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Sickle Cell Anemia with Splenic Sequestration

key information

source: Family Practice Notebook

year: 2018

summary/abstract:

Sickle Cell Anemia with Splenic Sequestration:
II. Epidemiology
Most common in ages 1 to 4 years old

III. Pathophysiology
A. Splenic infarction typically occurs by Puberty in patients with Hemoglobin SS
B. However, less severe forms of Sickle Cell Disease can occur in adulthood

IV. Precautions
Rapidly progressive course with significant risk of decompensation secondary to acute Anemia

V. Signs
A. Anemia
B. Abdominal Pain
C. Splenomegaly
1. Acute splenic enlargement (especially in under age 5 years, before auto-infarction)
2. Contrast with Sickle Cell Hemolytic Crisis in which the Spleen will be small or non-palpable

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