Mild splenic sequestration crises in sickle-hemoglobin C disease.

Abstract:

:Acute splenic sequestration crisis ( ASSC ), a common complication in homozygous sickle cell anemia, has been described infrequently in sickle-hemoglobin C (SC) disease in the absence of high altitude exposure. In this report, we describe three children with hemoglobin SC disease who developed episodes of ASSC that were milder than those generally described in sickle cell anemia. In one patient, an antecedent triggering event was not recognized. The other two children may have had a predisposing cause for massive intrasplenic sickling in that one had an associated mononucleosis syndrome and the other an episode of hypovolemic shock following severe epistaxis. ASSC may occur in patients with SC disease and does not necessarily require a hypoxic trigger associated with high altitudes.

journal_name

Clin Pediatr (Phila)

journal_title

Clinical pediatrics

authors

Andrews J,Buchanan GR

doi

10.1177/000992288402300612

subject

Has Abstract

pub_date

1984-06-01 00:00:00

pages

354-5

issue

6

eissn

0009-9228

issn

1938-2707

journal_volume

23

pub_type

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