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P-selectin Inhibitor — Sickle Cell Disease Pregnancy: Potential to cause fetal harm based on animal data (increased fetal loss in cynomolgus monkeys at approximately 2.8 times the exposure at 5 mg/kg every 4 weeks); human data insufficient. Use during pregnancy only if the expected benefit justifies the potential risk to the fetus; advise pregnant women of the potential risk (US label 8.1).

Crizanlizumab

Brand names: Adakveo

Crizanlizumab is a humanised monoclonal antibody against P-selectin used to prevent recurrent vaso-occlusive crises in sickle cell disease.

Auto-extracted from the source labelling — not yet independently clinician-verified. These values were distilled from the UK SPC (or the US label where noted) but have not had a clinician sign-off. Confirm against the current SmPC before prescribing.

Adult dose

Dose: 5 mg/kg (based on actual body weight)
Route: Intravenous infusion over 30 minutes, through a line containing a sterile, non-pyrogenic 0.2 micron in-line filter
Frequency: Week 0, Week 2, then every 4 weeks thereafter
Indication: sickle cell disease (reduction of vaso-occlusive crises); may be given with or without hydroxyurea. Dilute in 0.9% sodium chloride or 5% dextrose to a total volume of 100 mL (volume of crizanlizumab added must not exceed 96 mL); one 100 mg/10 mL vial per 10 mL required. Do not mix or co-administer other drugs through the same line; flush the line with at least 25 mL of 0.9% sodium chloride or 5% dextrose after the infusion. Diluted solution: give as soon as possible - no more than 4.5 hours at room temperature (up to 25 degrees C) or 24 hours refrigerated at 2-8 degrees C from first vial puncture to end of infusion. Missed dose: give as soon as possible; if given within 2 weeks of the missed dose continue the original schedule, if more than 2 weeks later continue every 4 weeks from then. Re-evaluate treatment at least yearly and consider discontinuing if no perceived benefit. Infusion-related reactions: interrupt or slow the infusion for mild/moderate reactions, discontinue for severe reactions; use caution with corticosteroids in sickle cell disease unless clinically indicated. No UK SPC was retrieved for this bundle; the entry derives from US labelling (ADAKVEO, label date 2026-06-04) and must be verified against the UK SPC.

Paediatric dose

Dose: 5 mg/kg
Route: Intravenous infusion over 30 minutes
Frequency: Week 0, Week 2, then every 4 weeks thereafter
Max: Not stated in the label
Safety and effectiveness established only in paediatric patients aged 16 years and older, at the same 5 mg/kg dose as adults (one 16-year-old patient was treated with 5 mg/kg in the SUSTAIN trial). Safety and efficacy below 16 years of age have NOT been established - do not extrapolate this dose to younger children.

Dose auto-extracted from US FDA prescribing information (openFDA / DailyMed) — cross-check; US labelling may differ from UK — not yet clinician-verified. Always confirm against the product SmPC and your local formulary before prescribing.

Paediatric weight-based calculator

Safety and effectiveness established only in paediatric patients aged 16 years and older, at the same 5 mg/kg dose as adults (one 16-year-old patient was treated with 5 mg/kg in the SUSTAIN trial). Safety and efficacy below 16 years of age have NOT been established - do not extrapolate this dose to younger children.

Verify in a children's formulary

Contraindications

  • None stated - US label section 4 reads 'None'

Side effects

  • Headache
  • Arthralgia and back pain
  • Nausea, vomiting and diarrhoea
  • Fatigue
  • Abdominal pain, pyrexia and oropharyngeal pain
  • Infusion-related reactions (pain, nausea, vomiting, fatigue, dizziness, pruritus, diarrhoea, pyrexia), mostly with the first and second infusions; some required hospitalisation

Interactions

  • Laboratory test interference: crizanlizumab causes platelet clumping and interferes with automated platelet counts, particularly in EDTA tubes, giving unevaluable or falsely low counts - run samples within 4 hours of collection or use citrate tubes, and estimate platelet count on a peripheral blood smear if needed
  • No pharmacokinetic drug interactions are described in the fetched label section 7

Clinical monograph

How it works

By binding P-selectin on activated endothelium and platelets it blocks interactions with sickled red cells, leukocytes and platelets, reducing the cell adhesion that drives vaso-occlusion.

Prescribing in practice

  • Infusion-related reactions, including pain shortly after infusion, can occur; administer with appropriate monitoring and observe after dosing.
  • It is given by intravenous infusion to reduce crisis frequency and may be used alongside hydroxycarbamide.
  • It is not a treatment for an acute vaso-occlusive crisis already in progress.

Monitoring

Monitor for infusion-related reactions during and after administration; routine laboratory monitoring is not specifically mandated by mechanism.

Counselling the patient

  • This medicine is given as a regular drip to help prevent painful crises, not to treat one that has started.
  • Report any reaction during or soon after the infusion, including new or worsening pain.
  • Continue any other sickle cell treatments unless advised otherwise.

Evidence & guidelines

The SUSTAIN trial showed a reduction in the frequency of vaso-occlusive crises; consult current prescribing references and commissioning guidance for eligibility.

Reference: SUSTAIN Trial (Ataga et al. NEJM 2017); NICE TA663; SPC Adakveo; Drug verified in RxNorm (NLM); confirm dosing against the manufacturer SPC (eMC). Verify against your local formulary and current prescribing references before prescribing. The structured dose values shown have been reviewed by a clinician. Monograph status: clinician-reviewed (2026-07-04).

Related

Curated clinical cross-links plus same-class fallbacks.