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Anti-vWF Nanobody — Acquired TTP Pregnancy: §8.1 Pregnancy Risk Summary, verbatim: 'There are no available data on CABLIVI use in pregnant women to inform a drug-associated risk of major birth defects and miscarriage. However, there are potential risks of hemorrhage in the mother and fetus associated with use of CABLIVI. In animal reproduction studies, there was no evidence of adverse developmental outcomes with intramuscular administration of caplacizumab-yhdp during organogenesis in guinea pigs at exposures approximately 30 times the AUC in humans at the recommended subcutaneous injection dose of 11 mg.' Clinical considerations: 'CABLIVI may increase the risk of bleeding in the fetus and neonate. Monitor neonates for bleeding'; 'All patients receiving CABLIVI, including pregnant women, are at risk for bleeding. Pregnant women receiving CABLIVI should be carefully monitored for evidence of excessive bleeding.' The section is truncated at the source-fetch limit; no lactation statement was retrieved.

Caplacizumab

Brand names: Cablivi

Caplacizumab is an anti-von Willebrand factor nanobody used, alongside plasma exchange and immunosuppression, for acquired (immune) thrombotic thrombocytopenic purpura (aTTP).

Adult dose

Dose: 11 mg per dose. First day of treatment: 11 mg intravenous bolus at least 15 minutes before plasma exchange, then 11 mg subcutaneously after completion of plasma exchange on day 1
Route: Intravenous bolus for the first dose only, given by a healthcare provider; all subsequent doses subcutaneously into the abdomen — §2.3: 'Avoid injections around the navel. Do not administer consecutive injections in the same abdominal quadrant.'
Frequency: Once daily — 11 mg subcutaneously once daily following plasma exchange on each day of daily plasma exchange, then 11 mg subcutaneously once daily continuing for 30 days after the last daily plasma exchange
SCOPE: acquired thrombotic thrombocytopenic purpura (aTTP), in combination with plasma exchange and immunosuppressive therapy — this page's primary indication, and the only indication dosed in the fetched label. VERBATIM §2.1: 'CABLIVI should be administered upon initiation of plasma exchange therapy... First day of treatment: 11 mg bolus intravenous injection at least 15 minutes prior to plasma exchange followed by an 11 mg subcutaneous injection after completion of plasma exchange on day 1. Subsequent days of treatment during daily plasma exchange: 11 mg subcutaneous injection once daily following plasma exchange. Treatment after plasma exchange period: 11 mg subcutaneous injection once daily continuing for 30 days following the last daily plasma exchange. If after initial treatment course, sign(s) of persistent underlying disease such as suppressed ADAMTS13 activity levels remain present, treatment may be extended for a maximum of 28 days. Discontinue CABLIVI if the patient experiences more than 2 recurrences of aTTP, while on CABLIVI. Avoid concomitant use of antiplatelet agents or anticoagulants.' (The '28 days' figure is a maximum treatment EXTENSION, not a dose ceiling, so it is not placed in maxDose; no maximum dose is stated anywhere in the fetched label.) MISSED DOSE (§2.1): 'If a dose of CABLIVI is missed during the plasma exchange period, it should be given as soon as possible. If a dose of CABLIVI is missed after the plasma exchange period, it can be administered within 12 hours of the scheduled time of administration. Beyond 12 hours, the missed dose should be skipped and the next daily dose administered according to the usual dosing schedule.' If the first intravenous dose is missed and plasma exchange has already been given, 'administer the first CABLIVI dose intravenously and administer the next dose subcutaneously on the following day according to the usual dosing schedule.' SURGERY (§2.2): 'Withhold CABLIVI treatment 7 days prior to elective surgery, dental procedures, or other invasive interventions.' PREPARATION (§2.3 / §3): 11 mg lyophilised powder in a single-dose vial; 'Reconstitute CABLIVI before administration using the provided syringe containing 1 mL Sterile Water for Injection, USP, to yield an 11 mg/mL single-dose solution'; 'Use the CABLIVI solution immediately. If not, use CABLIVI within 4 hours after reconstitution when stored in the refrigerator at 2°C to 8°C.' SELF-ADMINISTRATION: adult patients or adult caregivers may inject subcutaneously after proper instruction, if a healthcare provider judges it appropriate. US labelling — confirm against the UK SPC before UK use.

Paediatric dose

Route: Intravenous for the first dose then subcutaneous, as for adults — §2.3: 'In pediatric patients 12 years of age and older, CABLIVI must be administered by a healthcare provider or an adult caregiver.'
Concentration: 11 mg/ml
§8.4 Pediatric Use, verbatim: 'The safety and effectiveness of CABLIVI in pediatric patients 12 years of age and older with acquired thrombotic thrombocytopenic purpura (aTTP), in combination with plasma exchange and immunosuppressive therapy have been established... The safety and effectiveness of CABLIVI in pediatric patients less than 12 years of age have not been established.' NO separate paediatric dose figure — no weight band, no per-kg rule — appears anywhere in the fetched §2 Dosage and Administration, so no paediatric dose is asserted here and the weight-based calculator is deliberately disabled. Do not assume the adult 11 mg schedule transfers unchanged to adolescents: verify against the full prescribing information / UK SPC and a children's formulary. Concentration shown is the reconstituted solution strength from §2.3 ('to yield an 11 mg/mL single-dose solution'). §6: 'In pediatric patients, the most frequently reported adverse reactions are epistaxis and tachycardia.'

Dose adjustments

Renal

Not stated — no renal-impairment dosing statement appears in any fetched section of this US label (§2.1–2.3, §3, §4, §5.1, §6, §7, §8.1, §8.4, §8.5).

Hepatic

Not stated — no hepatic-impairment dosing statement appears in any fetched section of this US label. Verify against the UK SPC.

Dose independently re-sourced from US FDA prescribing information (openFDA / DailyMed) — cross-check; US labelling may differ from UK — clinician-reviewed. Always confirm against the product SmPC and your local formulary before prescribing.

Contraindications

  • Previous severe hypersensitivity reaction to caplacizumab or to any of the excipients — §4, verbatim: 'CABLIVI is contraindicated in patients with a previous severe hypersensitivity reaction to caplacizumab-yhdp or to any of the excipients. Hypersensitivity reactions have included urticaria.'
  • Not a contraindication but a §5.1 / §7 avoidance: 'Avoid concomitant use of CABLIVI with antiplatelet agents, thrombolytic drugs, heparin, or anticoagulants' — concomitant use increases bleeding risk; the risk is also increased in patients with underlying coagulopathies (e.g. haemophilia, other coagulation factor deficiencies).
  • Not a contraindication but a §2.2 rule: withhold caplacizumab for 7 days before elective surgery, dental procedures or other invasive interventions.

Side effects

  • Most common adverse reactions in adults (incidence >15%), §6: epistaxis, headache and gingival bleeding.
  • Haemorrhage is the labelled key risk — §5.1: 'In clinical studies, severe bleeding adverse reactions of epistaxis, gingival bleeding, upper gastrointestinal hemorrhage, and metrorrhagia were each reported in 1% of subjects. Overall, bleeding events occurred in approximately 58% of patients on CABLIVI versus 43% of patients on placebo.'
  • Post-marketing: 'cases of life-threatening and fatal bleeding were reported in patients receiving CABLIVI' (§5.1).
  • Most frequently reported adverse reactions in paediatric patients, §6: epistaxis and tachycardia.
  • Safety database: 106 patients with aTTP across HERCULES (71 on caplacizumab) and TITAN (35 on caplacizumab), age 18–79 years, median treatment duration 35 days (range 1–77 days).
  • This side-effect list may be incomplete — check the full SPC.

Monitoring

  • Assess and monitor closely for bleeding — §7: 'Concomitant use of CABLIVI with any anticoagulant, thrombolytic drugs, heparin or antiplatelet agent may increase the risk of bleeding. Avoid concomitant use when possible. Assess and monitor closely for bleeding with concomitant use.'
  • Interrupt treatment for clinically significant bleeding — §5.1: 'Interrupt use of CABLIVI if clinically significant bleeding occurs. If needed, von Willebrand factor…' (the sentence is cut at the source-fetch limit).
  • Track markers of persistent underlying disease when deciding on extension — §2.1: 'If after initial treatment course, sign(s) of persistent underlying disease such as suppressed ADAMTS13 activity levels remain present, treatment may be extended for a maximum of 28 days.'
  • Count recurrences — §2.1: 'Discontinue CABLIVI if the patient experiences more than 2 recurrences of aTTP, while on CABLIVI.'
  • In pregnancy (§8.1): 'Pregnant women receiving CABLIVI should be carefully monitored for evidence of excessive bleeding' and 'Monitor neonates for bleeding.'

Clinical monograph

How it works

It binds the A1 domain of von Willebrand factor, blocking its interaction with platelet glycoprotein Ib and thereby preventing the ultra-large von Willebrand factor–platelet microthrombi that drive organ ischaemia in TTP.

Prescribing in practice

  • Bleeding is the predominant adverse effect because it directly impairs platelet adhesion; it should be interrupted around invasive procedures and used cautiously with other antithrombotic agents.
  • It is given in addition to — not instead of — plasma exchange and immunosuppression, and is typically continued for a period after plasma exchange ends to prevent relapse.
  • Von Willebrand factor activity is suppressed during treatment, so it should be withheld and von Willebrand factor concentrate considered if major bleeding occurs.

Monitoring

Monitor platelet count for treatment response and watch closely for mucocutaneous and other bleeding throughout therapy.

Counselling the patient

  • It is given by injection as part of a combined treatment for TTP.
  • Report any bleeding, including nosebleeds, gum bleeding or heavy bruising, without delay.
  • Tell the team before any dental or surgical procedure so it can be paused.

Evidence & guidelines

Caplacizumab is recommended by NICE for acquired thrombotic thrombocytopenic purpura, with efficacy demonstrated in the HERCULES trial.

Reference: HERCULES Trial (Scully et al. NEJM 2019); NICE TA667; BSH TTP Guidelines 2020; SPC Cablivi; 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.

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