Document Detail


Case report: splenomegaly and splenic sequestration in an adult with sickle cell anemia.
MedLine Citation:
PMID:  8969620     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Splenomegaly in adult patients with homozygous sickle cell anemia (HbSS) is uncommon and splenic sequestration crises are rare. This paper describes a patient with HbSS who, at the age of 24, began to experience acute splenic sequestration crises. These episodes occurred with sufficient frequency and severity to warrant splenectomy. This case is presented to emphasize that, although rare, splenomegaly can persist in adults with homozygous HbSS and can be associated with severe and even life-threatening splenic sequestration. The incidence of splenomegaly in adults with HbSS and the factors linked to it will be discussed and the published reports of splenic sequestration crises in this patient population reviewed. It appears that high hemoglobin F (HbF) levels and alpha-thalassemia may be important etiologic factors in causing persistence of splenomegaly and predisposing patients to splenic sequestration crises.
Authors:
S Moll; E P Orringer
Publication Detail:
Type:  Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  The American journal of the medical sciences     Volume:  312     ISSN:  0002-9629     ISO Abbreviation:  Am. J. Med. Sci.     Publication Date:  1996 Dec 
Date Detail:
Created Date:  1997-01-15     Completed Date:  1997-01-15     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0370506     Medline TA:  Am J Med Sci     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  299-302     Citation Subset:  AIM; IM    
Affiliation:
Department of Medicine, Duke University Medical Center, Durham, North Carolina, USA.
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MeSH Terms
Descriptor/Qualifier:
Adult
Anemia, Sickle Cell / complications,  metabolism*
Fetal Hemoglobin / genetics,  metabolism
Hemoglobins / metabolism
Homozygote
Humans
Male
Radiography
Splenectomy
Splenomegaly / metabolism*
Technetium Tc 99m Sulfur Colloid / diagnostic use
Grant Support
ID/Acronym/Agency:
HL28391/HL/NHLBI NIH HHS; RR00046/RR/NCRR NIH HHS
Chemical
Reg. No./Substance:
0/Hemoglobins; 0/Technetium Tc 99m Sulfur Colloid; 9034-63-3/Fetal Hemoglobin

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


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