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Record W4411102757 · doi:10.1093/jscdis/yoaf013.015

Design of the RHEMEDY Study: A Phase 2/3 Safety, Efficacy and Pharmacokinetics Study of CSL889 in Adults and Adolescents With Sickle Cell Disease During Vaso-Occlusive Crisis

2025· article· en· W4411102757 on OpenAlexaboutno aff
Victor R. Gordeuk, David C. Brousseau, Christian Kassasseya, Paul Shore, Nicole Avalos, Kerstin Jung, Jeannine S. McCune, Francine R. Wilson, Hu Zou, Gregory J. Kato, Paula Tanabe, Bart J. Biemond

Bibliographic record

VenueJournal of sickle cell disease. · 2025
Typearticle
Languageen
FieldMedicine
TopicHemoglobinopathies and Related Disorders
Canadian institutionsnot available
Fundersnot available
KeywordsVaso-occlusive crisisPharmacokineticsMedicineDiseaseSickle cell anemiaInternal medicinePharmacology

Abstract

fetched live from OpenAlex

Abstract Background Vaso-occlusive crisis (VOC) is the primary reason for hospitalization among patients with sickle cell disease (SCD). The severe pain that characterizes VOC is due to impaired blood circulation triggered by adhesion of neutrophils, platelets and rigid erythrocytes to the blood vessel wall. Currently, there are no approved treatments aimed at immediately improving blood circulation during a VOC episode. Current treatment with analgesics such as opioids only addresses the resulting pain without improving blood circulation. Experiments in sickle cell mice show that free heme and depletion of hemopexin, the natural plasma protein that neutralizes heme, contribute to vaso-occlusion. Free heme activates sterile inflammatory pathways that promote adhesiveness of neutrophils, platelets and endothelial cells. Free heme is generated by intravascular hemolysis in SCD, which overwhelms and depletes plasma hemopexin. In sickle cell mice with vaso-occlusion triggered in various ways, restoring hemopexin levels through intravenous (IV) administration of hemopexin restores blood flow (Gentinetta 2022). Our recent phase 1 study [NCT04285827] demonstrated the safety and pharmacokinetics (PK) of single IV doses of hemopexin purified from human plasma (CSL889) in adults with SCD with or without VOC. The RHEMEDY study (CSL889_2001, NCT06699849) seeks to evaluate the safety, effectiveness, and PK of CSL889 in adults and adolescents with SCD experiencing VOC. The goal is to administer CSL889 in an acute treatment center (emergency departments or infusion centers) or hospital as soon as possible after onset of acute pain due to VOC. Methods RHEMEDY is a 2-part, phase 2/3, multicenter, randomized, multiple-dose, double-blind, placebo-controlled adaptive study. Adults (and adolescents > =12 years; US, Canada, and others as approved) with SCD (any genotype) presenting within 72 hours of onset of a VOC requiring treatment with parenteral (injected or intranasal) opioids will be eligible. In part A (corresponding to Phase 2), 160 subjects will be randomized equally to one of 3 different dosing regimens of CSL889 or placebo The first dose of investigational product (IP) must be administered within 12 hours of the first parenteral opioid; IP will be added to standard-of-care treatment for VOC. The IP will be given IV once daily for 5 days or until their VOC resolves, whichever occurs first. In part B (Phase 3), 100 subjects will be randomized equally to a regimen selected from part A or placebo. Sample size can be adjusted up to 300 subjects based on observations at interim analysis. Informed consent will be sought preferably when subjects are in their usual state of health, prior to VOC. An independent data monitoring committee will oversee study conduct and safety. Results The primary efficacy endpoint is the time to resolution of VOC, measured by the time from first IP administration to discontinuation of parenteral opioids. Secondary endpoints include hospital admission rate, length of acute care and hospital stay, percentage of subjects experiencing VOC complications (acute chest syndrome [ACS], acute kidney injury, stroke), re-presentation to acute care for VOC/ACS, total opioid consumption, and speed of adequate pain relief. Safety endpoints include treatment-emergent adverse events and treatment-emergent anti-drug antibodies. PK parameters will be calculated, primarily from the first 24 adult and first 8 adolescent subjects. Conclusions The RHEMEDY study of CSL889 will provide data that may support registration of CSL889 to treat acute VOC. CSL889 has potential to treat an underlying cause of VOC with a new approach to shorten a VOC episode. The trial will assess any potential reduction in need for hospitalization or in duration of hospitalization. Figure 1Overall study design.Figure 2Individual subject flow.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.004
metaresearch head score (Gemma)0.002
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Randomized trial · Consensus signal: Randomized trial
GenreCandidate signal: Protocol · Consensus signal: none
Teacher disagreement score0.006
Threshold uncertainty score0.023

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0040.002
Meta-epidemiology (narrow)0.0020.001
Meta-epidemiology (broad)0.0030.002
Bibliometrics0.0000.000
Science and technology studies0.0010.001
Scholarly communication0.0010.001
Open science0.0010.000
Research integrity0.0020.004
Insufficient payload (model declined to judge)0.0060.002

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.005
GPT teacher head0.254
Teacher spread0.249 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designRandomized trial
Domainnot available
GenreProtocol

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

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Citations0
Published2025
Admission routes1
Has abstractyes

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