II. Epidemiology

  1. Most common in ages 1 to 4 years old

III. Pathophysiology

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

IV. Precautions

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

V. Signs

  1. Anemia
  2. Abdominal Pain
  3. 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

VI. Labs

  1. 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

VII. Imaging: Bedside Ultrasound

  1. Spleen will apear very large and round
    1. Normal Spleens will appear Kidney-bean shaped

VIII. Differential Diagnosis

  1. Sickle Cell Hemolytic Crisis
    1. Hemolysis associated with an increased AST, LDH and Indirect Bilirubin over baseline

IX. Management

  1. Admission to intensive care unit
  2. Blood Transfusion (pRBC)
    1. Start with 2 units 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
  3. Post-acute stabilization care (following Blood Transfusions)
    1. Observe for occlusive complications from transfusion (hyperviscosity results in increased Cerebrovascular Accident risk)

X. References

  1. Glassberg and Weingart in Majoewsky (2012) EM: Rap 12(8): 5-6

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Ontology: Hb-SS disease with splenic sequestration (C2873762)

Concepts Disease or Syndrome (T047)
ICD10 D57.02
English Hb-SS disease with splenic sequestration