Interferon for Lymphomatoid Granulomatosis

Phase-Based Progress Estimates
1
Effectiveness
2
Safety
National Institutes of Health Clinical Center, 9000 Rockville Pike, Bethesda, MD
Lymphomatoid Granulomatosis+3 More
Interferon - Biological
Eligibility
Any Age
All Sexes
Eligible conditions
Select

Study Summary

Treatment and Natural History Study of Lymphomatoid Granulomatosis

See full description

Eligible Conditions

  • Lymphomatoid Granulomatosis
  • Non-Hodgkin's Lymphoma (NHL)
  • Disorders, Lymphoproliferative

Treatment Effectiveness

Effectiveness Estimate

1 of 3

Study Objectives

This trial is evaluating whether Interferon will improve 1 primary outcome and 1 secondary outcome in patients with Lymphomatoid Granulomatosis. Measurement will happen over the course of After completion of treatment.

After completion of treatment
Overall response and long-term efficacy
Year 1
Overall Survival

Trial Safety

Safety Estimate

2 of 3
This is better than 68% of similar trials

Trial Design

2 Treatment Groups

2
1 of 2
1
1 of 2
Experimental Treatment

This trial requires 105 total participants across 2 different treatment groups

This trial involves 2 different treatments. Interferon is the primary treatment being studied. Participants will be divided into 2 treatment groups. There is no placebo group. The treatments being tested are in Phase 2 and have already been tested with other people.

2
Drug
EPOCH-R every 3 weeks for up to 6 cycles, based on response.
1
Biological
Interferon starting at 7.5 million Units subQ 3 times a week and increasing on the designated schedule, as tolerated. Patients continue taking interferon for 1 year beyond CR. Patients who progress may crossover to receive EPOCH-R.
Treatment
First Studied
Drug Approval Stage
How many patients have taken this drug
Interferon
1998
Completed Phase 3
~690

Trial Logistics

Trial Timeline

Approximate Timeline
Screening: ~3 weeks
Treatment: Varies
Reporting: post treatment: every 3 months x 1 year, then every 4 months x 1 year, then every 6 months x 1 year then yearly
This trial has the following approximate timeline: 3 weeks for initial screening, variable treatment timelines, and roughly post treatment: every 3 months x 1 year, then every 4 months x 1 year, then every 6 months x 1 year then yearly for reporting.

Closest Location

National Institutes of Health Clinical Center, 9000 Rockville Pike - Bethesda, MD

Eligibility Criteria

This trial is for patients born any sex of any age. There are 4 eligibility criteria to participate in this trial as listed below.

Mark “yes” if the following statements are true for you:
Patients who are at least 12 years old will be able to participate in the study. show original
The patients must have a tissue-diagnosis of grade 1, 2 and/or 3 Lymph node granuloma (or a diagnosis consistent with Lymph node granuloma) confirmed by the Laboratory of Pathology, NCI show original
Patients with any stage of cancer will be eligible. show original
Previously untreated patients and patients who have been treated are both eligible. show original

Patient Q&A Section

What causes lymphomatoid granulomatosis?

"Most cases of LGL are attributable to the Epstein-Barr virus, although it has been associated with the virus in only 50-70% of cases. The disease appears in young women, most often in those who reside in tropical regions, in whom the Epstein-Barr-associated virus occurs more frequently than in Caucasian populations. The Epstein-Barr virus is a latent helper virus which is difficult to isolate, making it difficult to study the precise role of the virus in causing the lymphoid neoplasm. A number of other viruses including the herpes simplex viruses, cytomegalovirus, human papilloma virus, human T-lymphotropic virus, and HHV-7 seem to contribute." - Anonymous Online Contributor

Unverified Answer

What are common treatments for lymphomatoid granulomatosis?

"The current treatment of lymphomatoid granulomatosis is very variable, based solely on past and current experience with this rare peripheral systemic disease. There is no evidence that a single drug regimen, such as high-dose steroids, is the best treatment. These cases often require the removal of internal lymph nodes, and in nearly half of instances the procedure is unsuccessful and requires the creation of an alternative fistula. The use of immunosuppressant agents such as ciclosporin at such times is questionable because of potential toxicity but anecdotal evidence suggests that the agents may help." - Anonymous Online Contributor

Unverified Answer

Can lymphomatoid granulomatosis be cured?

"Sclerosing periorbital dermatitis (SOD) and subcutaneous osteoma may not be true manifestations of LG, but may simply be complications of an underlying disease. In patients with cutaneous SOD, the diagnosis should be reconsidered. The presence of an underlying LG must always be considered when cutaneous SOD occurs. Although the symptoms of LG are almost invariably refractory to conventional therapies, it may be curable in some patients." - Anonymous Online Contributor

Unverified Answer

What is lymphomatoid granulomatosis?

"Although the name implies a disease that invades the kidney, liver or skin, LG is mainly a form of sarcoidosis that can affect both the kidneys and liver, and is characterized by granuloma and nodules in various organs. The kidney is the most commonly affected organ." - Anonymous Online Contributor

Unverified Answer

What are the signs of lymphomatoid granulomatosis?

"This case may be the earliest demonstration of LGB, representing a spectrum of presentations. The clinical manifestations are diverse and may be nonspecific. The clinician should be sensitive to these manifestations and suspect LGB at presentation." - Anonymous Online Contributor

Unverified Answer

How many people get lymphomatoid granulomatosis a year in the United States?

"Onset of LGLP in the U.S. is generally earlier in children and young adults than in other countries. U.S. diagnosis is higher and more sporadic than other countries; in some regions, LGLP is a more important and common disorder than its European counterparts." - Anonymous Online Contributor

Unverified Answer

Is interferon typically used in combination with any other treatments?

"The use of Interferon in this study was not associated with a reduction in treatment failure. The use of Interferon and steroids in patients with LMCA may be justified as part a multidisciplinary treatment strategy. The number of patients with a response to therapy was low." - Anonymous Online Contributor

Unverified Answer

How serious can lymphomatoid granulomatosis be?

"Patients with LG usually present to their provider with chronic cough and bronchoalveolar lavage and CT findings consistent with LGS such as multifocal bronchiectasis, pulmonary cysts, and diffuse ground-glass opacities. Although LGS can be asymptomatic, the average survivals in reported patients with LGS are <10 years, so this disease should be considered in patients with unexplained pulmonary infiltrates on imaging." - Anonymous Online Contributor

Unverified Answer

Who should consider clinical trials for lymphomatoid granulomatosis?

"There would be an improvement in the therapy of LG with more than 98% of a randomized, placebo-controlled, multicenter trial. Physicians should consider clinical trials in those with an early-onset of disease after age 40 years old." - Anonymous Online Contributor

Unverified Answer

What are the latest developments in interferon for therapeutic use?

"A recent development in gene therapy for the treatment of chronic myeloid leukemias involves the use of the chimeric oncogene zinc finger-NF-κB-IL-6 transgene (zFN-IL-6-Z) in a clinical trial by Novartis. The transgene is engineered with zinc finger proteins capable of binding to a promoter containing transcription factor consensus sites. This zinc finger-NF-κB-IL-6 transgene is being explored as a novel gene therapy for CML with two distinct zinc finger nls fused to the NF-κB-IL-6 zinc finger domain. ZFN-IL-6 is now undergoing clinical trials." - Anonymous Online Contributor

Unverified Answer

Does interferon improve quality of life for those with lymphomatoid granulomatosis?

"Interferon may be the treatment of choice if the disease involves only one or two organs, because it improves quality of life in both the short and long term." - Anonymous Online Contributor

Unverified Answer

What are the chances of developing lymphomatoid granulomatosis?

"It has been suggested that these two conditions may be closely related or even synonymous, as both can be found in the same patients. LGL and/or LGL-B resemble diffuse B-cell lymphomas. Given the fact that systemic rituximab therapy appears to be effective in LGL and LGL-B, it is likely that these lesions are autoimmune diseases of B-cell origin with some clonal B cell involvement. Data from a recent study support this hypothesis given the presence of high levels of clonal B cells in peripheral lymphoid tissues, high immunoglobulin levels and the absence of tumor markers by immunohistochemistry." - Anonymous Online Contributor

Unverified Answer
Please Note: These questions and answers are submitted by anonymous patients, and have not been verified by our internal team.
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