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Eltrombopag vs Standard Front Line Management for Newly Diagnosed Immune Thrombocytopenia (ITP) in Children

Phase 3
Completed
Conditions
Immune Thrombocytopenia
Interventions
Drug: Steroids
Drug: Rho(D) Immune Globulin
Registration Number
NCT03939637
Lead Sponsor
Baylor College of Medicine
Brief Summary

This is an investigator initiated, multicenter, open label, randomized phase 3 study for subjects with newly diagnosed ITP from ages 1 to less than 18 years old.

Detailed Description

This is a prospective, open label, randomized, two-arm, multi-center Phase 3 trial.

Patients with newly diagnosed ITP are randomized 2:1 to receive the experimental treatment, eltrombopag, or investigator's choice of 3 standard therapies. The primary objective is to determine if the proportion of patients with platelet response is significantly greater in patients treated with eltrombopag compared to those treated with standard therapies.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
122
Inclusion Criteria
  • Age: 1- <18 years
  • Newly diagnosed ITP (<3 months from diagnosis (first abnormal platelet count), per international working group definition17)
  • Platelets <30 x 10^9/L at screening
  • Requires pharmacologic treatment from the perspective of the treating clinician.

Need to treat is at the discretion of the investigator, but there should be clinical equipoise about the use of eltrombopag vs standard treatment options (patients should not, in the opinion of the investigator, require concomitant therapy at time of enrollment).

  • Treatment options include one of three standard therapies, (IVIg, steroids, or Anti-D). For example, if patient has previously shown no response to IVIg or steroids and is Rh-negative, patient would not be eligible for study.

  • Patient population includes both:

    1. Upfront treatment: Patient within 10 days of ITP diagnosis who has not received previous treatment OR

    2. Treatment failure: Patients who have failed standard management (observation or treatment with one or more first-line agents)

      • Failure of observation: no platelet recovery (>30 x 10^9/L) with observation >10 days from diagnosis, with need to treat
      • Poor response to first-line agent (platelets remain <30 x10^9/L)
      • Initial response to first-line agent, but response wanes and platelets fall below 30 x10^9/L
  • Family willing and able to return for required lab studies

Exclusion Criteria
  • Severe bleeding: Buchanan Overall Grade 4 or 5 bleeding, or severe bleeding requiring emergent treatment at the discretion of the provider. (e.g., intracranial hemorrhage, pulmonary hemorrhage, bleeding with ongoing need for pRBC transfusion)

  • Prior treatment with TPO-RA (eltrombopag or romiplostim)

  • Known secondary ITP (due to lupus, CVID, ALPS)

  • Known HIV (or history of HIV positivity) or Hepatitis C (screening not required if no clinical suspicion)

  • Evans Syndrome: positive direct Coombs with evidence of active hemolysis (elevated lactate dehydrogenase (LDH) or reticulocyte count not attributable to recent treatment or bleeding)

  • Any Malignancy

  • History of stem cell transplant or solid organ transplant

  • aspartate aminotransferase (AST) or ALT >2 x upper limit of normal (ULN)

  • Total bilirubin >1.5 × ULN

  • Subjects with liver cirrhosis (as determined by the investigator)

  • Creatinine >2.5 × ULN

  • Known active or uncontrolled infections not responding to appropriate therapy

  • On anticoagulation or anti-platelet agents

  • Known thrombophilic risk factors. Exception: Subjects for whom the potential benefits of participating in the study outweigh the potential risks of thromboembolic events, as determined by the investigator.

  • Baseline ophthalmic problems that may potentiate cataract development

  • Impaired cardiac function, such as:

    • Known prolonged QTc, with corrected QTc >450 msec
    • Other clinically significant cardio-vascular disease (e.g., uncontrolled hypertension, history of labile hypertension),
    • History of known structural abnormalities (e.g. cardiomyopathy).
  • History or current diagnosis of cardiac disease indicating significant risk of safety for patients participating in the study such as uncontrolled or significant cardiac disease, including any of the following:

    • Recent myocardial infarction (within last 6 months),
    • Uncontrolled congestive heart failure,
    • Unstable angina (within last 6 months),
    • Clinically significant (symptomatic) cardiac arrhythmias (e.g., sustained ventricular tachycardia, and clinically significant second or third degree AV block without a pacemaker.)
    • Long QT syndrome, family history of idiopathic sudden death, congenital long QT syndrome or additional risk factors for cardiac repolarization abnormality, as determined by the investigator.
  • Known immediate or delayed hypersensitivity reaction to eltrombopag or its excipient.

  • Pregnant, breastfeeding, or unwilling to practice birth control during participation in the study. Women of childbearing potential (have achieved menarche) must have a negative serum or urine pregnancy test and agree to use basic methods of contraception (if sexually active) or maintain abstinence for the duration of the study. Basic contraception methods include:

    • Total abstinence (when this is in line with the preferred and usual lifestyle of the subject. Periodic abstinence (e.g., calendar, ovulation, symptothermal, post-ovulation methods) and withdrawal are not acceptable methods of contraception
    • Female sterilization (have had surgical bilateral oophorectomy with or without hysterectomy), total hysterectomy, or tubal ligation at least six weeks before taking study treatment. In case of oophorectomy alone, only when the reproductive status of the woman has been confirmed by follow up hormone level assessment
    • Male sterilization (at least 6 months prior to screening). The vasectomized male partner should be the sole partner for that subject
    • Barrier methods of contraception: Condom or Occlusive cap. For the UK: with spermicidal foam/gel/film/cream/ vaginal suppository
    • Use of oral, injected or implanted hormonal methods of contraception or placement of an intrauterine device (IUD) or intrauterine system (IUS), or other forms of hormonal contraception that have comparable efficacy (failure rate <1%), for example hormone vaginal ring or transdermal hormone contraception. In case of use of oral contraception women should have been stable on the same pill for a minimum of 3 months before taking study treatment.
  • Male patients who are sexually active and do not agree to abstinence or to use a condom during intercourse while taking eltrombopag, and for 7 days after stopping treatment.

  • History of alcohol/drug abuse

  • Presence of a medical condition that in the opinion of the Investigator would compromise the safety of the patient or the quality of the data.

  • Concurrent participation in an investigational study within 30 days prior to enrollment or within 5-half-lives of the investigational product, whichever is longer. Note: parallel enrollment in a non-therapeutic trial such as disease registry or biology study is permitted.

Other Eligibility Criteria Considerations All patients and/or their parents or legal guardians must sign a written informed consent (and assent when applicable)

  • Patients and/or parents who are unable to read at a grade 2 level will be excluded from the patient-reported outcome component of the study, as will non-English speaking patients and/or parents when there is no availability of translated versions in their spoken language . They will not be excluded from all other aspects of the study

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Standard first-line therapySteroidsSubjects randomized to the standard therapy arm will receive one of three treatments at the discretion of the treating physician. Patients who previously failed standard management prior to study entry must be treated with a different agent than their original failed agent. e.g. Patient who failed steroids could receive either IVIg or anti-D if randomized to the standard treatment arm. Standard therapy will be administered as commercially available drug. Investigator may choose amongst the following: * IVIg: IVIG 1 g/kg x1 (no steroids for pre-medication or adjunctive therapy) * Steroids: Prednisone/Prednisolone 4 mg/kg/day (Max 120 mg/day) x 4 days * Rho(D) Immune Globulin: Anti-D globulin 75 mcg/kg x1 (no steroids for pre-medication or adjunctive therapy)
Standard first-line therapyRho(D) Immune GlobulinSubjects randomized to the standard therapy arm will receive one of three treatments at the discretion of the treating physician. Patients who previously failed standard management prior to study entry must be treated with a different agent than their original failed agent. e.g. Patient who failed steroids could receive either IVIg or anti-D if randomized to the standard treatment arm. Standard therapy will be administered as commercially available drug. Investigator may choose amongst the following: * IVIg: IVIG 1 g/kg x1 (no steroids for pre-medication or adjunctive therapy) * Steroids: Prednisone/Prednisolone 4 mg/kg/day (Max 120 mg/day) x 4 days * Rho(D) Immune Globulin: Anti-D globulin 75 mcg/kg x1 (no steroids for pre-medication or adjunctive therapy)
Standard first-line therapyIVIGSubjects randomized to the standard therapy arm will receive one of three treatments at the discretion of the treating physician. Patients who previously failed standard management prior to study entry must be treated with a different agent than their original failed agent. e.g. Patient who failed steroids could receive either IVIg or anti-D if randomized to the standard treatment arm. Standard therapy will be administered as commercially available drug. Investigator may choose amongst the following: * IVIg: IVIG 1 g/kg x1 (no steroids for pre-medication or adjunctive therapy) * Steroids: Prednisone/Prednisolone 4 mg/kg/day (Max 120 mg/day) x 4 days * Rho(D) Immune Globulin: Anti-D globulin 75 mcg/kg x1 (no steroids for pre-medication or adjunctive therapy)
EltrombopagEltrombopagPatients randomized to eltrombopag will be treated for 12 weeks, with the possibility to continue therapy for up to 1 year depending on response.
Primary Outcome Measures
NameTimeMethod
Proportion of patients with a platelet response12 weeks

To determine if the proportion of patients with a platelet response is significantly greater in patients with newly diagnosed ITP treated with eltrombopag than those treated with standard first-line treatments

Secondary Outcome Measures
NameTimeMethod
Need for treatment6 months

No further need for treatment (binary) after 12 weeks or 6 months of study

Bleeding Score1 year

Poor bleeding score (binary) at 1, 2, 3, 4 weeks, 12 weeks, and 1 year after study enrollment defined as World Health Organization (WHO) Bleeding Scale ≥ 2 or Modified Buchanan Scale ≥ 3

Platelet response12 weeks

5.2.3 Platelet response (binary), defined as ≥ 3 of 4 weeks with platelets \>50 x109/L during weeks 6-12 of therapy, but patient required a rescue treatment during weeks 1-2 of study.

Number of 2nd line therapies52 weeks

Number of 2nd-line therapies in weeks 13-52

Regulatory T-Cells1 year

Absolute change in percentage of CD4+25+Foxp3+ regulatory T cells from baseline at 12 weeks and 1 year

Blood Iron values1 year

Serum iron, total iron binding capacity (TIBC), transferrin saturation, ferritin, mean corpuscular volume (MCV), and hemoglobin at 12 weeks, 6 months, and 1 year after study enrollment

Number of rescue therapies12 weeks

Cumulative number of rescue therapies required during the first 12 weeks of treatment

Treatment response1 year

Treatment response (binary endpoints) at 1 year defined as:

* CR is defined as platelet count \>/= 150 x 10\^9/L

* Primary Remission at 1 year is defined as CR at 1 year with no second-line agents required and \>/= 3 months after discontinuing most recent platelet active medication

* Disease resolution at 1 year is defined as complete response (CR) at 1 year \>/= 3 months after discontinuing most recent platelet active medication. May have received a second-line therapy, excluding rituximab or splenectomy.

* Disease stability at 1 year is defined as platelets \>/= 50 x 10\^9/L but \<150 x 10\^9/L \>/= 3 months after discontinuing most recent platelet active medication.

KIT Scores1 year

Change in parent proxy-reported Kids ITP tool (KIT) overall scores from baseline at 1 week, 4 weeks, 12 weeks, and 1 year after study enrollment

Hockenberry Fatigue Scale-Parent1 year

Total scale intensity ratings (continuous) from the Hockenberry Fatigue Scale-Parent (FS-P) at 1 week, 4 weeks, 12 weeks, and 1 year

Safety evaluations1 year

Safety evaluations as defined by:

* Abnormal liver function tests (LFTs):

ALT ≥ 3 x upper limit of normal (ULN) in patients with normal baseline ALT ≥ 3 x baseline or ≥ 5 x ULN (whichever is lower) in patients with abnormal baseline ALT ≥ 3 x ULN AND bilirubin ≥ 1.5 x ULN (\>35% direct)

* Incidence of adverse events

* Incidence of serious adverse events

Trial Locations

Locations (27)

Children's Hospital of Orange County

🇺🇸

Orange, California, United States

Children's of Alabama

🇺🇸

Birmingham, Alabama, United States

Phoenix CHildren's Hospital

🇺🇸

Phoenix, Arizona, United States

Arkansas Children's Hospital

🇺🇸

Little Rock, Arkansas, United States

UCSF Benioff Children's Hospital

🇺🇸

San Francisco, California, United States

Children's Hospital Colorado

🇺🇸

Aurora, Colorado, United States

Children's National Medical Center

🇺🇸

Washington, District of Columbia, United States

University of Florida College of Medicine

🇺🇸

Gainesville, Florida, United States

Alfac Cancer and Blood Disorder Center: Scottish Rite

🇺🇸

Atlanta, Georgia, United States

Ann & Robert H. Lurie Children's Hospital of Chicago

🇺🇸

Chicago, Illinois, United States

Riley Hospital for Children-Indiana University

🇺🇸

Indianapolis, Indiana, United States

Boston Children's Hospital

🇺🇸

Boston, Massachusetts, United States

Children's Hospital and Clinics of Minnesota

🇺🇸

Minneapolis, Minnesota, United States

University of Nebraska Medical Center

🇺🇸

Omaha, Nebraska, United States

Hackensack University Medical Center

🇺🇸

Hackensack, New Jersey, United States

Columbia University Irving Medical Center

🇺🇸

New York, New York, United States

Weill Cornell Medical College

🇺🇸

New York, New York, United States

Levine Cancer Institute

🇺🇸

Charlotte, North Carolina, United States

Duke University Medical Center

🇺🇸

Durham, North Carolina, United States

Nationwide Children's Hospital

🇺🇸

Columbus, Ohio, United States

Randall Children's Hospital

🇺🇸

Portland, Oregon, United States

Oregon Health and Science University

🇺🇸

Portland, Oregon, United States

The Children's Hospital of Philadelphia

🇺🇸

Philadelphia, Pennsylvania, United States

Hasbro Children's Hospital

🇺🇸

Providence, Rhode Island, United States

St. Jude Children's Hospital

🇺🇸

Memphis, Tennessee, United States

Texas Children's Hospital

🇺🇸

Houston, Texas, United States

University of Wisconsin

🇺🇸

Madison, Wisconsin, United States

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