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.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

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.
Farlex Partner Medical Dictionary © Farlex 2012

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.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.

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.
McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.
References in periodicals archive ?
Fatal haemoptysis in a case of lymphomatoid granulomatosis treated with rituximab.
Successful treatment of mediastinal lymphomatoid granulomatosis with rituximab monotherapy.
Establishing the diagnosis of lymphomatoid granulomatosis usually requires an open lung or video-assisted thoracoscopic biopsy; because of the focal nature of lymphomatoid granulomatosis and the fact that it is not bronchocentric, a low diagnostic yield with bronchoscopic or transbronchial biopsies is likely.
Recent World Health Organization (WHO) classification has characterized lymphomatoid granulomatosis as a B-cell neoplasm.
Lymphomatoid granulomatosis (LyG) is an EBV-driven lymphoproliferative disorder that typically affects adults with a known immunodeficiency, such as human immunodeficiency virus (HIV), iatrogenic immunosuppression, and Wiskott-Aldrich syndrome.
Patients with lymphomatoid granulomatosis, a lymphoma of large B cells, may present with fever, weight loss, neurologic deficits, and respiratory manifestations.
Lymphomatoid granulomatosis is recognized as an angiocentric and angiodestructive disease arising in a variety of extranodal sites.
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.
Lymphomatoid granulomatosis is an angiocentric and angiodestructive lymphoproliferative disease involving extranodal sites, comprised of [EBV.sup.+] B cells admixed with numerous T cells and other inflammatory cells, which usually predominate (Figure 3, N through Q).
Lymphomatoid granulomatosis is graded into grades 1 to 3 based on the proportion of large [EBV.sup.+] B cells to the relative lymphocyte background.
Lymphomatoid granulomatosis (angiocentric immunoproliferative disorder) is an angioinvasive and angiodestructive lymphoid process that commonly presents in extranodal sites, often in the lung, but may involve other organs including the skin and central nervous system.