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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Lymphomatoid granulomatosis is a rare form of B-cell lymphoma a cancer of lymph tissue.
It is characterised by lymphocytes around blood vesselsi. By World Health Organization definition lymphomatoid granulomatosis is defined as an angiocentric and angiodestructive lymphoproliferative disease involving extranodal sites, composed of B cells positive for Epstein-Barr virus EBV and admixed with reactive T cells. It also often occurs in association with an underlying immunodeficiency state. It is a form of non-Hodkin’s lymphoma.
Lymphomatoid granulomatosis is twice as common in males as in females, and occurs between 30 and 50 years of age. The lungs are generally involved with the skin and central nervous system also commonly affected. Other organs can be involved including kidney, liver, spleen, lymph nodes, eyes lymphomatodi the gastrointestinal tract.
The most common presenting symptoms are cough, fever and skin lesions. Affected patients granuoomatosis feel generally unwell, with weight loss, neurological abnormalities, breathlessness and chest pain. There are usually no symptoms from the skin lesions, but granulomahosis can be tender or itchy.
Neurological examination may reveal cranial and peripheral nerve defects resulting in unsteadiness, blurred vision, weakness or numbness affecting facial muscles or hands and feet. Tissue biopsy is needed to confirm a diagnosis of lymphomatoid granulomatosis.
The results of other laboratory tests tend to be non-specific. Biopsy findings in lymphomatoid granulomatosis granuoomatosis a classic triad of features. Special stains immunohistochemistry show large numbers of reactive T-cells with varying numbers of malignant B-cells.
The histological grading is from low to high. Grade 1 only has small numbers of EBV-positive B cells. Grade 2 has increasing numbers of large EBV-positive B cells. Grade 3 more closely resembles conventional forms of diffuse large B-cell lymphoma. Lymphomatoid granulomatosis occurs in the setting of a poorly functioning immune system, for example:.
Lymphomatoid Granulomatosis – NORD (National Organization for Rare Disorders)
The ideal treatment for lymphomatoid granulomatosis is not known. Treatments that have been reported to be successful in at least some patients include:.
Others have progressive disease. The median survival is between 14 and 72 months. Prognosis is worse with grade 2 and 3 disease and if there is grwnulomatosis involvement.
Lymphomatoid Granulomatosis (LYG)
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If you have any concerns with your skin or its treatment, see a dermatologist for advice. Lymphomatoid granulomatosis complicating other haematological malignancies.
British Journal granluomatosis Dermatologypp— Prolonged remission of refractory lymphomatoid granulomatosis after autologous hemopoietic stem cell transplantation with post-transplantation maintenance interferon. Leukemia and Lymphoma, February ; 47 2: International Journal of Dermatology,45, — High activity of rituximab combined with cladribine and cyclophosphamide in a patient with pulmonary Lymphomatoid granulomatosis and bone marrow involvement.
Leukemia and Lymphoma, August ; 47 8: