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Updated: Aug 12, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy

[Splenic dysfunction in sickle cell disease: An update].

J Tennenbaum¹, G Volle², P Buffet³

¹Service de médecine interne, Centre de référence national de la drépanocytose de l'adulte, Hôpital européen Georges-Pompidou, Assistance publique-Hôpitaux de Paris, Paris, France.

La Revue De Medecine Interne

|January 29, 2023

Summary

This summary is machine-generated.

The spleen filters blood and supports immunity. In sickle cell disease, spleen dysfunction increases infection risk and can cause life-threatening complications like sequestration.

Keywords:

Drépanocytose Fonction splénique Hyposplenism Hyposplénisme Sickle cell disease Splenectomy Splenic function Splénectomie

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Published on: June 11, 2019

Area of Science:

Immunology
Hematology
Physiology

Context:

The spleen is vital for blood filtration and immune defense, particularly against encapsulated bacteria.
Impaired splenic function (hyposplenism) or splenectomy increases infection susceptibility and thrombosis risk.
Sickle cell disease frequently causes functional asplenia early in life due to red blood cell damage.

Purpose:

To provide an updated overview of spleen physiology and function.
To elucidate the pathophysiology of spleen damage in sickle cell disease.
To enhance understanding of spleen-related complications in sickle cell disease.

Summary:

The spleen's red pulp clears old red blood cells, while the white pulp defends against encapsulated bacteria.
Hyposplenism and splenectomy lead to increased risks of infections, thrombosis, and pulmonary hypertension.
Sickle cell disease causes early functional asplenia, with risks of hypersplenism and acute splenic sequestration.

Impact:

Improved understanding of spleen's role in immunity and blood homeostasis.
Better comprehension of sickle cell disease pathophysiology and its splenic complications.
Potential for improved management strategies for patients with sickle cell disease and splenic dysfunction.