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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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Massive Splenic Infarction in a Child With Sickle Cell Disease on Chronic Transfusion Therapy

Maria A Pereda1, Jaya Isaac2, Yaoping Zhang1

  • 1Departments of Pediatrics.

Journal of Pediatric Hematology/Oncology
|December 1, 2018
PubMed

Insights

Massive splenic infarction (MSI) is a rare complication in sickle cell disease. Elective splenectomy may be considered for patients with hypersplenism, despite associated risks.

Area of Science:

  • Hematology
  • Pediatric Medicine
  • Vascular Complications

Background:

  • Sickle cell disease (SCD) typically leads to splenic atrophy in early childhood.
  • Massive splenic infarction (MSI) is an uncommon complication in SCD patients.
  • Chronic transfusion therapy can paradoxically lead to hypersplenism in SCD.

Observation:

  • A 12-year-old boy with homozygous sickle cell anemia (Hb SS) developed MSI.
  • The patient's MSI occurred secondary to hypersplenism induced by chronic transfusion therapy.
  • This case highlights a rare presentation of splenic complications in SCD.

Findings:

  • MSI occurred in a pediatric patient with Hb SS despite expected splenic atrophy.
  • Hypersplenism, a consequence of chronic transfusion, was identified as the likely cause.
  • The case underscores the complex splenic dynamics in SCD management.

Implications:

  • Consider MSI in SCD patients presenting with abdominal pain, especially those on chronic transfusions.
  • Elective splenectomy might be a viable option for managing hypersplenism and preventing MSI.
  • Careful risk-benefit analysis is crucial when considering splenectomy due to potential complications.

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