L’érythroblastopénie : diagnostic, classification, traitement

Lobbes, Hervé

doi:10.1016/j.revmed.2022.10.385

Cited by 5 publications

(3 citation statements)

References 59 publications

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“…The definitive remission, though, may need adjuvant treatments, such as medications and bone marrow transplant, as seen in the presented case. Is important, then, to be aware of the possibility of PRCA in the face of a Thymoma and other-wise as well; be aware of the possibility of a Thymoma being the etiology for a PRCA in clinical investigation [11,12].…”

Section: Discussionmentioning

confidence: 99%

Thymectomy in Red Cell Aplasia - A Case Report

Santos Sena,

Silva de Oliveira,

Malafaia Ferreira

et al. 2023

ACMCR

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1.1. Background: Pure Red Cell Aplasia (PRCA) is a very rare syndrome responsible for severe anemia due to absence or significant reduction of erythroid precursors in the bone marrow. It’s etiology is variable, ranging from a congenital disease to an acquired one. Secondary acquired PRCA may be associated with several conditions, including Thymic Tumors. 1.2. Case Presentation: A 42-year-old male presented to the Internal Medicine department with constitutional symptoms: non-intentional weight-loss, palpitations, somnolence. Clinical investigation showed a severe normocytic normochromic anemia, with significant reticulocytopenia. Due to clinical status, several blood transfusions were needed. Further investigation with bone marrow biopsy showed intact precursors for platelets and leukocytes, with absence of erythroid precursors; the primary diagnostic hypothesis was Pure Red Cell Aplasia, with no etiology well defined, however. In order to rule out other conditions, imaging scans were performed; the chest CT showed a large mediastinal expansive formation, compatible with Thymoma. Patient was submitted to Thymectomy by the Thoracic Surgery team, with posterior improvement of clinical and hemodynamic status. Even so, for definitive remission of the PRCA, a bone marrow transplant was needed. The patient was successfully treated and remains healthy so far.

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Section: Discussionmentioning

confidence: 99%

Thymectomy in Red Cell Aplasia - A Case Report

Santos Sena,

Silva de Oliveira,

Malafaia Ferreira

et al. 2023

ACMCR

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“…[3,6] While rare, other pathogens, including Epstein–Barr virus (EBV), cytomegalovirus (CMV), hepatitis viruses, and bacteria, have also been reported. [7]…”

Section: What Is Prca?mentioning

confidence: 99%

“…[58] Notably, aPRCA cases resulting from the administration of recombinant epoetin alfa significantly decreased after improvements were made to the syringe packaging. [2,7]…”

Section: Why Does the Abnormal Attack Happen?mentioning

confidence: 99%

Acquired pure red cell aplasia: unraveling the immune pathogenesis

Liu,

Zhang,

Dong

et al. 2023

J Bio-X Res

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Acquired pure red cell aplasia (aPRCA) is a rare hematological disorder characterized by normochromic, normocytic anemia, reticulocytopenia, and the absence of erythroblasts. The pathogenesis of aPRCA has remained elusive. This review delves into the intricate web of immune mechanisms underlying the development of this enigmatic condition. By exploring immune responses, cytotoxic effects, and antibody-mediated processes, we dissect the immune-driven assault on erythroid progenitors. The classification of aPRCA, including its primary and secondary forms, is elucidated, with a particular emphasis on etiological factors such as viruses, drugs, thymoma, and large granular lymphocytic leukemia. Furthermore, we discuss the implications of cytogenetic changes in erythroid progenitors and immune cells in the pathophysiology of aPRCA. This comprehensive overview aims to shed light on the complex interplay between immune dysregulation and erythroid failure in aPRCA, offering insights that will be crucial for better understanding and treating this disease.

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Immunoglobuline polivalenti per via endovenosa

Papo

2024

EMC - AKOS - Trattato di Medicina

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L’érythroblastopénie : diagnostic, classification, traitement

Cited by 5 publications

References 59 publications

Thymectomy in Red Cell Aplasia - A Case Report

Thymectomy in Red Cell Aplasia - A Case Report

Acquired pure red cell aplasia: unraveling the immune pathogenesis

Immunoglobuline polivalenti per via endovenosa

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