lymphomatoid granulomatosis

(redirected from angiocentric lymphoma)


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.
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.

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 ?
The search strategy was based on combinations of "(EBV DNA) OR (Epstein-Barr virus DNA) OR (Epstein-Barr viral DNA) OR (EBV deoxyribonucleic acid) OR (Epstein Barr virus deoxyribonucleic acid) OR (Epstein-Barr viral deoxyribonucleic acid)" and "(NK/T cell lymphoma) OR (natural killer/T cell lymphoma) OR (angiocentric lymphoma)" in the [Title/Abstract].
NK/T-cell lymphoma, nasal type, was formerly designated as an angiocentric lymphoma owing to common findings of necrosis and angiocentric infiltration or angioinvasion [2, 3].
The differential diagnosis of necrotizing sinonasal lesions includes infections; autoimmune disease/vasculitis, particularly granulomatous necrotizing vasculitis; neoplasia, particularly angiocentric lymphoma; and iatrogenic lesions (caused by drug abuse, particularly cocaine use).
In contrast, a study in Japan reported angiocentric lymphoma (35.9%) followed by B-cell lymphoma (22.6%) as the predominant histological types in Japan.
Until recently, it has remained obscure whether Angiocentric lymphoma with the NK-cell profile is a true NK-cell neoplasm or a bonafide Tcell neoplasm with aberrant expression of NK-cell markers; Hence, a few investigators have preferred the noncommittal lineage designation NK/T-cell lymphoma rather than putative NK-cell lymphoma.
Tu-mour cells with positive T-cell markers (angiocentric lymphoma and peripheral T-cell lymphoma) are pre-dominant in nasal cavities.
(4,5) On the contrary, according to Hatta et al, (6) the most common histological type, in Japan, is angiocentric lymphoma (35.9%), followed by B-cell lymphoma (22.6%), peripheral T-cell lymphoma types (15.1%), and other lymphomas and nonspecific types.
Angiocentric lymphoma of the head and neck: patterns of systemic failure after radiation treatment.
Clinicopathologic study of CD56 (NCAM)-positive angiocentric lymphoma occurring in sites other than the upper and lower respiratory tract.