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Simultaneous acute splenic sequestration and transient aplastic crisis in children with sickle cell disease

Amber Mayfield Yates1, Jane S Hankins, Nicole A Mortier

  • 1Department of Hematology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA. amber.yates@stjude.org

Pediatric Blood & Cancer
|May 7, 2009
PubMed

Insights

Acute splenic sequestration crisis (ASSC) and transient aplastic crisis (TAC) can occur together in children with sickle cell disease (SCD). This concurrent presentation leads to severe illness, requiring close monitoring for complications.

Area of Science:

  • Hematology
  • Pediatrics
  • Infectious Diseases

Background:

  • Acute splenic sequestration crisis (ASSC) is a common pediatric sickle cell disease (SCD) emergency.
  • Transient aplastic crisis (TAC), often caused by parvovirus B19, is another SCD complication.
  • ASSC typically involves splenomegaly and anemia, while TAC presents with anemia and reticulocytopenia.

Observation:

  • Five older children with SCD (HbSC and HbSS genotypes) experienced concurrent ASSC and TAC.
  • These patients had a severe clinical course, indicating a compounded disease impact.
  • The concurrent crises occurred during acute parvovirus B19 infection.

Findings:

  • Older children with SCD can develop ASSC alongside TAC during parvovirus B19 infection.
  • This dual complication presents a severe clinical picture in pediatric SCD patients.
  • The presence of splenomegaly in the context of TAC warrants further investigation for ASSC.

Implications:

  • Clinicians should consider concurrent ASSC and TAC in older SCD patients with parvovirus B19.
  • Close monitoring for splenomegaly and multi-system dysfunction is crucial in these cases.
  • Early recognition and management can mitigate the severe consequences of combined SCD crises.

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