivasan, Nikhilesh
Nguyen, Jenny
Spilman, Ryan
Scherbak, Dmitriy
A Curious Case of Hemolytic Anemia with Pseudoreticulopenia
title A Curious Case of Hemolytic Anemia with Pseudoreticulopenia
title_full A Curious Case of Hemolytic Anemia with Pseudoreticulopenia
title_fullStr A Curious Case of Hemolytic Anemia with Pseudoreticulopenia
title_full_unstemmed A Curious Case of Hemolytic Anemia with Pseudoreticulopenia
title_short A Curious Case of Hemolytic Anemia with Pseudoreticulopenia
title_sort curious case of hemolytic anemia with pseudoreticulopenia
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9050323/
https://www.ncbi.nlm.nih.gov/pubmed/35495180
http://dx.doi.org/10.1155/2022/6423143
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