Hematology and Oncology Book


Sickle Cell Anemia with Splenic Sequestration

Aka: Sickle Cell Anemia with Splenic Sequestration, Splenic Sequestration in Sickle Cell Anemia, Splenic Sequestration
  1. See Also
    1. Sickle Cell Anemia
    2. Asplenic
    3. Transient Red Cell Aplasia
    4. Acute Chest Syndrome
    5. Acute Vaso-Occlusive Episode in Sickle Cell Anemia
    6. Aplastic Crisis in Sickle Cell Anemia
    7. Cerebrovascular Accident in Sickle Cell Anemia
    8. Dactylitis in Sickle Cell Anemia (Hand Foot Syndrome in Sickle Cell Anemia)
    9. Hematuria in Sickle Cell Anemia
    10. Osteomyelitis in Sickle Cell Anemia
    11. Priapism in Sickle Cell Anemia
    12. Pulmonary Hypertension in Sickle Cell Anemia
    13. Septic Arthritis in Sickle Cell Anemia
    14. Sickle Cell Anemia Related Pulmonary Hypertension
  2. Epidemiology
    1. Most common in ages 1 to 4 years old (decreased Incidence after age 5 years old)
    2. Lifetime Prevalence: 30%
  3. 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
  4. Precautions
    1. Rapidly progressive course with significant risk of decompensation secondary to acute Anemia (life threatening)
  5. Symptoms
    1. Decreased activity
  6. 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
  7. 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
  8. Imaging: Bedside Ultrasound
    1. Spleen will apear very large and round
      1. Normal Spleens will appear Kidney-bean shaped
  9. Differential Diagnosis
    1. Sickle Cell Hemolytic Crisis
      1. Hemolysis associated with an increased AST, LDH and Indirect Bilirubin over baseline
  10. 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
  11. References
    1. Glassberg and Weingart in Majoewsky (2012) EM: Rap 12(8): 5-6

Hb-SS disease with splenic sequestration (C2873762)

Concepts Disease or Syndrome (T047)
ICD10 D57.02
English Hb-SS disease with splenic sequestration
Derived from the NIH UMLS (Unified Medical Language System)

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