angiocentric T-cell lymphoma (lymphomatoid granulomatosis, polymorphic reticulosis, midline malignant reticulocytosis, Stewart's granuloma)
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Introduction
Disorder with combined features of inflammation & neoplastic processes.
Etiology
- conisidered a pleomorphic T-cell lymphoma
Epidemiology
- mean age at diagnosis is 48 years
- male:female ratio is 1.7:1
Pathology
- histologic features vary in spectrum in the same individual
- benign interstitial pneumonitis
- lymphoma in 50%
- multisystem involvement is common
- pulmonary
- skin
- brain
- kidney
- airway involvement is unusual, but may be extensive
- generally due to lymphoma
- most frequent cause of death
- skin
- angiocentric & angiodestructive granulomatous infiltrate
- pleomorphic & highly atypical lymphoreticular cells
- immunophenotypic markers of T-cells
- often massive necrosis
Clinical manifestations
- cough
- dyspnea
- hemoptysis
- fever
- weight loss
- malaise
- central nervous system symptoms
- peripheral neuropathy
- skin lesions (30-50%)
- macules, papules, nodules, plaques, vesicles
- initially erythematous lesions
- ulceration may occur later
- ichthyosis
- alopecia
- distribution*
- gluteal areas, lower legs, head & neck
* angiocentric lymphoma may present as lethal midline granuloma of the face with massive destruction of soft tissue
Laboratory
Diagnostic procedures
Radiology
- chest X-ray (similar to Wegener's granulomatosis)
- patchy, ill-defined infiltrates
- multiple nodules - 1/3 cavitate
- pleural effusion (25%)
- hilar adenopathy (suggests lymphomatous changes)
- computed tomography (CT) & magnetic resonance imaging (MRI) of brain
Differential diagnosis
- Wegener's granulomatosis
- polymorphic reticulosis
Management
- prognosis: poor, mortality is 60-90%
- early treatment with prednisone & cyclophosphamide may transiently halt progression of disease
More general terms
Additional terms
- granulomatosis
- granulomatosis with polyangiitis; Wegener's granulomatosis
- midline granuloma (facial granuloma)
References
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 790
- ↑ Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 554
- ↑ Hale CS Skin nonmelanocytic tumor Lymphoma and related disorders - T cell / NK cell neoplasms Angiocentric lymphoma Pathology Outlines https://www.pathologyoutlines.com/topic/skintumornonmelanocyticangiocentriclymphoma.html