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Intracranial extramedullary hematopoiesis in a patient with beta-thalassemia presenting with head trauma

BACKGROUND: Beta-thalassemia is an inherited hemoglobinopathy, whereby reduced or absent expression of beta-globin genes causes impaired erythropoiesis. Extramedullary hematopoiesis (EMH) occurs in 1% of all patients with beta-thalassemia major receiving regular transfusions and is exceedingly rare...

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Autores principales: Nichols, Noah M., Hackett, Ashia M., Carrasquilla, Alejandro, Solodnik, Paul, Byun, Hang, Jones, Salazar, Hickman, Zachary L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Scientific Scholar 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9699845/
https://www.ncbi.nlm.nih.gov/pubmed/36447872
http://dx.doi.org/10.25259/SNI_730_2022
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author Nichols, Noah M.
Hackett, Ashia M.
Carrasquilla, Alejandro
Solodnik, Paul
Byun, Hang
Jones, Salazar
Hickman, Zachary L.
author_facet Nichols, Noah M.
Hackett, Ashia M.
Carrasquilla, Alejandro
Solodnik, Paul
Byun, Hang
Jones, Salazar
Hickman, Zachary L.
author_sort Nichols, Noah M.
collection PubMed
description BACKGROUND: Beta-thalassemia is an inherited hemoglobinopathy, whereby reduced or absent expression of beta-globin genes causes impaired erythropoiesis. Extramedullary hematopoiesis (EMH) occurs in 1% of all patients with beta-thalassemia major receiving regular transfusions and is exceedingly rare intracranially. CASE DESCRIPTION: We report a case of a male in his 20s with beta thalassemia who presented with head trauma found to have intracranial EMH mimicking multiple extra-axial hematomas. Making the correct diagnosis was critical in avoiding prolonged neuromonitoring and unnecessary interventions. CONCLUSION: Intracranial extramedullary hematopoietic pseudotumor is an exceedingly rare entity and seldom appears in a neurosurgeon’s differential diagnosis. This case illustrates how this condition can easily mimic an acute intracranial hemorrhage in a patient with beta-thalassemia who presents with head trauma. We review the topic to further inform clinicians who may encounter this condition in their practice.
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spelling pubmed-96998452022-11-28 Intracranial extramedullary hematopoiesis in a patient with beta-thalassemia presenting with head trauma Nichols, Noah M. Hackett, Ashia M. Carrasquilla, Alejandro Solodnik, Paul Byun, Hang Jones, Salazar Hickman, Zachary L. Surg Neurol Int Case Report BACKGROUND: Beta-thalassemia is an inherited hemoglobinopathy, whereby reduced or absent expression of beta-globin genes causes impaired erythropoiesis. Extramedullary hematopoiesis (EMH) occurs in 1% of all patients with beta-thalassemia major receiving regular transfusions and is exceedingly rare intracranially. CASE DESCRIPTION: We report a case of a male in his 20s with beta thalassemia who presented with head trauma found to have intracranial EMH mimicking multiple extra-axial hematomas. Making the correct diagnosis was critical in avoiding prolonged neuromonitoring and unnecessary interventions. CONCLUSION: Intracranial extramedullary hematopoietic pseudotumor is an exceedingly rare entity and seldom appears in a neurosurgeon’s differential diagnosis. This case illustrates how this condition can easily mimic an acute intracranial hemorrhage in a patient with beta-thalassemia who presents with head trauma. We review the topic to further inform clinicians who may encounter this condition in their practice. Scientific Scholar 2022-11-18 /pmc/articles/PMC9699845/ /pubmed/36447872 http://dx.doi.org/10.25259/SNI_730_2022 Text en Copyright: © 2022 Surgical Neurology International https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
spellingShingle Case Report
Nichols, Noah M.
Hackett, Ashia M.
Carrasquilla, Alejandro
Solodnik, Paul
Byun, Hang
Jones, Salazar
Hickman, Zachary L.
Intracranial extramedullary hematopoiesis in a patient with beta-thalassemia presenting with head trauma
title Intracranial extramedullary hematopoiesis in a patient with beta-thalassemia presenting with head trauma
title_full Intracranial extramedullary hematopoiesis in a patient with beta-thalassemia presenting with head trauma
title_fullStr Intracranial extramedullary hematopoiesis in a patient with beta-thalassemia presenting with head trauma
title_full_unstemmed Intracranial extramedullary hematopoiesis in a patient with beta-thalassemia presenting with head trauma
title_short Intracranial extramedullary hematopoiesis in a patient with beta-thalassemia presenting with head trauma
title_sort intracranial extramedullary hematopoiesis in a patient with beta-thalassemia presenting with head trauma
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9699845/
https://www.ncbi.nlm.nih.gov/pubmed/36447872
http://dx.doi.org/10.25259/SNI_730_2022
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