Document Detail


Extra-medullary hematopoiesis causing bilateral optic atrophy in beta thalassemia/Hb E disease.
MedLine Citation:
PMID:  17487139     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: To report a rare presentation of bilateral optic nerve compression from extra-medullary hematopoiesis in beta thalassemia/Hb E disease. DESIGN: Interventional case report. CLINICAL PRESENTATION: A 13-year-old Thai girl was reported with slowly progressive bilateral visual loss due to optic neuropathy as a result of compression from extra-medullary hematopoiesis. Computed tomography of orbit and brain revealed a homogeneous enhancing midline soft tissue mass originating from the ethmoid and sphenoid sinuses along with extensive involvement of the skull. INTERVENTION: Blood transfusion once every month and low-dose radiotherapy to sphenoid and ethmoid bones with some visual improvement. CONCLUSION: Extra-medullary hematopoiesis in beta thalassemia can involve paranasal sinuses and cause compressive neuropathy. In patients with beta thalassemia that required multiple blood transfusions with progressive visual loss, ectopic marrow compression of optic nerve should be ruled out.
Authors:
Nimitr Ittipunkul; Tada Martin; Rutchada Siriwanasan; Kanokporn Olanratanachai; Jack Rootman
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of the Medical Association of Thailand = Chotmaihet thangphaet     Volume:  90     ISSN:  0125-2208     ISO Abbreviation:  J Med Assoc Thai     Publication Date:  2007 Apr 
Date Detail:
Created Date:  2007-05-09     Completed Date:  2007-11-13     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7507216     Medline TA:  J Med Assoc Thai     Country:  Thailand    
Other Details:
Languages:  eng     Pagination:  809-12     Citation Subset:  IM    
Affiliation:
Department of Ophthalmology, Faculty of Medicine, Chiang Mai University, Thailand.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Female
Hematopoiesis, Extramedullary*
Humans
Optic Nerve / physiopathology*,  radiography
Tomography, X-Ray Computed
beta-Thalassemia / complications*

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