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angiocentric T-cell lymphoma (lymphomatoid granulomatosis, polymorphic reticulosis, midline malignant reticulocytosis, Stewart's granuloma)
Disorder with combined features of inflammation & neoplastic processes.
Etiology:
- conisidered a pleomorphic T-cell lymphoma
Epidemiology:
1) mean age at diagnosis is 48 years
2) male:female ratio is 1.7:1
Pathology:
1) histologic features vary in spectrum in the same individual
a) benign interstitial pneumonitis
b) lymphoma in 50%
2) multisystem involvement is common
a) pulmonary
b) skin
c) brain
d) kidney
3) airway involvement is unusual, but may be extensive
a) generally due to lymphoma
b) most frequent cause of death
4) skin
a) angiocentric & angiodestructive granulomatous infiltrate
b) pleomorphic & highly atypical lymphoreticular cells
c) immunophenotypic markers of T-cells
d) often massive necrosis
Clinical manifestations:
1) cough
2) dyspnea
3) hemoptysis
4) fever
5) weight loss
6) malaise
7) central nervous system symptoms
8) peripheral neuropathy
9) skin lesions (30-50%)
a) macules, papules, nodules, plaques, vesicles
b) initially erythematous lesions
c) ulceration may occur later
d) ichthyosis
e) alopecia
d) 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:
1) complete blood count (CBC)
2) renal function tests
3) urinalysis
4) skin biopsy
Special laboratory:
1) electrocardiogram: may show myocardial ischemia
2) upper GI endoscopy
3) bronchoscopy
Radiology:
1) chest X-ray (similar to Wegener's granulomatosis)
a) patchy, ill-defined infiltrates
b) multiple nodules - 1/3 cavitate
c) pleural effusion (25%)
d) hilar adenopathy (suggests lymphomatous changes)
2) computed tomography (CT) & magnetic resonance imaging (MRI) of brain
Differential diagnosis:
1) Wegener's granulomatosis
2) polymorphic reticulosis
Management:
1) prognosis: poor, mortality is 60-90%
2) early treatment with prednisone & cyclophosphamide may transiently halt progression of disease
Interactions
disease interactions
Related
granulomatosis
granulomatosis with polyangiitis; Wegener's granulomatosis
midline granuloma (facial granuloma)
General
peripheral T-cell lymphoid neoplasm
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