Document Detail


Functional asplenia in hemoglobin SC disease.
MedLine Citation:
PMID:  7718896     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The incidence of functional asplenia in sickle-hemoglobin C (SC) disease has not been defined, and the use of prophylactic penicillin to prevent life-threatening septicemia in this disorder is controversial. The percentage of red blood cells with pits (pit count) is a reliable assay of splenic function in other disorders but has not been validated in hemoglobin SC disease. To address these issues, we conducted a prospective, multicenter study of splenic function in persons with hemoglobin SC disease. Baseline clinical data were recorded, and red blood cell pit counts were performed on 201 subjects, aged 6 months to 90 years, with hemoglobin SC; 43 subjects underwent radionuclide liver-spleen scanning. Pit counts greater than 20% were associated with functional asplenia as assessed by liver-spleen scan, whereas pit counts less than 20% were found in subjects with preserved splenic function. Pit counts greater than 20% were present in 0 of 59 subjects (0%) less than 4 years of age, in 19 of 86 subjects (22%) 4 to 12 years of age, and in 25 of 56 subjects (45%) greater than 12 years of age. Other subjects with hemoglobin SC, who had previously undergone surgical splenectomy, had higher pit counts (59.7% +/- 9.5%) than splenectomized subjects without hemoglobinopathy (38.5% +/- 8.8%) or with sickle cell anemia (20.5% +/- 1.9%; P < .001). Two subjects with hemoglobin SC disease (not splenectomized), ages 14 and 15 years, with pit counts of 40.3% and 41.7% died from pneumococcal septicemia. These data indicate that functional asplenia occurs in many patients with hemoglobin SC disease, but its development is usually delayed until after 4 years of age. The pit count is a reliable measure of splenic function in hemoglobin SC disease, but values indicative of functional asplenia (> 20% in our laboratory) are higher than in other disorders. The routine administration of prophylactic penicillin to infants and young children with hemoglobin SC disease may not be necessary.
Authors:
P A Lane; J L O'Connell; J L Lear; Z R Rogers; G M Woods; K L Hassell; D L Wethers; D W Luckey; G R Buchanan
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Publication Detail:
Type:  Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Blood     Volume:  85     ISSN:  0006-4971     ISO Abbreviation:  Blood     Publication Date:  1995 Apr 
Date Detail:
Created Date:  1995-05-25     Completed Date:  1995-05-25     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  2238-44     Citation Subset:  AIM; IM    
Affiliation:
Colorado Sickle Cell Treatment and Research Center, University of Colorado School of Medicine, Denver, USA.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Aged, 80 and over
Altitude*
Anoxia / blood*,  complications
Child
Child, Preschool
Colorado
Disease Susceptibility
Erythrocyte Count*
Erythrocytes, Abnormal*
Hemoglobin SC Disease / complications,  physiopathology*
Humans
Infant
Middle Aged
Mononuclear Phagocyte System / physiopathology*
Prospective Studies
Risk
Sepsis / etiology*
Spleen / physiopathology*,  radionuclide imaging
Vacuoles / ultrastructure
Grant Support
ID/Acronym/Agency:
05357//PHS HHS; RR-00069/RR/NCRR NIH HHS

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


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