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Intracranial extramedullary hematopoiesis in patients with thalassemia: a case report and review of the literature.
MedLine Citation:
PMID:  22220514     Owner:  NLM     Status:  Publisher    
BACKGROUND: Extramedullary hematopoiesis (EH) is a compensatory phenomenon that results in the production of blood cell precursors outside the marrow in patients with chronic hemolytic anemia and ineffective erythropoiesis. EH usually involves the liver, spleen, and lymph nodes. It can also be found at paravertebral, intrathoracic, or pelvic locations. Intracranial EH is a rare entity and often asymptomatic but can sometimes lead to symptomatic tumor-like masses. Treatment options are controversial and include hypertransfusion, surgical excision, radiotherapy, and hydroxyurea (HU). STUDY DESIGN AND METHODS: Successful treatment of an intracranial EH mass with HU and blood transfusions in a beta-thalassemia major patient was discussed along with a review of the published literature on intracranial EH in thalassemia. RESULTS: In our patient, the extramedullary hematopoietic mass in the interhemispheric fissure showed a marked improvement after 6 months of HU and hypertransfusion therapy. In the English literature, there are a few cases with intracranial EH and thalassemia, which were treated with different treatment modalities, with different outcomes. CONCLUSION: There is no standard treatment approach in patients with symptomatic EH. HU with hypertransfusion regimen is a reasonable first-choice modality in treating intracranial EH masses.
Ahmet Emre Eskazan; Muhlis Cem Ar; Zafer Baslar
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-1-4
Journal Detail:
Title:  Transfusion     Volume:  -     ISSN:  1537-2995     ISO Abbreviation:  -     Publication Date:  2012 Jan 
Date Detail:
Created Date:  2012-1-6     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0417360     Medline TA:  Transfusion     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2012 American Association of Blood Banks.
From Department of Hematology, Diyarbakir Training and Research Hospital, Diyarbakir; Department of Hematology, Istanbul Training and Research Hospital; and Division of Hematology, Department of Internal Medicine, Istanbul University Cerrahpasa Faculty of Medicine, Istanbul, Turkey.
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