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# Sickle cell disease agents

Newer drugs that reduce pain crises in sickle cell disease by protecting red cells or stopping them sticking to vessel walls.

Updated Nov 1, 2026

## What they are

In sickle cell disease, haemoglobin S polymerises when it gives up oxygen, so red cells become rigid and stick to the endothelium, blocking small vessels (vaso-occlusive crises). Hydroxycarbamide remains the base treatment (own page). L-glutamine (oral) and crizanlizumab (an antibody against P-selectin) are added to reduce crises. Gene therapies are now authorised in some countries.

## Mechanism of action

L-glutamine is thought to improve the redox balance (NAD) of sickle red cells and raise their reduced glutathione, protecting them from oxidative damage. Crizanlizumab binds P-selectin on activated endothelium and platelets and blocks the contacts between vessel wall, platelets, red cells and leucocytes that start a vaso-occlusive crisis.

Target: Red cell oxidative stress and adhesion to the endothelium (P-selectin)

## Key points

- Hydroxycarbamide raises fetal haemoglobin and is still the first drug
- L-glutamine: oral amino-acid powder, thought to reduce oxidative stress in red cells
- Crizanlizumab: anti–P-selectin antibody against vaso-occlusion
- Filgrastim can trigger severe crises in sickle cell disease

## Drugs in this class

- [**L-glutamine** An amino acid given as an oral powder to reduce pain crises and other acute complications of sickle cell disease from… Redox balance of sickle red cells (NAD and reduced glutathione)](https://durgical.com/en/pharmacology/l-glutamine/)
- [**Crizanlizumab** A humanised antibody against P-selectin, given by infusion to reduce pain crises in sickle cell disease from age 16. P-selectin on activated endothelium and platelets](https://durgical.com/en/pharmacology/crizanlizumab/)

## Others in the class

- hydroxycarbamide (hydroxyurea), on its own page
- exagamglogene autotemcel (gene therapy)
- lovotibeglogene autotemcel (gene therapy)

## Test yourself

Think of the answer, then tap the question to see it.

## Which adhesion molecule does crizanlizumab block?

P-selectin.

## What is the usual base drug for sickle cell disease?

Hydroxycarbamide (hydroxyurea).

## How is L-glutamine given?

As an oral powder mixed with a drink or soft food.

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Educational content on how drugs work; not a prescribing guide and no substitute for a health professional's advice. No doses are given.

Sources: [ATCDDD - ATC/DDD Index](https://atcddd.fhi.no/atc_ddd_index/?code=B06AX&showdescription=no) (WHO Collaborating Centre for Drug Statistics Methodology, 2026); [DailyMed - L-GLUTAMINE powder, for solution](https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=bb4ec712-14b7-47e3-8cb9-c71922712e55) (DailyMed (U.S. National Library of Medicine), 2026).

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Representative model, for educational purposes only.

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Source: https://durgical.com/en/pharmacology/classes/sickle-cell-disease-agents/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/pharmacology/classes/sickle-cell-disease-agents/