II. Epidemiology
- Most common in ages 1 to 4 years old (decreased Incidence after age 5 years old)
- Lifetime Prevalence: 30%
III. Pathophysiology
- Vaso-Occlusion within the Spleen prevents normal Red Blood Cell release
- Splenic infarction typically occurs by Puberty in patients with Hemoglobin SS
- However, less severe forms of Sickle Cell Disease (e.g. HgbSC, HgbB+thal) can occur in adulthood
IV. Precautions
- Rapidly progressive course with significant risk of decompensation secondary to acute Anemia (life threatening)
V. Symptoms
- Decreased activity
VI. Signs
- Pallor
-
Anemia
- Younger children may present in Hypovolemic Shock
- Abdominal Pain
-
Splenomegaly
- Acute splenic enlargement (especially in under age 5 years, before auto-infarction)
- Contrast with Sickle Cell Hemolytic Crisis in which the Spleen will be small or non-palpable
VII. Labs
-
Hemoglobin
- Severe Anemia (below baseline)
-
Reticulocyte Count increased
- Similar increase as with Sickle Cell Hemolytic Crisis
- Contrast with Transient Red Cell Aplasia in which the Reticulocyte Count is very low
VIII. Imaging: Bedside Ultrasound
IX. Differential Diagnosis
- Sickle Cell Hemolytic Crisis
- Hemolysis associated with an increased AST, LDH and Indirect Bilirubin over baseline
X. Management
- Admission to Intensive Care unit
- Hematology Consultation
- Intravenous crystalloid (LR or NS) 10 cc/kg
- Often given while awaiting RB transfusion
- Avoid Fluid Overload
-
Blood Transfusion (pRBC)
- Start with 10 cc/kg blood in children up to 2 units in adults immediately
- Blood should typically be cross-matched, Leukocyte depleted, and irradiated but do not delay transfusion
- Do not wait for Hemoglobin to drop on serial labs
- Post-acute stabilization care (following Blood Transfusions)
- Observe for occlusive complications from transfusion
- Hyperviscosity results in increased Cerebrovascular Accident risk
XI. References
- Glassberg and Weingart in Majoewsky (2012) EM: Rap 12(8): 5-6