ClinicalTrials.gg
RecruitingNCT07285005S-KYUpdated Sep 21, 2026

A Study to Investigate Efficacy and Safety of KP-001 Compared With Placebo in Patients Aged ≥2 Years With Common VM, Common LM, or KTS/CLOVES Syndrome

A Phase 3 interventional study of KP-001 and Placebo in Venous Malformations, Lymphatic Malformations and Klippel-Trenaunay Syndrome, sponsored by Kaken Pharmaceutical. Recruiting at 5 sites in 3 countries. Open to participants aged 2 Years and older. Per ClinicalTrials.gov, last updated 2026-09-21.

Sponsored by Kaken Pharmaceutical · Phase 3, Interventional, and Treatment

From the registry’s dates

  • Started May 2026; still recruiting 5 months later.
Phase
Phase 3
Study type
Interventional
Enrollment
150
Allocation
Randomized
Ages
2 Years and older
Sex
All
01

Study summary

This is a phase 3, double-blind, randomized, placebo-controlled, parallel group, adaptive, multicenter study planned to be conducted at multiple sites in North America, Canada, Taiwan and South Korea.

The purpose of this study is to measure the efficacy and safety of KP-001 compared with placebo in patients aged ≥2 years with common VM, common LM, or KTS/CLOVES syndrome.

An independent data monitoring committee (DMC) will be established to determine whether to discontinue or continue the study. It will also determine the redesign of the number of cases based on the result of the interim analysis.

The study will comprise the following:

  • Screening Period: Up to 42 days prior to the first dose of study intervention.
  • Treatment Period 1: This is a double-blind period in which KP-001 100 mg (or lower dose depending on their body weight) or placebo will be administered to patients once daily after breakfast until Week 24.
  • Treatment Period 2: After 24 weeks of double blind treatment, all patients will switch to the KP-001 open label extension and treated up to Week 52.
  • Follow-up Visit: This visit will occur 30 days after the last dose of study intervention, and assessments will be performed per the SoA.
  • Discontinuation Visit: Patients who discontinue study intervention will be requested to continue participating in the study and assessments will be performed per the SoA. If the patients request to withdraw from the study, all tests and evaluations when possible will be performed at Discontinuation visit.
Read the detailed description

Safety and Exploratory Assessments Vital signs (blood pressure, heart rate, respiratory rate, and body temperature) will be monitored at each scheduled visit for safety.

Safety laboratory assessments will include hematology, serum chemistry, coagulation parameters (e.g., PT/INR, aPTT, fibrinogen), and urinalysis.

Coagulation biomarkers other than D-dimer will be collected for exploratory/safety purposes only and will not be included in the reported outcome measures.

Exploratory analyses may include the relationship between changes in Symptom Numeric Rating Scale (NRS) scores and Patient Global Impression of Severity (PGI-S) scores.

02

Conditions studied

  • Venous Malformations
  • Lymphatic Malformations
  • Klippel-Trenaunay Syndrome
  • CLOVES Syndrome
03

In context

Lymphatic Abnormalities

42 studies on the registry are indexed under Lymphatic Abnormalities; 17 are open to participants now.

This study's planned enrollment of 150 is above the median of 46 across 28 interventional studies indexed under Lymphatic Abnormalities.

Browse Lymphatic Abnormalities studies →

Lead sponsor

Kaken Pharmaceutical is the lead sponsor of 10 studies on the registry; 1 is open to participants now.

Counted across the registry records on this site, refreshed daily.

04

Who can participate

Ages eligible
2 Years and older
Sexes eligible
All
Accepts healthy volunteers
No

Go through the criteria one by one — yes, no or not sure — and take the list to your doctor or the study team. Kept in this browser only.

Inclusion criteria

  1. Patients aged 2 years or older at the time of consent or assent.
  2. Patients diagnosed with ISSVA classification of common VM, common (cystic) LM (including mixed type consisting mainly of either VM or LM), or KTS/CLOVES syndrome.
  3. Patients who cannot be cured by resection, who are difficult to resect based on the assessment of the Investigator, or who are considered to available treatment by the Investigator, or who have a to available treatment.
  4. Patients with at least one target lesion at least 4 cm in the longest diameter.
  5. Patients with at least one MRI-volumetric target lesion at screening that is determined to be evaluable by the central imaging evaluator.
  6. Patients with symptomatic disease, defined as:

    ・ For patients ≥8 years old: Pain NRS of ≥1 and ≤8 score at the screening visit will be eligible only if their daily pain NRS recorded via ePRO from screening to Day 1 (Week 0) does not show a maximum absolute change of ≥6 points. If patients are taking analgesic medication, there must be no change in the type or dosage of analgesic during the screening period. If patients do not have qualifiable pain, then Fatigue or Bleeding/Oozing NRS of ≥1 and ≤8 score at the screening visit is required. Patients with ≥6 points absolute change in pain NRS are eligible if at least one of either Fatigue OR Bleeding/Oozing NRS is ≥1 and ≤8.

    • For patients ≥3 years old to \<8 years old: Wong-Baker FACES pain rating scale equal to or greater than 2 points at screening visit. Patients who are ≥3 years old to \<8 years old without qualifiable pain will be recruited providing they have symptomatic disease as judged in the opinion of the Investigator (ie, any visible lesion or lesion affecting activities of daily living), and as far as they meet other inclusion criteria.
    • For patients 2 years old: Symptomatic disease as judged in the opinion of the Investigator (ie, any visible lesion or lesion affecting activities of daily living), and patients will be recruited as far as they meet other inclusion criteria.
  7. Patients whose pain from vascular malformations has been stable for at least 30 days prior to screening and, if taking analgesic medication, does not require a change in the type of analgesic medication or its dosage during the screening period.
  8. Patients who agree that they or their partner (if either of them is of ) will use appropriate (eg, condom and spermicide combination, low-dose pills or other appropriate contraceptive methods, sterilization, intrauterine device) from the time of consent until 90 days after the last dose of study intervention.
  9. Patients or their LAR who are able to give age-appropriate at the time of screening.
  10. Patients who are judged by the Investigator to be able to comply with the instructions of the Investigator and the study coordinator regarding the matters specified in the protocol, such as the use of study intervention and concomitant use of prohibited drugs.

Exclusion criteria

Exclusion Criteria:

  1. Patients with the following diseases: Simple telangiectatic malformation, lymphangiomatosis, lymphangiectatic malformation associated with Gorham's disease, lymphangiectasia, familial cutaneous mucocutaneous venous malformation, blue rubber ball-like nevus syndrome, M-CM/MCAP, CLAPO syndrome, Proteus syndrome, Parkes Weber syndrome, Sturge-Weber syndrome, Mafucci syndrome, Osler's disease, Cowden's disease, or Adams-Oliver syndrome.
  2. Patients with uncontrolled diabetes mellitus ( ≥ 7.0%) or diseases with abnormal glucose metabolism (glycogenic diseases, galactosemia, primary lactose intolerance, etc).
  3. Patients with ischemic heart disease, arrhythmia, or heart failure ( III or IV).
  4. Patients with gastrointestinal disorders that affect drug absorption, as determined by the Investigator.
  5. Patients with concomitant or pre-existing serious drug to PI3Kα inhibitors.
  6. Patients with allergy history of grade ≥ 3 and/or history of grade ≥ 3 allergic reactions to drug.
  7. Patients with known hypersensitivity to quinine.
  8. Patients with concomitant or pre-existing alcohol or drug abuse.
  9. Patients with ANC of \<1.5×10\^9/L.
  10. Patients with acute or chronic kidney disease and/or dialysis dependence. Patients with screening \<30 mL/min/1.73m\^2 using the beside Schwartz equation for patients aged \<18 years of age or CKD-EPI formula for >18 years of age will also be excluded.
  11. Patients who are judged by the Investigator to have hepatic impairment.
  12. Patients with total ≥1.5× for age (unless there is a history of Gilbert Syndrome), ≥2×ULN for age, or AST ≥2×ULN for age will be excluded.
  13. Patients with target lesion infection that require treatment within 28 days prior to screening.
  14. Patients who have undergone invasive treatment, including sclerotherapy or laser therapy, for the target lesion within 84 days prior to screening.
  15. Patients who have used other PI3Kα inhibitors or sirolimus within 84 days prior to screening.
  16. Patients who have participated in other clinical studies within 90 days prior to the date of consent.
  17. Patients who have participated in a clinical study of KP-001 for any period and have received an drug in the past year.
  18. Patients wearing orthodontic appliances, cochlear implants, etc, that may affect MRI, or patients in whom MRI is not feasible or, for example, patients who may have a to sedation and would require sedation in order to have MRI completed.
  19. Patients who are unable to take oral medications.
  20. Pregnant women, lactating female patients, female patients who may be pregnant, female patients who wish to become pregnant during the study period and up to 90 days after the last dose of study intervention, or male patients who have partners who wish to become pregnant.
  21. Patients with any other illness or medical condition who are judged by the Investigator to be inappropriate as patients for this study.
  22. Patients who have received or plan to receive during the study period.
Terms in these criteria, in plain language(14)

Underlined terms in the criteria open these too. Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.

Relapsed and refractory

Relapsed: the illness came back after treatment had worked. Refractory: it did not respond to treatment, or stopped responding while still on it.

Contraindication

A reason a treatment, test or procedure should not be given to a particular person, such as an allergy, another illness or a medicine they take.

Childbearing potential

Able to become pregnant: from the first period until menopause, unless permanently unable to through surgery (removal of the womb, or of both ovaries or both fallopian tubes).

Criteria for people of childbearing potential usually ask for a negative pregnancy test and for contraception during the study and for a time after it.

Contraception

Ways of preventing pregnancy. “Highly effective” methods fail less than 1% of the time a year when used correctly — for example an IUD or an implant.

Criteria usually say which methods count and how long to keep using them after the last dose, sometimes for male participants too. Some studies accept a partner’s vasectomy if it was confirmed successful and that person is the participant’s only sexual partner.

Informed consent

Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.

You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.

HbA1c

A blood test of average blood sugar over the past two to three months, given as a percentage or in mmol/mol. 6.5% (48 mmol/mol) or more is the usual threshold for diabetes, normally confirmed by a second test.

NYHA class (heart failure)

The New York Heart Association’s four classes of how much heart failure limits physical activity.

I
No limits: ordinary activity causes no undue tiredness or breathlessness.
II
Slight limits: comfortable at rest, ordinary activity causes symptoms.
III
Marked limits: comfortable at rest, less than ordinary activity causes symptoms.
IV
Symptoms even at rest; any activity makes them worse.

Studies set their own limit — many outside heart failure leave out class III–IV, while heart failure studies may ask for a particular class. Check the class this study names.

Hypersensitivity

An allergy, or another reaction of the immune system, to a substance. “Hypersensitivity to the study drug” means a known allergic reaction to it — not every side effect a medicine can have.

GFR and eGFR (kidney filtering)

GFR (glomerular filtration rate) is how much blood the kidneys filter each minute. It is usually estimated from a blood test — eGFR, by formulas such as CKD-EPI or MDRD — and some studies ask for it to be measured directly instead. Usually given in mL/min/1.73 m².

Check the method, the units and the minimum the study states. A value of 90 or above is in the normal-or-high range, but kidney damage can show in other tests too.

Bilirubin

A yellow substance made when red blood cells are broken down, which the liver clears. A high level can mean a problem with the liver or the bile ducts.

Criteria often allow more for people with Gilbert’s syndrome, a harmless inherited condition that raises bilirubin.

ULN (upper limit of normal)

The top of the normal range for a blood test, as set by the laboratory that runs it. Each lab prints its own range next to your result.

“≤ 2.5 × ULN” means the result can be up to two and a half times that top value.

ALT and AST (liver enzymes)

Two enzymes measured in a routine blood test of the liver. Higher levels can mean the liver is under strain or damaged. SGPT and SGOT are older names for ALT and AST.

Criteria usually give a limit as a multiple of the upper limit of normal (ULN), often higher when cancer has spread to the liver.

Investigational drug or device

A drug or device being studied in a clinical trial.

It may be new, or already approved and being studied for another use, dose or form. Criteria often leave out people who took part in another study of one within a recent period, commonly 30 days or five half-lives.

Live vaccines

Vaccines made with a weakened form of the germ, such as measles–mumps–rubella (MMR), chickenpox and yellow fever. They can be risky when the immune system is weakened. Flu injections are not live; the nasal flu spray is.

05

Study design

Phase
Phase 3
Primary purpose
Treatment
Allocation
Randomized
Intervention model
Parallel assignment
Masking
Quadruple (Participant, Care provider, Investigator, Outcomes assessor)
Enrollment
150 participants (estimated)

Study arms

  • Experimental
    KP-001

    Drug: KP-001 · Drug: Placebo

  • Placebo comparator
    placebo

    Drug: KP-001 · Drug: Placebo

Interventions

  • DrugKP-001

    Oral repeated dose 100mg or lower

  • DrugPlacebo

    Oral repeated dose

06

What researchers measure

Primary outcomes

  1. The ratio of volume of target lesions based on MRI

    To confirm the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) in reducing volume of target lesions at 24 weeks

    Time frame: pre-does and at 24weeks post-dose

Secondary outcomes

  1. Change from baseline in NRS of symptom

    To confirm the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) on disease specific patient-reported outcomes from Week 20 through Week 24

    Time frame: Week 20 through Week 24

  2. The ratio of volume of target lesions based on MRI

    To confirm the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) in reducing volume of target lesions at 12 and 52 weeks

    Time frame: Pre-does and at 12 and 52 weeks post-dose

  3. The proportion of patients defined as a responder at 12, 24, and 52 weeks

    To confirm the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) using response rate based on change in target lesion volume (MRI) at 12, 24, and 52 weeks

    Time frame: pre-does and at 12, 24 and 52 weeks post-dose

  4. Change from baseline of Brief Pain Inventory short form

    To evaluate the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) on patient-reported outcomes at 4, 12, 24, 36, and 52 weeks

    Time frame: pre-does and at 4, 12, 24, 36, and 52 weeks post-dose

  5. Change from baseline of PGI-S (Patient Global Impression of Severity and PGI-I (Patient Global Impression of Improvement)

    To evaluate the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) on patient-reported outcomes at 4, 12, 24, 36, and 52 weeks

    Time frame: pre-does and at 4, 12, 24, 36, and 52 weeks post-dose

  6. Change from baseline of EQ-5D

    To evaluate the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) on patient-reported outcomes at 4, 12, 24, 36, and 52 weeks

    Time frame: pre-does and at 4, 12, 24, 36, and 52 weeks post-dose

  7. Change from baseline of NRS of symptom

    To evaluate the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) on patient-reported outcomes at 4, 12, 24, 36, and 52 weeks

    Time frame: pre-does and at 4, 12, 24, 36, and 52 weeks post-dose

  8. Change from baseline of Wong-Baker Faces of symptom

    To evaluate the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) on patient-reported outcomes at 4, 12, 24, 36, and 52 weeks

    Time frame: pre-does and at 4, 12, 24, 36, and 52 weeks post-dose

  9. Change from baseline of PedsQL Fatigue Scale

    To evaluate the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) on patient-reported outcomes at 4, 12, 24, 36, and 52 weeks

    Time frame: pre-does and at 4, 12, 24, 36, and 52 weeks post-dose

  10. Change from baseline in CGI-S (Clinician Global Impression of Severity) and CGI-I (Clinician Global Impression of Improvemen) at 12, 24, 36, and 52 weeks

    To evaluate the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) in improving clinician-reported outcomes at 12, 24, 36, and 52 weeks

    Time frame: pre-does and at 12, 24, 36, and 52 weeks post-dose

  11. The proportion of patients achieving both ≥20% reduction in target lesion volume AND ≥1 score reduction in NRS of symptom

    To confirm the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) using response rate based on change in target lesion volume (MRI) AND disease specific patient-reported outcomes at 12, 24, and 52 weeks

    Time frame: pre-does and at 12, 24 and 52 weeks post-dose

  12. Change from baseline in non-target lesions by MRI

    To evaluate the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) in changing non-target lesion at 12, 24, and 52 weeks

    Time frame: pre-does and at 12, 24 and 52 weeks post-dose

  13. Presence of new lesions based on MRI

    To evaluate the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) in suppressing presence of new lesions at 12, 24, and 52 weeks

    Time frame: pre-does and at 12, 24 and 52 weeks post-dose

  14. Time to relapse

    To evaluate the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) at 12, 24, and 52 weeks regarding time to relapse

    Time frame: pre-does and at 12, 24 and 52 weeks post-dose

  15. Time to disease progression

    To evaluate the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) at 12, 24, and 52 weeks regarding time to disease progression

    Time frame: pre-does and at 12, 24 and 52 weeks post-dose

  16. Additional treatment for pain related to vascular malformation

    To evaluate the efficacy of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) for up to 24 weeks regarding concomitant medication for vascular malformation

    Time frame: Up to 24 weeks post-dose

  17. Plasma KP-001 concentration data obtained from sparse sampling for population PK analysis

    To evaluate the pharmacokinetics of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) following multiple oral doses

    Time frame: 8weeks, 16weeks, 20weeks and 32 weeks post-dose

  18. Adverse events

    To determine the safety of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) for up to 52 weeks

    Time frame: up to 52 weeks post-dose

  19. Clinical events relevant to the vascular malformations (bleeding, infection, thromboembolism, and others)

    To determine the safety of KP-001 100 mg or lower based on body weight administered once daily in patients with vascular malformations (common VM, common LM, or KTS/CLOVES syndrome) for up to 52 weeks

    Time frame: up to 52 weeks post-dose

Other outcomes

  1. Change in D-dimer From Baseline to Week 24

    Serum D-dimer will be measured as a coagulation biomarker of disease activity in patients with vascular malformations. The change from baseline to Week 24 will be evaluated to explore its clinical relevance.

    Time frame: up to 52 weeks post-dose

07

Study locations

5 of 5 sites recruiting
  • MUSC Children's Health Primary Care
    Charleston, South Carolina 29492, United States
    Recruiting
    • Kennedy Mcleod · Contactmcleodke@musc.edu · 843-792-4091
    • Lara Wine Lee · Principal investigator
  • Kyungpook National University Hospital - Pediatric
    Daegu, Daegu 41944, South Korea
    Recruiting
    • Jeon Jiyoung · Contactjyjeon@knuh.kr · +82-10-9251-6355
    • Ji Yoon Kim · Principal investigator
  • Seoul National University Hospital
    Seoul, Seoul 03080, South Korea
    Recruiting
    • EunYoung Kim · Contact49007@snuh.org · +82-2-2072-0505
    • Jung Min Ko · Principal investigator
  • Asan Medical Center
    Seoul, Seoul 05505, South Korea
    Recruiting
  • National Taiwan University Hospital - HsinChu Branch
    Hsinchu, Hsinchu 300, Taiwan
    Recruiting
    • Wei-Tse Hsu · Contactweitse0123@gmail.com · +886-963-911-971
    • Meng-Ju Li · Principal investigator
08

Updates

Tracking since Sep 25, 2026
No changes since tracking began. The registry record was last updated on Sep 21, 2026, before this site started recording changes on Sep 25, 2026. Its history is on ClinicalTrials.gov ↗
09

Registry details

Key details

Study ID
NCT07285005
Lead sponsor
Kaken Pharmaceutical
Responsible party
Sponsor
First posted
Dec 16, 2025
Start date
May 13, 2026
Primary completion
Feb 2028 (estimated)
Completion
Feb 2028 (estimated)
Last update
Sep 21, 2026

Study contacts

Sr. Clinical Trial Manager, Clinical Operations
Contact
S-KY@aadibio.com
+818059831020

Oversight

Data monitoring committee
Yes
FDA-regulated drug
Yes
FDA-regulated device
No
View the source record on ClinicalTrials.gov ↗

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