National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Lymphomatoid granulomatosis


Lymphomatoid granulomatosis is a rare disorder characterized by an overproduction of white blood cells known as B lymphocytes. These B cells can build up in the tissues of the body, causing damage to the blood vessels.[1] In many cases of lymphomatoid granulomatosis, the abnormal B cells contain the Epstein-Barr virus. The disease is more common in men, usually after the fifth decade of life.[1][2] Lymphomatoid granulomatosis most commonly affects the lungs, though other areas of the body may also be affected. Signs and symptoms vary but can include cough, shortness of breath, tightness of the chest, fever, weight loss, and fatigue. Skin lesions and central nervous system changes such as headaches, seizures, and ataxia may also be seen. Rarely, the disorder can affect the kidneys or liver. The cause of the disorder is not well understood, though a combination of genetic and immune factors are thought to play a part. Treatment depends on the extent of the disease but may include interferon alfa-2b and combination chemotherapy with rituximab. Occasionally, the disorder resolves on its own without treatment.[1] There has been some debate as to whether lymphomatoid granulomatosis should be viewed as a as a B-cell lymphoma or a lymphoproliferative disease or whether it should be viewed merely as a condition that can develop into a B-cell lymphoma. The prognosis is variable, though lymphomatoid granulomatosis can progress and become fatal in some cases.[2] 
Last updated: 1/4/2017

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Lymphomatoid granulomatosis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Financial Resources

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.

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  1. Jaffe, Elaine and Wilson, Wyndham. Lymphomatoid Granulomatosis. National Organization for Rare Disorders. 2016; https://rarediseases.org/rare-diseases/lymphomatoid-granulomatosis/.
  2. Kamangar, Nader. Lymphomatoid Granulomatosis. Medscape. December 31, 2015; http://emedicine.medscape.com/article/299751-overview.