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Titre du document / Document title

Splenomegaly and splenic sequestration in an adult with sickle cell anemia

Auteur(s) / Author(s)

MOLL S. (1) ; ORRINGER E. P. (2) ;

Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)

(1) Division of Hematology/Oncology, Department of Medicine, Duke University Medical Center, Durham, North Carolina, ETATS-UNIS
(2) Division of Hematology/Oncology, Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, ETATS-UNIS

Résumé / Abstract

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 α-thalassemia may be important etiologic factors in causing persistence of splenomegaly and predisposing patients to splenic sequestration crises.

Revue / Journal Title

The American journal of the medical sciences    ISSN  0002-9629   CODEN AJMSA9 

Source / Source

1996, vol. 312, no6, pp. 299-302 (19 ref.)

Langue / Language

Anglais

Editeur / Publisher

Lippincott Williams & Wilkins, Hagerstown, MD, ETATS-UNIS  (1827) (Revue)

Mots-clés anglais / English Keywords

Splenomegaly

;

Sequestration in spleen

;

Sickle cell anemia

;

Homozygozity

;

Case study

;

Complication

;

Adult

;

Human

;

Hemopathy

;

Hemolytic anemia

;

Hemoglobinopathy

;

Genetic disease

;

Mots-clés français / French Keywords

Splénomégalie

;

Séquestration splénique

;

Anémie hématie falciforme

;

Homozygotie

;

Etude cas

;

Complication

;

Adulte

;

Homme

;

Hémopathie

;

Anémie hémolytique

;

Hémoglobinopathie

;

Maladie héréditaire

;

Mots-clés espagnols / Spanish Keywords

Esplenomegalia

;

Secuestro esplénico

;

Anemia glóbulo falciforme

;

Homocigosis

;

Estudio caso

;

Complicación

;

Adulto

;

Hombre

;

Hemopatía

;

Anemia hemolítica

;

Hemoglobinopatía

;

Enfermedad hereditaria

;

Localisation / Location

INIST-CNRS, Cote INIST : 2054, 35400006123989.0080

Nº notice refdoc (ud4) : 2520538



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