A patient with sickle cell hemoglobinopathy may suffer sudden death due to a number of factors. One reported cause is a sequestration crisis.
Hemoglobinopathies associated with organ sequestrations:
(1) homozygous sickle cell disease
(2) sickle cell-beta+ thalassemia
(3) hemoglobin SC
In the North America most cases have been seen in Afro-Americans.
Sequestration crises associated with sudden death:
(1) spleen (most common)
(2) bone marrow
(3) possibly other organs - lung and liver
Pathogenesis: obstruction of the venous outflow with acute congestion
Clinical features:
(1) sudden onset of sequestration
(2) shock
(3) hypoxemia
Complications:
(1) possibly sepsis
(2) intracranial hemorrhage
(3) multiorgan failure
(4) spenic rupture (in splenic sequestration)
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Specialty: Clinical Laboratory
