Sickle Cell Anemia with Splenic Sequestration


Sickle Cell Anemia with Splenic Sequestration, Splenic Sequestration in Sickle Cell Anemia, Splenic Sequestration

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