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A Phase II Study with ICL670 (Exjade®), a Once-Daily Oral Iron Chelator, in Patients with Various Transfusion-Dependent Anemias and Iron Overload.

2004· article· en· W2550586375 on OpenAlexaffabout
John B. Porter, Elliott Vichinsky, Christian Rosé, Antonio Piga, Nancy F. Olivieri, Norbert Gattermann, Johan Maertens, B. Rabault, Insa Gathmann, Daniele Alberti

Bibliographic record

VenueBlood · 2004
Typearticle
Languageen
FieldMedicine
TopicHemoglobinopathies and Related Disorders
Canadian institutionsToronto General Hospital
Fundersnot available
KeywordsDeferasiroxMedicineThalassemiaMyelodysplastic syndromesDeferiproneAnemiaDeferoxaminePopulationPediatricsInternal medicineBone marrow

Abstract

fetched live from OpenAlex

Abstract Iron overload is a potentially life-threatening medical problem for patients with chronic transfusion-dependent anemias who cannot receive adequate iron chelation with existing therapies. ICL670 (deferasirox) is an investigational, tridentate, once-daily oral iron chelator that has demonstrated high iron binding potency and selectivity. The efficacy and safety of ICL670 is being investigated across a broad range of patient ages in a Phase II multicenter study in 7 countries in patients who are transfusion-dependent due to a variety of chronic anemias. The study population includes patients with various rare chronic anemias (n=98) and β-thalassemia patients who cannot be adequately treated with deferoxamine (n=86). The rare anemia category comprises 47 patients with myelodysplastic syndrome, 28 with Diamond-Blackfan anemia and 23 with other anemias of diverse etiologies. Between March and November 2003, 184 patients were enrolled in the following countries: Italy (57), US (36), France (20), UK (20), Canada (18), Germany (17), Belgium (16). Based on liver iron content (LIC) at baseline (2–3, >3–7, >7–14 and >14 mg Fe/g dw), patients were allocated to receive once daily oral ICL670 at doses of 5, 10, 20 or 30 mg/kg, respectively. Treatment was for one year initially, to be followed by an extension phase. LIC, the primary outcome measure, was assessed at baseline by liver biopsy or, when biopsy was contraindicated and also in some pediatric patients, non-invasively by magnetic susceptometry using a Superconducting QUantum Interference Device (SQUID). LIC will be reassessed after 12 months of therapy in each patient using the same methodology as at baseline. Liver biopsies are analyzed at a single center (Rennes, France) and 3 centers (Turin, Italy; Hamburg, Germany; Oakland, US) are performing SQUID assessments. At baseline, median (25–75th percentiles) LIC was 19.6 mg Fe/g dw (15.1–28.8) by biopsy and 9.1 (6.8–13.3) in those patients assessed by SQUID. Baseline patient demographics and disease characteristics (median values) are summarized in the table. ICL670 has been generally well tolerated. As of 30 April, 28 patients, predominantly with rare anemias, had discontinued the study due mainly to complications of the underlying disease. The key efficacy and safety data from the initial 12 months of therapy will be available for presentation in early December 2004. Disease group (by initial dose) Rare anemias (n = 98) β-thalassemia (n = 86) ≤ 10 mg/kg n = 16 20 mg/kg n = 29 30 mg/kg n = 53 ≤ 10 mg/kg n = 10 20 mg/kg n = 23 30 mg/kg n = 53 Age (yrs) median 56 39 49 21 23 25 No. of ≥ pts 2 - <16 yrs 4 10 6 4 8 3 No. of ≥ pts 16 - <50 yrs 2 7 21 6 14 49 No. of ≥ pts 50yrs 10 12 26 0 1 1 No. of males/females 10/6 13/16 27/26 3/7 15/8 25/28 LIC (mg Fe/g dry weight) 4.3 9.7 21.8 4.3 9.7 23.1 No. of pts with biopsy/SQUID 2/14 7/22 43/10 6/4 13/10 49/4 Serum ferritin (ng/ml) 1568 2751 3661 2131 2951 4516

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 distilled prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.000
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: none
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.539
Threshold uncertainty score0.838

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.000
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.000
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0000.000

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 head0.219
Teacher spread0.215 · 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 teacher head, not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
Domainnot available
GenreEmpirical

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".

Quick stats

Citations30
Published2004
Admission routes2
Has abstractyes

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