HEM iSMART-D: Trametinib + Dexamethasone + Chemotherapy in Children With Relapsed or Refractory Hematological Malignancies

NCT ID: NCT05658640

Last Updated: 2025-09-16

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

PHASE1/PHASE2

Total Enrollment

26 participants

Study Classification

INTERVENTIONAL

Study Start Date

2023-11-14

Study Completion Date

2029-04-01

Brief Summary

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HEM-iSMART is a master protocol which investigates multiple investigational medicinal products in children, adolescents and young adults (AYA) with relapsed/refractory (R/R) ALL and LBL. Sub-protocol D is a phase I/II trial evaluating the safety and efficacy of trametinib in combination with dexamethasone, cyclophosphamide and cytarabine in children and AYA with R/R ped ALL/LBL whose tumor present with alterations in the RAS-RAF-MAPK pathway.

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Detailed Description

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HEM-iSMART is a master protocol with sub-protocols. The overarching objective is that introducing targeted therapy using a biomarker driven approach for treatment stratification may improve the outcome of children with R/R acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LBL) It is characterized by a shared framework that allows for the investigation of multiple IMPs and generate pivotal safety and efficacy evidence within the sub-protocols to establish and define the benefits and risks of new treatments for children with R/R leukemia.

Sub-Protocol D within HEM-iSMART, is a phase I/II, multicenter, international, open-label clinical trial designed to evaluate the safety, tolerability, pharmacokinetics (PK) and efficacy of trametinib in combination with dexamethasone, cyclophosphamide and cytarabine in children, adolescents and young with R/R ALL and LBL. Patients with actionable alterations in the RAS-RAF-MAPK pathway will be eligible for sub-protocol D including but not limited to KRAS, NRAS, HRAS, FLT3, PTPN11, MAP2K1, MP2K1 hotspot mutations, cCBL; NF1 del.

Conditions

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Acute Lymphoblastic Leukemia, in Relapse Lymphoblastic Lymphoma (Precursor B-Lymphoblastic Lymphoma/Leukaemia) Recurrent Lymphoblastic Lymphoma (Precursor T-Lymphoblastic Lymphoma/Leukaemia) Recurrent Lymphoblastic Lymphoma (Precursor B-Lymphoblastic Lymphoma/Leukaemia) Refractory Lymphoblastic Lymphoma (Precursor T-Lymphoblastic Lymphoma/Leukaemia) Refractory

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Sub-study D

Trametinib + dexamethasone + cyclophosphamide and cytarabine.

Each cycle lasts 28 days.

Cycle 1: Trametinib is given orally continuously once a day in tablets or oral formulation depending on the age and weight of the patient. Dexamethasone is given intravenously (IV)/orally on days 1 to 5. Cyclophosphamide is given IV on day 3. Cytarabine is given IV in two blocks of 4 days each one week apart from day 5.

Cycle 2 and subsequent cycles: Trametinib is given orally continuously once a day in tablets or oral formulation depending on the age and weight of the patient. Dexamethasone is given intravenously (IV)/orally on days 1 to 5. Cyclophosphamide is given IV on day 1. Cytarabine is given IV in two blocks of 4 days each one week apart from day 3.

Patients in dose level -1, receive only 1 block of cytarabine per cycle.

All patients receive age adapted intrathecal chemotherapy.

Group Type EXPERIMENTAL

Trametinib

Intervention Type DRUG

Oral

Dexamethasone

Intervention Type DRUG

Oral/ Intravenous

Cyclophosphamide

Intervention Type DRUG

Intravenous

Cytarabine

Intervention Type DRUG

Intravenous

Intrathecal chemotherapy

Intervention Type DRUG

IT: Methotrexate +/- prednisone/hydrocortisone/cytarabine according to the degree of central nervous involvement

Interventions

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Trametinib

Oral

Intervention Type DRUG

Dexamethasone

Oral/ Intravenous

Intervention Type DRUG

Cyclophosphamide

Intravenous

Intervention Type DRUG

Cytarabine

Intravenous

Intervention Type DRUG

Intrathecal chemotherapy

IT: Methotrexate +/- prednisone/hydrocortisone/cytarabine according to the degree of central nervous involvement

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

1. Children between 1 year (≥ 12 months) and 18 years of age at the time of first diagnosis and less than 21 years at the time of inclusion. Patients under 6 years old must weigh at least 7 kg at the time of enrollment. Patients over 6 years old must weigh at least 10 kg at the time of enrollment.
2. Performance status: Karnofsky performance status (for patients \>12 years of age) or Lansky Play score (for patients

* 12 years of age) ≥ 50% (Appendix I).
3. Written informed consent from parents/legal representative, patient, and age-appropriate assent before any study specific screening procedures are conducted, according to local, regional or national guidelines.
4. Patients must have had molecular profiling and flow-cytometric analysis of their recurrent or refractory disease at a time-point before the first inclusion into this trial (see section 9.1 of this protocol for detailed description of the molecular diagnostics required). Drug response profiling and methylation is highly recommended but not mandatory.

Patients with molecular profiling at first diagnosis lacking molecular diagnostics at relapse or refractory disease may be allowed to be included after discussion with the sponsor.
5. Patients whose tumor present RAS pathway activating mutations including but not limited to KRAS, NRAS, HRAS, FLT3, PTPN11, MAP2K1, MP2K1 hotspot mutations, cCBL; NF1 del, as detected by molecular profiling.
6. Adequate organ function:

* RENAL AND HEPATIC FUNCTION (Assessed within 48 hours prior to C1D1) :

* Serum creatinine ≤ 1.5 x upper limit of normal (ULN) for age or calculated creatinine clearance as per the Schwartz formula or radioisotope glomerular filtration rate ≥ 60 mL/min/1.73 m2.
* Direct bilirubin ≤ 2 x ULN (≤ 3.0 × ULN for patients with Gilbert's syndrome).
* Alanine aminotransferase (ALT)/serum glutamic pyruvic transaminase (SGPT) ≤ 5 x ULN; aspartate aminotransferase (AST)/serum glutamic oxaloacetic transaminase/SGOT ≤ 5 x ULN. Note: Patients with hepatic disfunction related to the underling disease can be eligible even if they do not fulfill the aforementioned values for hepatic transaminases. In these cases, patients need to be discussed with the sponsor to confirm the eligibility.
* CARDIAC FUNCTION:

* Shortening fraction (SF) \>29% (\>35% for children \< 3 years) and/or left ventricular ejection fraction (LVEF) ≥50% at baseline, as determined by echocardiography or MUGA.
* Absence of QTcF prolongation (QTc prolongation is defined as \>450 msec on baseline ECG, using the Fridericia correction), or other clinically significant ventricular or atrial arrhythmia.

Exclusion Criteria

7. Pregnancy or positive pregnancy test (urine or serum) in females of childbearing potential. Pregnancy test must be performed within 7 days prior to C1D1.
8. Sexually active participants not willing to use highly effective contraceptive method (pearl index \<1) as defined in CTFG HMA 2020 (Appendix II) during trial participation and until 6 months after end of antileukemic therapy.
9. Breast feeding.
10. Impairment of gastrointestinal (GI) function or GI disease that may significantly alter drug absorption of oral drugs (e.g., ulcerative diseases, uncontrolled nausea, vomiting, diarrhea, or malabsorption syndrome) in case of oral IMPs.
11. Have a known immediate or delayed hypersensitivity reaction or idiosyncrasy to the study drugs, or drugs chemically related to study treatment or excipients that contraindicate their participation, including conventional chemotherapeutics (i.e. cytarabine and cyclophosphamide, intrathecal agents) and corticoids.
12. Known active viral hepatitis or known human immunodeficiency virus (HIV) infection or any other uncontrolled infection.
13. Severe concomitant disease that does not allow treatment according to the protocol at the investigator's discretion.
14. Subjects unwilling or unable to comply with the study procedures.
15. Previous treatment with trametinib.
16. Current use of a prohibited medication or herbal preparation or requires any of these medications during the study.

See Section 7 and Appendix III for details. Drugs inducing QTc changes (prolongation of the QT interval or inducing Torsade de Points) are not permitted.
17. Unresolved toxicity greater than NCI CTCAE v 5.0 ≥ grade 2 from previous anti-cancer therapy, including major surgery, except those that in the opinion of the investigator are not clinically relevant given the known safety/toxicity profile of the study treatment (e.g., alopecia and/or peripheral neuropathy related to platinum or vinca alkaloid based chemotherapy) (Common Terminology Criteria for Adverse Events (CTCAE) (cancer.gov).
18. Active acute graft versus host disease (GvHD) of any grade or chronic GvHD of grade 2 or higher. Patients receiving any agent to treat or prevent GvHD post bone marrow transplant are not eligible for this trial.
19. Received immunosuppression post allogenic HSCT within one moth of study entry.
20. History or current evidence of retina vein occlusion (RVO) or central serous retinopathy are excluded.
21. Wash-out periods of prior medication:

1. CHEMOTHERAPY: At least 7 days must have elapsed since the completion of cytotoxic therapy, with the exception of hydroxyurea, 6-mercaptopurine, oral methotrexate and steroids which are permitted up until 48 hours prior to initiating protocol therapy. Patients may have received intrathecal therapy (IT) at any time prior to study entry.
2. RADIOTHERAPY: Radiotherapy (non-palliative) within 21 days prior to the first dose of drug. Palliative radiation in past 21 days is allowed.
3. HEMATOPOIETIC STEM CELL TRANSPLANTATION (HSCT): Autologous HSCT within 2 months prior to the first study drug dose; Allogeneic HSCT within 3 months prior to the first study drug dose.
4. IMMUNOTHERAPY: At least 42 days must have elapsed after the completion of any type of immunotherapy other than monoclonal antibodies (e.g. CAR-T therapy)
5. MONOCLONAL ANTIBODIES AND INVESTIGATIONAL DRUGS: At least 21 days or 5 times the half-life (whichever is shorter) from prior treatment with monoclonal antibodies or any investigational drug under investigation must have elapsed before the first study drug.
6. SURGERY: Major surgery within 21 days of the first dose. Gastrostomy, ventriculo-peritoneal shunt, endoscopic ventriculostomy, tumor biopsy and insertion of central venous access devices are not considered major surgery.
Minimum Eligible Age

1 Year

Maximum Eligible Age

21 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Innovative Therapies For Children with Cancer Consortium

OTHER

Sponsor Role collaborator

IBFM

INDUSTRY

Sponsor Role collaborator

Fight Kids Cancer

OTHER

Sponsor Role collaborator

Princess Maxima Center for Pediatric Oncology

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Paco Bautista, MD PhD

Role: PRINCIPAL_INVESTIGATOR

Princess Máxima Center

Michel Zwaan, Prof. dr.

Role: STUDY_CHAIR

Princess Máxima Center

Locations

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St. Anna Kinderspital

Vienna, , Austria

Site Status NOT_YET_RECRUITING

Universitair Ziekenhuis Gent

Ghent, , Belgium

Site Status NOT_YET_RECRUITING

Rigshospitalet Copenhagen

Copenhagen, , Denmark

Site Status RECRUITING

Helsinki University Hospital, New Children's Hospital

Helsinki, , Finland

Site Status NOT_YET_RECRUITING

Hôpital des Enfants GH Pellegrin - CHU de Bordeaux

Bordeaux, , France

Site Status NOT_YET_RECRUITING

CHRU Lille - Hôpital Jeanne de Flandre

Lille, , France

Site Status NOT_YET_RECRUITING

Centre Léon Bérard

Lyon, , France

Site Status NOT_YET_RECRUITING

Hopital La Timone - Enfants

Marseille, , France

Site Status NOT_YET_RECRUITING

CHU Nantes Hôpital Mère-Enfant

Nantes, , France

Site Status NOT_YET_RECRUITING

Hôpital Robert Debré

Paris, , France

Site Status NOT_YET_RECRUITING

Universitätsklinikum Augsburg

Augsburg, , Germany

Site Status NOT_YET_RECRUITING

Charité Universitätsmedizin Berlin

Berlin, , Germany

Site Status RECRUITING

Universitätsklinikum Essen

Essen, , Germany

Site Status NOT_YET_RECRUITING

Universitätsklinikum Frankfurt

Frankfurt, , Germany

Site Status NOT_YET_RECRUITING

Universitätsklinikum Münster

Münster, , Germany

Site Status NOT_YET_RECRUITING

Our Lady's Hospital for Sick Children

Dublin, , Ireland

Site Status NOT_YET_RECRUITING

Schneider's Children's Medical Center

Petah Tikva, , Israel

Site Status NOT_YET_RECRUITING

Sheba Medical Center Hospital

Ramat Gan, , Israel

Site Status NOT_YET_RECRUITING

IRCCS Istituto Giannina Gaslini

Genova, , Italy

Site Status NOT_YET_RECRUITING

Fondazione MBBM c/o Centro ML Verga

Monza, , Italy

Site Status NOT_YET_RECRUITING

Padova Azienda Ospedaliera

Padua, , Italy

Site Status NOT_YET_RECRUITING

Ospedale Pediatrico Bambino Gesù, IRCCS

Roma, , Italy

Site Status NOT_YET_RECRUITING

Ospedale Infantile Regina Margherita

Turin, , Italy

Site Status NOT_YET_RECRUITING

Princess Máxima Center for Pediatric Oncology

Utrecht, Utrecht, Netherlands

Site Status RECRUITING

Oslo University Hospital

Oslo, , Norway

Site Status RECRUITING

Hospital Vall d'Hebron

Barcelona, , Spain

Site Status NOT_YET_RECRUITING

Hospital Sant Joan de Déu de Barcelona

Barcelona, , Spain

Site Status NOT_YET_RECRUITING

Hospital Infantil Universitario Niño Jesús

Madrid, , Spain

Site Status NOT_YET_RECRUITING

La Fe

Valencia, , Spain

Site Status NOT_YET_RECRUITING

Karolinska university hospital

Stockholm, , Sweden

Site Status RECRUITING

University Children's Hospital Zürich

Zurich, , Switzerland

Site Status NOT_YET_RECRUITING

Bristol Royal Hospital for Children

Bristol, , United Kingdom

Site Status NOT_YET_RECRUITING

Great Ormond Street Hospital for Children NHS Trust

London, , United Kingdom

Site Status NOT_YET_RECRUITING

Royal Manchester Children's Hospital

Manchester, , United Kingdom

Site Status NOT_YET_RECRUITING

Great North Children's Hospital

Newcastle, , United Kingdom

Site Status NOT_YET_RECRUITING

Royal Marsden NHS Trust

Sutton, , United Kingdom

Site Status NOT_YET_RECRUITING

Countries

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Austria Belgium Denmark Finland France Germany Ireland Israel Italy Netherlands Norway Spain Sweden Switzerland United Kingdom

Central Contacts

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Anne Elsinghorst

Role: CONTACT

+316 5000 6270

Facility Contacts

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Andishe Attarbaschi

Role: primary

Barbara De Moerloose

Role: primary

Rutta Tuckuviene

Role: primary

Antti Kyrönlahti

Role: primary

Stephane Ducassou

Role: primary

Bigitte Nelken

Role: primary

Nathalie Garnier

Role: primary

Paul Saultier

Role: primary

Fanny Rialland

Role: primary

Marion Strullu

Role: primary

Michael Frühwald

Role: primary

Arend von Stackelberg

Role: primary

Dirk Reinhardt

Role: primary

Jan-Hemming Klusmann

Role: primary

Birgit Burkhardt

Role: primary

Andrea Malone

Role: primary

Gil Gilad

Role: primary

Bella Bielorai

Role: primary

Concetta Micalizzi

Role: primary

Carmelo Rizzari

Role: primary

Marta Pillon

Role: primary

Franco Locatelli

Role: primary

Franca Fagioli

Role: primary

Anne A.L Elsinghorst

Role: primary

Jochen Büchner

Role: primary

Pablo Velasco

Role: primary

Jose Luis Dapena Diaz

Role: primary

Alba Rubio San Simón

Role: primary

Carol Fuentes

Role: primary

Anna Nilsson

Role: primary

Nastassja Scheidegger

Role: primary

Kristina Marshall

Role: primary

David O'Connor

Role: primary

Denise Bonney

Role: primary

Frederik van Delft

Role: primary

Donna Lancaster

Role: primary

Other Identifiers

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2021-003398-79

Identifier Type: -

Identifier Source: org_study_id

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