Splenic sequestration

Background

  • Leading cause of pediatric mortality related to sickle cell disease[1]
  • More common in pediatric patients as many adults with SCD have autoinfarcted their spleen
  • Earliest life threatening complication of SCD with median age 1-43
  • Red blood cells sickle, aggregate, and occlude splenic vasculature[2][3]
  • Sequestering leads to splenomegaly
    • Defined as splenomegaly and a 2g/dL drop in hemoglobin
      • Some resources differentiate between major and minor episodes depending on the hgb drop
  • Lifelong prevalence 7-30%3
  • Often precipitated by underlying infection (pneumonia, gastroenteritis)

Clinical Features

Complications

Differential Diagnosis

Sickle cell crisis


Evaluation

  • Labs
    • CBC
    • BMP
    • LDH
    • Reticulocyte count
    • LFTs
    • Blood cultures
  • Imaging not necessary to make diagnosis

Management

  • Pain control
  • IV fluid resuscitation and blood transfusion[4]
  • Treat underlying cause if found - infection common
  • Possible splenectomy
    • Recurrence rate is 50-75%1,2,3[5]
  • Hematology consultation

Disposition

  • Admission

See Also

Sickle cell crisis

External Links

References

  1. Brousse V et al. Acute splenic sequestration crisis in sickle cell disease: cohort study of 190 pediatric patients. British Journal of Haematology. 2012;156:643-648.
  2. Brousse V et al. The spleen and sickle cell disease: the sick(led) spleen. British Journal of Haematology. 2014;166:165-176.
  3. Lovett PB et al. Sickle Cell Disease in the Emergency Department. Emerg Med Clin N Am. 2014;32(3):629-647.
  4. Abboud MR. Standard management of sickle cell disease complications. Hematology/Oncology and Stem Cell Therapy. 2020;3(1):85-90.
  5. Kane I, Nagalli S. Splenic Sequestration Crisis. [Updated 2020 Jul 6]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK553164/