lymphomatoid granulomatosis

(redirected from angiocentric immunoproliferative lesion)
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Related to angiocentric immunoproliferative lesion: angiocentric lymphoproliferative lesion

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.