Related Experiment Video

Updated: Apr 16, 2026

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
07:24

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

2.0K

Immune parameter analysis of children with sickle cell disease on hydroxycarbamide or chronic transfusion therapy

Robert S Nickel1, Ifeyinwa Osunkwo, Aneesah Garrett

  • 1Aflac Cancer and Blood Disorders Centre, Children's Healthcare of Atlanta, Emory University, Atlanta, GA, USA; Centre for Transfusion and Cellular Therapies, Department of Pathology and Laboratory Medicine, Emory University, Atlanta, GA, USA.

British Journal of Haematology
|March 11, 2015
PubMed

Insights

Sickle cell disease (SCD) involves immune system changes. Hydroxycrabamide (HC) treatment normalized most immune cell counts in pediatric SCD patients, unlike other therapies.

Area of Science:

  • Immunology
  • Hematology
  • Pediatrics

Background:

  • Sickle cell disease (SCD) is recognized as an inflammatory condition impacting immune cell function.
  • Altered immune phenotypes are characteristic of SCD, affecting disease progression and complications.

Purpose of the Study:

  • To investigate the effects of different disease-modifying therapies on immune cell populations in pediatric SCD patients.
  • To compare immune marker profiles in SCD patients receiving hydroxycrabamide (HC), chronic transfusion (CT), or no therapy against healthy controls.

Main Methods:

  • A cross-sectional study analyzing 18 immune markers in 114 pediatric SCD patients and 29 healthy controls.
  • Patients were stratified into treatment groups: HC, CT, or no disease-modifying therapy.
  • Multiparameter analysis was used to assess immune cell counts and phenotypes.

Main Results:

  • SCD patients not receiving disease-modifying therapy showed globally elevated immune cell counts.
  • Abnormal immune cell phenotypes persisted in patients on chronic transfusion (CT) despite reduced sickle haemoglobin.
  • Hydroxycrabamide (HC) treatment was associated with the normalization of most leukocyte populations in univariate and multivariate analyses.

Conclusions:

  • Hydroxycrabamide (HC) treatment demonstrates a significant role in normalizing immune cell counts in pediatric SCD patients.
  • HC therapy appears to mitigate the immune dysregulation associated with sickle cell disease.
  • These findings support the use of HC as a beneficial treatment for managing immune alterations in SCD.

Keywords:
chronic transfusionhydroxycarbamideimmune phenotypeimmunophenotypesickle cell disease

More Related Videos

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload05:23

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload

Published on: March 14, 2017

20.7K
Endothelialized Microfluidics for Studying Microvascular Interactions in Hematologic Diseases11:08

Endothelialized Microfluidics for Studying Microvascular Interactions in Hematologic Diseases

Published on: June 22, 2012

16.8K

Related Concept Videos

Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test01:22

Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test

In clinical practice, the direct measurement of hepatic blood flow to evaluate liver function presents significant challenges due to the intricate and specialized nature of the necessary techniques. Consequently, healthcare professionals often rely on empirical estimates derived from thorough patient examinations and liver function tests to gauge liver health. Among the tools at their disposal, the Child–Pugh and MELD scoring systems stand out for their ability to categorize and assess...
263
Multiple Allele Traits01:49

Multiple Allele Traits

The Concept of Multiple Allelism
39.2K
Pharmacokinetics in Pediatric Patients: Drug Metabolism01:24

Pharmacokinetics in Pediatric Patients: Drug Metabolism

In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses...
362
Regulation of Hematopoietic Stem Cells01:01

Regulation of Hematopoietic Stem Cells

All blood and immune cells are produced from the multipotent hematopoietic stem cells (HSCs) by the process of hematopoiesis. However, they all have a limited life span. In addition, many are depleted in immune surveillance or combatting an injury or infection. This makes blood one of the most regenerative tissues. Hematopoiesis helps replenish these blood and immune cells, restoring the body's normal functioning. However, overproduction of blood and immune cells can make them cancerous or...
4.5K
Hemodialysis II: Procedure and Complications01:24

Hemodialysis II: Procedure and Complications

DialyzersA hemodialysis (HD) dialyzer is a plastic cartridge containing thousands of parallel hollow fibers, which serve as semipermeable membranes. These fibers are typically made from cellulose-based or other synthetic materials. During HD, blood is pumped into the top of the cartridge and distributed among these fibers. Simultaneously, dialysis fluid, known as dialysate, is introduced into the bottom of the cartridge, bathing the outside of the fibers. Across the semipermeable membrane,...
2.0K