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Revue de Medecine Interne 2020-Aug

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

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C Marcombes
E Lafont
V Jullien
E Flamarion
J Dion
N Costedoat-Chalumeau
J Pouchot
J Arlet

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抽象

Introduction: Patients with sickle cell trait (SCT) are commonly considered as asymptomatic carriers. However, some clinical manifestations may occur.

Methods: Here we present a retrospective descriptive study about SCT subjects with at least one complication diagnosed in a sickle cell disease referral center, in Paris, between 2008 and 2019. We also performed a literature review on the complications of SCT subjects.

Results: Six patients (between 19 and 65 years old) were included. SCT was already known only for 4 of them at the time of the complication. Four patients presented with a splenic infarct after a stay in high altitude or a plane trip, one of them was associated with papillary necrosis; one patient had isolated papillary necrosis, and the last one had splenic sequestration. These complications happened for most of them after exposure to an unusual situation of hypoxia or deshydratation. Five out of 6 patients had a marked elevated C reactive protein.

Conclusion: SCT may cause acute ischemic complications in a context of prolonged hypoxia or dehydration. The most commonly reported are the splenic infarct and the renal papillary necrosis. A study of hemoglobin should be considered in these clinical situations in patients with compatible ethnic origin.

Keywords: Infarctus splénique; Nécrose papillaire rénale; Renal papillary necrosis; Sickle cell trait; Splenic infarction; Splenic sequestration; Séquestration splénique; Trait drépanocytaire.

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