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A large intrathoracic extramedullary hematopoiesis in alpha-thalassemia: A case report

RATIONALE: Extramedullary hematopoiesis (EMH) is a rare disease characterized by the formation of hematopoietic elements outside the bone marrow driven by several hematological disease. To the best of our knowledge, EMH is relatively common in patient with beta-thalassemia or hereditary spherocytosi...

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Autores principales: Chen, Jianan, Liu, Yuan-Ling, Lu, Min-zhen, Gao, Xing-Lin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6946295/
https://www.ncbi.nlm.nih.gov/pubmed/31689767
http://dx.doi.org/10.1097/MD.0000000000017612
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author Chen, Jianan
Liu, Yuan-Ling
Lu, Min-zhen
Gao, Xing-Lin
author_facet Chen, Jianan
Liu, Yuan-Ling
Lu, Min-zhen
Gao, Xing-Lin
author_sort Chen, Jianan
collection PubMed
description RATIONALE: Extramedullary hematopoiesis (EMH) is a rare disease characterized by the formation of hematopoietic elements outside the bone marrow driven by several hematological disease. To the best of our knowledge, EMH is relatively common in patient with beta-thalassemia or hereditary spherocytosis but rarely reported in patients with alpha-thalassemia. Here, we discuss a large intrathoracic EMH (measuring 95 mm × 66 mm) without presenting severe complications in alpha-thalassemia along with literature review. PATIENT CONCERNS: A 55-year-old Chinese female patient with alpha-thalassemia presented with ipsilateral pleural effusion and low hemoglobin level. DIAGNOSIS: Lung cancer was suspected at first and the mass was subjected to CT-guided percutaneous mediastinum biopsy and the pathology confirmed the final diagnosis of extramedullary hematopoiesis. INTERVENTIONS: Blood transfusion, thoracentesis and regular follow up were scheduled rather than surgical interventions or radiotherapy since our patient did not exhibit significant symptoms. OUTCOMES: After 6 months’ regular follow up, the patient exhibited no evidence of disease progress. LESSONS: EMH is frequently misdiagnosed and should be differentiated from other masses in thoracic cavity, especially when the underlying hematological disease is discovered. Treatment methods of EMH include surgical resection, hyper-transfusion, hydroxyurea, low-dose radiation or a combination of them.
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spelling pubmed-69462952020-01-31 A large intrathoracic extramedullary hematopoiesis in alpha-thalassemia: A case report Chen, Jianan Liu, Yuan-Ling Lu, Min-zhen Gao, Xing-Lin Medicine (Baltimore) 6700 RATIONALE: Extramedullary hematopoiesis (EMH) is a rare disease characterized by the formation of hematopoietic elements outside the bone marrow driven by several hematological disease. To the best of our knowledge, EMH is relatively common in patient with beta-thalassemia or hereditary spherocytosis but rarely reported in patients with alpha-thalassemia. Here, we discuss a large intrathoracic EMH (measuring 95 mm × 66 mm) without presenting severe complications in alpha-thalassemia along with literature review. PATIENT CONCERNS: A 55-year-old Chinese female patient with alpha-thalassemia presented with ipsilateral pleural effusion and low hemoglobin level. DIAGNOSIS: Lung cancer was suspected at first and the mass was subjected to CT-guided percutaneous mediastinum biopsy and the pathology confirmed the final diagnosis of extramedullary hematopoiesis. INTERVENTIONS: Blood transfusion, thoracentesis and regular follow up were scheduled rather than surgical interventions or radiotherapy since our patient did not exhibit significant symptoms. OUTCOMES: After 6 months’ regular follow up, the patient exhibited no evidence of disease progress. LESSONS: EMH is frequently misdiagnosed and should be differentiated from other masses in thoracic cavity, especially when the underlying hematological disease is discovered. Treatment methods of EMH include surgical resection, hyper-transfusion, hydroxyurea, low-dose radiation or a combination of them. Wolters Kluwer Health 2019-11-01 /pmc/articles/PMC6946295/ /pubmed/31689767 http://dx.doi.org/10.1097/MD.0000000000017612 Text en Copyright © 2019 the Author(s). Published by Wolters Kluwer Health, Inc. http://creativecommons.org/licenses/by/4.0 This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0
spellingShingle 6700
Chen, Jianan
Liu, Yuan-Ling
Lu, Min-zhen
Gao, Xing-Lin
A large intrathoracic extramedullary hematopoiesis in alpha-thalassemia: A case report
title A large intrathoracic extramedullary hematopoiesis in alpha-thalassemia: A case report
title_full A large intrathoracic extramedullary hematopoiesis in alpha-thalassemia: A case report
title_fullStr A large intrathoracic extramedullary hematopoiesis in alpha-thalassemia: A case report
title_full_unstemmed A large intrathoracic extramedullary hematopoiesis in alpha-thalassemia: A case report
title_short A large intrathoracic extramedullary hematopoiesis in alpha-thalassemia: A case report
title_sort large intrathoracic extramedullary hematopoiesis in alpha-thalassemia: a case report
topic 6700
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6946295/
https://www.ncbi.nlm.nih.gov/pubmed/31689767
http://dx.doi.org/10.1097/MD.0000000000017612
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