Clinical Trials Logo

Clinical Trial Details — Status: Completed

Administrative data

NCT number NCT02744027
Other study ID # 15-012331
Secondary ID
Status Completed
Phase N/A
First received April 11, 2016
Last updated March 19, 2018
Start date April 2016
Est. completion date December 2017

Study information

Verified date March 2018
Source Children's Hospital of Philadelphia
Contact n/a
Is FDA regulated No
Health authority
Study type Interventional

Clinical Trial Summary

Lymphatic Anomalies (LA) is characterized by proliferation of lymphatic tissue causing deterioration of pulmonary function. Understanding changes in lymphatic anatomy in these patients is hindered by the difficulty of imaging the lymphatic system. Dynamic Contrast Enhanced MR Lymphangiogram (DCMRL) may be useful in investigating pathological changes in the lymphatic system.


Description:

Lymphatic anomalies (LA) are a spectrum of rare diseases classified into lymphatic tumors and malformations. Complicated LA cases in the past have been called lymphangiomatosis because of varied disseminated involvement including soft tissue and viscera such as the spleen, liver and bone. Pleural effusions and pericardial effusions are often associated with these lesions. These are now classified into different phenotypes such as Generalized Lymphatic Anomaly (GLA), Gorham's Stout Disease (GSD) and Kaposiform Lymphangiomatosis (KLA). These complicated phenotypes can cause massive osteolysis causing a morbidity and mortality from infection or paralysis or worsening pulmonary function and effusions (GSD, GLA, KLA).

The major cause of mortality and morbidity in these patients is the deterioration of pulmonary function by chronic chylous effusions and progressive interstitial lung disease. Unfortunately, little is known about biomarkers, risk stratification or the pathophysiology of this progression. The understanding of changes in patients' lymphatic anatomy with LA is hindered by the difficulty of imaging the lymphatic system. Dynamic Contrast Enhanced MR Lymphangiogram (DCMRL) is a technique that has recently been developed, allowing dynamic MR imaging of the lymphatic system by injecting gadolinium contrast agent in the groin lymph nodes.

This technique has been previously used to identify pathological lymphatic perfusion of the lung parenchyma in patients with plastic bronchitis and neonatal chylothorax. Based on these imaging findings, a treatment algorithm has been designed and used to successfully treat the majority of those patients with these conditions.


Recruitment information / eligibility

Status Completed
Enrollment 10
Est. completion date December 2017
Est. primary completion date December 2017
Accepts healthy volunteers No
Gender All
Age group 2 Years to 80 Years
Eligibility Inclusion Criteria:

- Subjects diagnosed with Lymphatic Anomalies (GLA, KL, Gorham disease) with radiological and or pathological confirmation of lung involvement will be considered for the study.

- Girls > 11 y.o. with a negative urine pregnancy test and contraception use.

- Registration in the International Lymphangiomatosis and Gorham's Disease Alliance (LGDA) Patient Registry or referral by a physician.

Exclusion Criteria:

- Subjects with contraindications to contrast enhanced MRI: allergy to gadolinium imaging agents, impaired renal function (GFR<30) and presence of paramagnetic objects.

- Claustrophobia or contraindications to sedation/anesthesia

- Uncorrectable coagulopathy (bleeding disorders).

- Pregnant or lactating females.

Study Design


Intervention

Other:
Dynamic Contrast Enhanced Magnetic Resonance Lymphangiography (DCMRL)
Following completion of the T2 lymphatic mapping, contrast agent administered will be calculated based on weight. Investigators will use dosage of 0.1cc/kg of contrast which will be injected by hand simultaneously into each lymphatic node (LN) at a rate of 0.5-1 cc per min. One minute after the injection, scanning will be initiated using high spatial and temporal resolution magnetic resonance angiography (syngo time-resolved angiography with stochastic trajectories [TWIST]) sequence. The sequence parameters will be adjusted with a time delay such that a complete volume will be acquired approximately every 20-60 seconds. This will be followed by additional scans with a high-resolution navigator gated 3-dimensional flash inversion recovery (IR) sequence. In all subjects the scan area covered the neck, chest, and abdomen as caudal as feasible.
Heavy Weighted T2 Imaging (T2W)
Magnetic Resonance imaging (MRI) will be performed and T2W MRI lymphatic mapping will be performed using a respiratory navigated and cardiac gated 3 dimensional turbo spin echo sequence with pre-set parameters. Scan time varies from 2-5 min depending upon the size of the subject.
Lymph Node Access
The procedure will be performed under sedation and anesthesia if participants are aged 16 or under. The procedure will be performed with local anesthesia for participants aged over 16 years old. Using ultrasound guidance two inguinal lymph nodes (one each side) will be directly accessed with 25-gauge spinal needles attached to a short connector tubing. A shallow angle for puncture will be used to create a long subcutaneous tract to assist in stabilizing the needle. A small amount of an oil-based contrast or water soluble contrast will be injected under fluoroscopy guidance to confirm the correct position of the needles inside the lymph nodes. After stabilizing the needle, the subjects will be transferred into the MRI suite.

Locations

Country Name City State
United States Children's Hospital of Philadelphia Philadelphia Pennsylvania

Sponsors (2)

Lead Sponsor Collaborator
Children's Hospital of Philadelphia University of Pennsylvania

Country where clinical trial is conducted

United States, 

Outcome

Type Measure Description Time frame Safety issue
Primary Describe lymphatic anatomy of participants with lymphatic anomalies (LA) The primary endpoint will be the description of lymphatic anatomy assessing if participants have a single or branched Thoracic Duct. 2 Days
Secondary Development of a classification system for lymphatic flow Development of a classification system based on retrograde or anterograde flow of lymphatic fluid. 2 days
Secondary Description of Lymphatic malformations in participants with lymphatic anomalies Assessing if lymphatic malformation are present or absent in participants with lymphatic anomalies. 2 days
See also
  Status Clinical Trial Phase
Completed NCT02497131 - Study on the Role of Brentuximab Vedotin as Single Agent in the Treatment of Relapsed/Refractory CD30+ PTCL Patients Phase 2
Recruiting NCT01451502 - Infusion of Cell Populations From Unlicensed Umbilical Cord Blood Units N/A
Active, not recruiting NCT03264131 - BV-CHEP Chemotherapy for Adult T-cell Leukemia or Lymphoma Phase 2
Completed NCT03744676 - A Safety Trial of Lisocabtagene Maraleucel (JCAR017) for Relapsed and Refractory (R/R) B-cell Non-Hodgkin Lymphoma (NHL) in the Outpatient Setting (TRANSCEND-OUTREACH-007) Phase 2
Completed NCT02335242 - Sildenafil for the Treatment of Lymphatic Malformations Phase 2
Completed NCT03135535 - Micro-mobile Foot Compression and Diabetic Foot Phase 2
Active, not recruiting NCT05441943 - Lymphaticovenous Anastomosis as Treatment for Lymphedema N/A
Recruiting NCT06267807 - Lymphatic Phenotype in Noonan Syndrome Spectrum Disorders N/A
Withdrawn NCT05431179 - A Study of Zilovertamab and Ibrutinib in Patients With Relapsed or Refractory Mantle Cell Lymphoma Phase 3
Recruiting NCT05731141 - A Prospective Natural History Study of Lymphatic Anomalies
Recruiting NCT03696784 - Anti-CD19 CAR-T Cells With Inducible Caspase 9 Safety Switch for B-cell Lymphoma Phase 1
Completed NCT03709407 - "Effects of Manual Lymphatic Drainage in the Neck in Healthy Subjects. Experimental Randomized Crossover Study" N/A
Active, not recruiting NCT02663297 - Administration of T Lymphocytes for Prevention of Relapse of Lymphomas Phase 1
Completed NCT05683444 - Octreotide Improves Human Lymphatic Fluid Transport a Translational Trial Early Phase 1
Completed NCT02960841 - Effectiveness of Intracavitary Manual Lymphatic Drainage Phase 4
Recruiting NCT05263583 - Sepantronium Bromide for the Treatment of High-grade B-cell Lymphoma Phase 2
Terminated NCT02499627 - Bendamustine Plus Brentuximab Vedotin in HL and CD30+ PTCL in First Salvage Setting Phase 2
Recruiting NCT03373019 - Chidamide Combined With R-GDP in Treating Patients With Relapsed or Refractory Diffuse Large B-cell Lymphoma (DLBCL) Phase 2
Recruiting NCT03602157 - Study of CAR-T Cells Expressing CD30 and CCR4 for r/r CD30+ HL and CTCL Phase 1
Recruiting NCT02690545 - Study of CD30 CAR for Relapsed/Refractory CD30+ HL and CD30+ NHL Phase 1/Phase 2