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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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Acute Splenic Sequestration Crisis in Hemoglobin SC Disease: Efficiency of Red Cell Exchange

Anjanaa Vijayanarayanan1, Ayodeji J Omosule2, Hannan Saad3

  • 1Pathology, Henry Ford Hospital, Detroit, USA.

Cureus
|January 27, 2021
PubMed

Insights

Acute splenic sequestration crisis (ASSC) in adults with sickle cell disease (SCD) is rare. Automated red blood cell exchange can effectively manage ASSC in adults, avoiding splenectomy.

Area of Science:

  • Hematology
  • Sickle Cell Disease Management

Background:

  • Acute splenic sequestration crisis (ASSC) is a known complication of sickle cell disease (SCD) primarily in children.
  • ASSC in adults is uncommon, leading to poorly defined management strategies and outcomes.
  • Hemoglobin (Hb) SC disease is a specific type of SCD.

Observation:

  • This article details the management of ASSC in an adult female patient with Hb SC disease.
  • The patient presented with symptoms indicative of ASSC.

Findings:

  • Automated red blood cell (RBC) exchange was successfully employed to manage the ASSC.
  • This intervention successfully avoided the need for a planned splenectomy.
  • This represents the third reported case of RBC exchange use for ASSC in adults with Hb SC disease.

Implications:

  • RBC exchange should be considered for adult patients with Hb SC disease experiencing ASSC unresponsive to simple transfusions.
  • This treatment can alleviate patient symptoms and prevent complications associated with splenectomy, particularly in younger adults.
  • Further research into adult ASSC management in SCD is warranted.

Keywords:
acute splenic sequestrationadultcasehemoglobin sc diseasered cell exchange

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