Lymphomatoid granulomatosis (LYG) is a rare angiocentric and angiodestructive EBV-associated B-cell lymphoproliferative disorder. It is hypothesized that. Pulmonary lymphomatoid granulomatosis (PLG) is an uncommon pulmonary disorder characterized by multiple pulmonary nodular lesions with. Lymphomatoid granulomatosis (LYG or LG) is a very rare lymphoproliferative disorder first characterized in Lymphomatoid means lymphoma-like and.
|Published (Last):||1 June 2017|
|PDF File Size:||13.2 Mb|
|ePub File Size:||3.62 Mb|
|Price:||Free* [*Free Regsitration Required]|
Lymphomatoid granulomatosis is a rare disorder characterized by overproduction proliferation of white blood cells called lymphocytes lymphoproliferative disorder.
The abnormal cells infiltrate and accumulate form lesions or nodules within tissues. The lesions or nodules damage lykphomatoid destroy the blood vessels within these tissues. The lungs are most commonly affected in lymphomatoid granulomatosis. Symptoms often include cough, shortness of breath dyspnea and lyphomatoid tightness. Other areas of the body such as the skin, kidneys or central nervous system are also frequently affected.
The abnormal cells in lymphomatoid granulomatosis are B-cells B lymphocytes containing the Epstein-Barr virus. There are two main types of lymphocytes: B-lymphocytes, which may produce specific antibodies to “neutralize” certain invading microorganisms, and T-lymphocytes, which may directly destroy microorganisms or assist in the activities of other lymphocytes.
Because lymphomatoid granulomatosis is caused by the overproduction of abnormal B-cells, affected individuals may eventually develop B-cell lymphoma, a form of non-Hodgkin lymphoma. Lymphoma is a general term for cancer of the lymphatic system. The symptoms and progression of lymphomatoid granulomatosis vary greatly from person to person. The disorder may occasionally resolve without treatment spontaneous remission in some affected individuals or more commonly it will progress and cause life-threatening complications.
Occasionally, it is an incidental and asymptomatic finding lymphpmatoid chest radiographs less than 5 percent. In almost all affected individuals, lymphomatoid granulomatosis affects the lungs and often causes a cough, shortness of breath dyspnea and chest tightness.
In most patients, the cough is non-productive and is rarely associated with blood hemoptysis. Systemic symptoms are not uncommon and may include fever, a general feeling of poor health malaiseweight loss, and fatigue.
Approximately percent of affect individuals will develop skin lesions such as a patchy reddish erythematous rash consisting of flat discolored lesions maculessmall, elevated bumps papules or, more rarely, solid, raised, flat-topped lesions plaques.
Small bumps or growths nodules just below the surface of the skin subcutaneous may also develop. Larger nodules may become open sores ulcerated. In approximately 30 percent of patients, the central nervous may be involved potentially resulting in mental status changes, headaches, seizures, paralysis of one side of the body hemiparesisor loss of the ability to coordinate voluntary movements ataxia.
Less commonly, the kidneys or liver may be involved, although this rarely leads to the development of symptoms. In some cases the liver may become enlarged hepatomegaly. In some extremely rare cases, lymphomatoid granulomatosis may only affect the skin or only the central nervous system isolated lymphomatoid granulomatosis. The exact cause of lymphomatoid granulomatosis is unknown. Lymphomatoid granulomatosis occurs with greater frequency in individuals with some form of immune system dysfunction including individuals with Sjogren syndrome, rheumatoid arthritis or chronic viral hepatitis.
It is likely that some combination of immunodeficiency, genetic and familial factors all play a role in the development of lymphomatoid granulomatosis. The therapy used varies, but is generally directed against eliminating the EBV-infected B-cells or boosting the immune system.
The classification of lymphomatoid granulomatosis has been difficult. Originally, the disorder was viewed as a benign process with the potential to progress to malignant lymphoma.
Researchers believed that defective cells were T-cells. Scientific advances in technology have allowed researchers to determine that the abnormal cells in lymphomatoid granulomatosis are B-cells infected by the Epstein-Barr virus. However, most of the cells within the tissues are T-cells, reacting against the abnormal T-cells. The Epstein-Barr virus is common among the general population and is relatively well-known because it is the cause of infectious mononucleosis IMusually with no long-lasting effects.
Lymphomatoid granulomatosis affects males twice as often as females. It is most common in adults after the fifth decade, but can occur at any age and has been reported in children. The prevalence of lymphomatoid granulomatosis is unknown. Churg-Strauss syndrome is a rare disorder that may affect multiple organ systems, especially the lungs. The disorder is characterized by the abnormal clustering of certain white blood cells hypereosinophilia in the blood and tissues, inflammation of blood vessels vasculitisand the development of inflammatory nodular lesions called granulomas granulomatosis.
Most affected individuals have a history of allergy. In addition, asthma and other associated lung pulmonary abnormalities i.
Nonspecific findings associated with Churg-Strauss syndrome typically include flu-like symptoms, such as fever, a general feeling of weakness and fatigue malaiseloss of appetite anorexiaweight loss, and muscle pain myalgia. Additional symptoms and findings may vary depending upon the specific organ systems involved. The nerves outside the central nervous system peripheral nerveskidneys, or gastrointestinal tract are often involved.
Without appropriate treatment, serious organ damage and potentially life-threatening complications may result. Although the exact cause of Churg-Strauss syndrome is unknown, many researchers indicate that abnormal functioning of the immune system plays an important role.
Symptoms may include ulcerations of the mucous membranes in the nose with secondary bacterial infection, a persistent runny nose, sinus pain, and chronic middle ear infection otitis media potentially resulting in hearing loss. In some cases, kidney abnormalities may progress to kidney failure, a serious complication. If the granulomatois are affected, a cough, expectoration of blood hemoptysisand inflammation of the thin membrane lining the outside of the lungs and the inside of the lung lynphomatoid be present.
Lymphomatoid Granulomatosis – NORD (National Organization for Rare Disorders)
Lymphoma is a general term for other cancers of the lymphatic system. Lymphomas result from errors in the production of a white blood cell lymphocyte or transformation of a lymphocyte into a malignant cell. Abnormal, uncontrolled growth and multiplication proliferation of malignant lymphocytes may lead to enlargement of a specific lymph node region or regions; involvement of other lymphatic tissues, such as the spleen and bone marrow; and spread to other bodily tissues and organs, potentially resulting in life-threatening complications.
The specific symptoms and physical findings may vary from patient to patient, depending upon the extent and region s of involvement and other factors. Sarcoidosis is a multisystem disorder that most often affects individuals between 20 and 40 years of age. Females appear to be affected more frequently than males.
Sarcoidosis is characterized by the abnormal formation of inflammatory masses or nodules granulomas consisting of certain granular white blood cells modified macrophages or epithelioid cells in certain organs of the body. The granulomas that are formed are thought to grsnulomatosis the normal structure of and, potentially, the normal functions of, the affected organ scausing symptoms associated with the particular body system s in question.
In individuals with sarcoidosis, such granuloma formation most commonly affects the lungs. The granupomatosis and severity of symptoms associated with sarcoidosis vary greatly, depending upon the specific organ s involved and the degree of such involvement. The exact cause of sarcoidosis is not known. However, possible infectious, environmental, genetic, and immunological factors are under investigation as potential causes of the disorder.
A diagnosis of lymphomatoid granulomatosis is made based upon a detailed patient history, a thorough clinical evaluation, and a variety of specialized tests such as the surgical removal and microscopic examination biopsy of tissue samples taken from an affect organ such as the lungs.
A skin biopsy is not reliable because the characteristic abnormal cells may be missing.
Certain x-ray studies e. A CT scan of the lungs or another affected organ can help determine the extent of lymphomatoid granulomatosis. Magnetic resonance imaging MRI of the brain and lumbar puncture LP should be performed to rule out involvement of the central nervous system. The most effective therapy for individuals with lymphomatoid granulomatosis is unknown. For individuals with minimal disease, observation may be recommended since long-term survival without treatment has occurred as well as spontaneous remission.
In most cases, however, treatment is recommended. Treatment recommendations are based on the grade of disease. In patients with grade III disease, interferon alfa-2b is not effective, and combination chemothearpy with rituximab should be used.
Corticosteroids alone are only recommended as a temporizing measure and should not be used for long term control of lymphomatoid granulomatosis. Similarly, rituximab alone is seldom effective for long term control. Neither of these agents effectively eradicates and abnormal EBV clones and corticosteroids can increase immusuppression and ultimately disease progression. If patients develop lymphomatoid granulomatosis on immune suppressive agents, they should be discontinued if at all possible since this may induce clinical remission.
If the disease is progressive or advanced, then treatment as outlined above should be instituted. Rarely patients with CNS involvement require brain radiation. To participate, patients must be diagnosed with LYG, 12 or older, and not pregnant or nursing.
For information, contact Dr. Wilson, the principal investigator, atwilsonw mail. Information on current clinical trials is posted on the Internet at www. All studies receiving U. Government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials sponsored by private sources, contact: For more information about clinical trials conducted in Europe, contact: A case of lymphomatoid granulomatosis masquerading as a lung abscess.
Guven A, Baskin D.
Lymphomatoid granulomatosis – Wikipedia
Lymphomatoid granulomatosis in a boy with long-term follow-up. Lymphomatoid granulomatosis after childhood acute lymphoblastic leukemia: Am J Surg Pathol. Radiotherapy to control CNS lymphomatoid granulomatosis: Association of lymphomatoid granulomatosis with Epstein-Barr viral lymphomatoi of B lymphocytes and response to interferon-alpha 2b. Accessed April 21, The content of the website and databases of the National Organization for Rare Disorders NORD is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD.
About News Events Contact.
Rare Disease Database
Synonyms of Lymphomatoid Granulomatosis benign lymph angiitis and granulomatosis malignant lymph angiitis and lyjphomatoid pulmonary angiitis. General Discussion Lymphomatoid granulomatosis is a rare disorder characterized by overproduction proliferation of white blood cells called lymphocytes lymphoproliferative disorder.
Lymphomatoid granulomatosis may eventually progress to a form of large B-cell lymphoma. Causes The exact cause of lymphomatoid granulomatosis is unknown.