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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Rare Disease Database
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 lymphomatoie 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.
Lymphomatoid Granulomatosis (LYG)
Other organs can be involved including kidney, liver, spleen, lymph nodes, eyes and the gastrointestinal tract. The most common presenting symptoms are cough, fever and skin lesions. Affected patients may feel generally unwell, with weight loss, neurological abnormalities, breathlessness and chest pain.
There are usually no symptoms lymphomatlid the skin lesions, but they 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 include 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:. The ideal treatment for lymphomatoid granulomatosis is not known. Treatments that have been reported to be successful granulmatosis 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 lymphomatold there is neurological involvement.
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Lymphomatoid Granulomatosis – NORD (National Organization for Rare Disorders)
Lymphomatoid granulomatosis complicating other haematological malignancies. British Journal of Dermatologypp— Prolonged remission llymphomatoid 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: