Document Detail


Extramedullary hematopoiesis in hereditary spherocytosis deficient in ankyrin: a case report.
MedLine Citation:
PMID:  12215014     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Hereditary spherocytosis (HS) is a common inherited hemolytic anemia due to red cell membrane defects. Extramedullary hematopoiesis is a compensatory response to insufficient bone marrow blood cell production. The preferred sites of extramedullary hematopoietic involvement are the spleen, liver, and lymph nodes, but in HS the posterior paravertebral mediastinum is also commonly involved. A nonsplenectomized 74-year-old man with mild HS, with primary deficiency in ankyrin, was found by magnetic resonance imaging to have thoracic paravertebral hematopoietic masses. The patient showed high serum levels of erythropoietin, which may have played a role in the development of extramedullary hematopoietic masses through a continuous hematopoietic stimulus to erythroid cells in the propositus. The long-standing history of respiratory infections and of hypoxia in the propositus may have been an additional etiological factor.
Authors:
E Granjo; R Bauerle; R Sampaio; P Manata; N Torres; A Quintanilha
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  International journal of hematology     Volume:  76     ISSN:  0925-5710     ISO Abbreviation:  Int. J. Hematol.     Publication Date:  2002 Aug 
Date Detail:
Created Date:  2002-09-06     Completed Date:  2003-07-31     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9111627     Medline TA:  Int J Hematol     Country:  Ireland    
Other Details:
Languages:  eng     Pagination:  153-6     Citation Subset:  IM    
Affiliation:
Instituto de Ciências Biomédicas Abel Salazar, Porto, Portugal. elisagranjo@hotmail.com
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MeSH Terms
Descriptor/Qualifier:
Aged
Ankyrins* / deficiency
Erythropoietin / blood
Hematopoiesis, Extramedullary*
Humans
Magnetic Resonance Imaging
Male
Respiratory Tract Infections
Spherocytosis, Hereditary / blood,  complications*
Thorax
Chemical
Reg. No./Substance:
0/Ankyrins; 11096-26-7/Erythropoietin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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