[Sickle cell trait complications: A case series of 6 patients]

C Marcombes1, E Lafont2, V Jullien1

  • 1Département de médecine interne, Centre de référence des syndromes drépanocytaires majeurs de l'adulte, Hôpital européen Georges Pompidou (AP-HP), 20 rue Leblanc, 75015 Paris, France.

La Revue De Medecine Interne
|August 10, 2020
PubMed

Insights

Sickle cell trait (SCT) can cause serious ischemic complications like splenic infarct and papillary necrosis, especially during hypoxia or dehydration. Consider hemoglobin testing in at-risk individuals with compatible ethnic origins.

Area of Science:

  • Hematology
  • Internal Medicine
  • Genetics

Background:

  • Sickle cell trait (SCT) is often viewed as asymptomatic.
  • However, clinical manifestations can occur in individuals with SCT.

Observation:

  • A retrospective study identified six SCT patients (aged 19-65) experiencing complications.
  • Complications included splenic infarct (often after hypoxia/dehydration), papillary necrosis, and splenic sequestration.
  • Most patients had elevated C-reactive protein levels.

Findings:

  • SCT can lead to acute ischemic events, particularly splenic infarct and renal papillary necrosis.
  • These complications are frequently associated with hypoxic or dehydrating conditions.
  • Diagnosis of SCT was unknown in 2 out of 6 patients prior to their complication.

Implications:

  • SCT patients may experience severe ischemic complications under specific environmental stressors.
  • Hemoglobin evaluation is recommended for patients presenting with these complications and relevant ethnic backgrounds.
  • This highlights the importance of recognizing SCT as a potential risk factor for certain medical emergencies.
Abstract

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