lymphomatoid granulomatosis


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granulomatosis

 [gran″u-lo″mah-to´sis]
any condition involving the formation of multiple granulomas.
allergic granulomatosis churg-strauss syndrome.
eosinophilic granulomatosis Langerhans cell histiocytosis.
Langerhans cell granulomatosis Langerhans cell histiocytosis.
lymphomatoid granulomatosis a multisystem disease involving predominantly the lungs, skin, central nervous system, and kidneys, caused by invasion and destruction of vessels by atypical lymphoreticular cells. Many affected patients develop frank lymphoma. It usually affects males, and the most frequent presenting symptoms are cough, shortness of breath, and chest pain. Extrapulmonary manifestations are common, with skin lesions being present in many cases.
granulomatosis sidero´tica a condition in which brownish nodules are seen in the enlarged spleen.
Wegener's granulomatosis a multisystem disease chiefly affecting males, characterized by necrotizing granulomatous vasculitis involving the upper and lower respiratory tracts, glomerulonephritis, and variable degrees of systemic, small vessel vasculitis, which is generally considered to represent an aberrant hypersensitivity reaction to an unknown antigen.

lym·pho·ma·toid gran·u·lo·ma·to·sis

angiocentric malignant lymphoma of the lung; may involve the upper respiratory tract and other parts of the body.
See also: polymorphic reticulosis.

lymphomatoid granulomatosis

[limfō′mətoid]
a condition of unknown cause in which lymphocytes and plasma cells infiltrate the blood vessels, producing an angiocentric lesion. It most often affects the lungs, causing chest pains, cough, and shortness of breath. See also Wegener's granulomatosis.

lymphomatoid granulomatosis

A lymphoproliferative disorder linked to Epstein-Barr viral infection, which affects adults aged 30 to 50. It is characterised by well-circumscribed bilateral nodules on chest films, often seen in immunosuppressed renal transplant recipients and in patients with Sjögren syndrome.

Clinical findings
80% have extrapulmonary involvement—e.g., skin, CNS, kidneys, liver, spleen, adrenal glands, heart, GI tract, etc.
 
Prognosis
64% mortality, median survival of 14 months; death is due to pulmonary destruction accompanied by sepsis; severe T-cell impairment may explain the malignant evolution of this condition.

Controversy
Lymphomatoid granulomatosis was once regarded as a type of pulmonary angiitis and granulomatosis; given that many of these lesions evolve to lymphoma, the WHO has flagged them as B-cell proliferations of uncertain malignant potential.

lymphomatoid granulomatosis

A lymphoproliferative disorder which presents in middle-aged subjects with well-circumscribed bilateral nodules seen on CXR; some cases occur in immunosuppressed renal transplant recipients and in Pts with Sjögren syndrome Clinical 80% have extrapulmonary involvement–eg, skin, CNS, kidneys, liver, spleen, adrenal glands, heart, GI tract, etc Prognosis 64% mortality; median survival of 14 months, death is due to pulmonary destruction accompanied by sepsis; severe T-cell impairment may explain the tendency for malignant degeneration. Cf Lymphoid interstitial pneumonia.

lymphomatoid granulomatosis

a rare pulmonary neoplasm of dogs characterized by infiltration by atypical lymphoreticular cells.
References in periodicals archive ?
Lymphomatoid granulomatosis is graded into grades 1 to 3 based on the proportion of large [EBV.
Association of lymphomatoid granulomatosis with Epstein-Barr viral infection of B lymphocytes and response to interferon-alpha2b.
Recent World Health Organization (WHO) classification has characterized lymphomatoid granulomatosis as a B-cell neoplasm.
Lymphomatoid granulomatosis of all grades is angiocentric, with accumulation of viable cells around the vessels, followed by angio-destruction, vascular invasion, luminal occlusion, and disruption of the vessels.
47) Lymphomatoid granulomatosis is a rarely diagnosed disorder; hence, it is difficult to accumulate more cases for more extensive studies and specific characterization.
Lymphomatoid granulomatosis, diagnosed more commonly in the past, is rare in our series owing to the utility of molecular and flow cytometric analysis to make a definitive diagnosis of clonal population.
Successful treatment of mediastinal lymphomatoid granulomatosis with rituximab monotherapy.
Association of lymphomatoid granulomatosis with EpsteinBarr viral infection of B lymphocytes and response to interferon-alpha 2b.
Lymphomatoid granulomatosis with mesenteric localization: nosologic problems [in French, author's translation].
Is Grade I Lymphomatoid Granulomatosis Associated With IgG4-Related Sclerosing Disease?
A recent study (8) has suggested that some cases formerly considered grade I lymphomatoid granulomatosis (or alternatively benign lymphocytic angiitis and granulomatosis) are distinct from grade II and grade III lymphomatoid granulomatosis and actually represent pulmonary manifestations of IgG4-related sclerosing disease.
In contrast to grade II or III lymphomatoid granulomatosis, atypical lymphocytes were not identified.