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.

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CVID or receiving prolonged immunosuppressive regimens related to underlying autoimmune disease e.

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.

Grading was performed predominantly on the lung biopsy at diagnosis; they were distributed as follows: We did not regularly see treatment effect in the biopsies, but there were two patients Patients 45, 48, Table 3 with a diagnosis of LYG grade 3 who were previously treated at their respective outside institution with chemotherapy i. The granulomas that are formed are thought to alter the normal structure of and, potentially, the normal functions of, the affected organ scausing symptoms associated with the particular body system s in question.

All studies receiving U. Most affected individuals have a history of allergy. 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.

B Focally there is coagulative-type necrosis.

Lymphomatoid granulomatosis.

A Problem for Gene Editing in Cancer? Grade 2 and 3 lesions usually did not pose a problem in grading because the LACs were more prominent and EBV was detectable in all cases see Table 3.

Causes The exact cause of lymphomatoid granulomatosis is unknown. Granulomatosiis, 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. Epstein-Barr virus RNA is found in monoclonal occasionally oligoclonal proliferations of B lymphocytes, whereas the T lymphocytes are polyclonal.

Neither of these agents effectively eradicates and abnormal EBV clones and corticosteroids can increase immusuppression and ultimately disease progression. 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.


Cutaneous lymphoid hyperplasia with bandlike and perivascular patterns with nodular pattern Jessner lymphocytic infiltrate of the skin.

A Lung biopsy from a 23 year-old male that was previously treated with CHOP showing B dense fibrosis around coagulative necrosis. Diffuse large B-cell lymphoma Intravascular large B-cell lymphoma Primary cutaneous marginal zone lymphoma Primary cutaneous immunocytoma Plasmacytoma Plasmacytosis Primary cutaneous follicle center lymphoma.

Since the areas of necrosis are always negative by in situ hybridization for EBER, this can greatly underestimate the number of EBER positive cells especially when the necrosis is extensive, as in high grade lesions. In most cases, however, treatment is recommended. The initial grading of LYG was based on cellular composition, cytologic atypia, and necrosis 7 and later based on the density and number of EBV positive large atypical B cells.

Definitive diagnosis of LYG, typically, requires either an open wedge lung biopsy or a thoracoscopic video-directed biopsy of affected lung tissue.

Lymphomatoid Granulomatosis (LYG)

Lymphomatoid granulomatosis LYG or LG is a very rare lymphoproliferative disorder first characterized in Patients with hemoptysis are more likely to have cavitary nodules. Organs usually affected are the skin, lungs, central nervous system while liver and kidney are affected to lesser extent.

Other EBV-associated lymphoproliferative disorders may present with cutaneous disease, and should be considered in the differential diagnosis 28 If the clinical picture is consistent with LYG multiple lung lesions, sparing of the lymph nodes, spleen, bone marrow, and low peripheral blood viral load and the lesions have morphologic and immunophenotypic features consistent with LYG, we feel that a diagnosis of LYG can be made in the post transplant setting.

Radiotherapy to control CNS lymphomatoid granulomatosis: However, most of the cells within the tissues are T-cells, reacting against the abnormal T-cells. Neutrophils, eosinophils, multinucleated giant cells, and well-formed granulomas were not seen.


In individuals with sarcoidosis, such granuloma formation most commonly affects the lungs. Lower-grade LYG occasionally undergoes spontaneous remission and is best managed with strategies designed to enhance the host’s underlying immune system, whereas high-grade LYG is best managed by combination chemoimmunotherapy but has inferior outcomes.

Please review our privacy policy. Leukemia and Lymphoma, August ; 47 8: Lymphomatoid granulomatosis after childhood acute lymphoblastic leukemia: This phenomenon is believed to be the result of the expansion of a few dominant T-cell clones reacting to EBV viral epitopes Treatment recommendations are based on the grade of disease.

The exact cause of lymphomatoid granulomatosis is unknown. The lungs are generally involved with the skin and central nervous system also commonly affected. For grading, the number of LACs and EBER positive cells should be used, and while necrosis coagulative-type is more extensive in higher grades, it can also be seen in grade 1 lesions.

Synonyms of Lymphomatoid Granulomatosis benign lymph angiitis and granulomatosis malignant lymph angiitis and granulomatosis pulmonary angiitis.

Rare Disease Database

However, one must be aware of the impact of necrosis in assessing the number of EBER positive cells. Accessed April 21, Lymphoid-related cutaneous conditions Epstein—Barr virus-associated diseases. DermNet NZ does not provide an online consultation service. lyjphomatoid

Originally, the disorder was viewed as a benign process with the potential to progress to malignant lymphoma. The specific symptoms and physical findings may vary from patient to patient, depending upon the extent and region s of involvement and other factors.

EBV positive mucocutaneous ulcer–a study of 26 cases associated with various sources of immunosuppression. In a recent review by Katzenstein et al. The role of Mig, the monokine induced lymlhomatoid interferon-gamma, and IP, the interferon-gamma-inducible protein, in tissue necrosis and vascular damage associated with Epstein-Barr virus-positive lymphoproliferative disease.