Angiocentric and angio-destructive infiltration by lymphoblasts/plasma cells, histiocytes/large atypical lymphocytes, mostly T-cells, with abnormal mitotic activity
Multi-system angiocentric lymphoproliferative disease of unclear etiology (EBV has been isolated from some lesions)
May transform into an aggressive NHL in 15-25% of cases
Abnormal T-cell function with monoclonal T-cell proliferation: has been demonstrated
Primary T-cell lymphomas (due to HTLV-I) can produce a syndrome like lymphomatoid granulomatosis
Pathologic Patterns
Destructive inflammatory granulomatous angiitis with lymphoid infiltrate (with atypical and immature cells with abundant mitoses/ paucity of PMN and eosinophils)
Diagnosis
PFT’s: decreased DLCO/ increased Vd/Vt ratio
ABG: elevated A-a gradient
CXR: resembles Wegener’s
Upper airway Bx: diagnostic
OLB: may be neccesary for diagnosis
CXR/Chest CT patterns: pulmonary lesions usually wax-and-wane with new lesions appearing while others regress/ hilar and mediastinal nodes are rarely present
Fluffy nodular infiltrates/lung nodules (most common pattern): lower-lobe predilection (usually peripheral, may coalesce)
May be cavitary
May produce large mass lesions
Endobronchial lesions: have been reported in some cases (bronchioles>lobar bronchi), may produce obstruction
CBC: usually normal
Dysproteinemia: usually absent
Clinical
Upper Airway Manifestations
Pulmonary Manifestations
Epidemiology: usually present
Clinical
Chest pain
Dyspnea
Cough
Pulmonary Hypertension (see Pulmonary Hypertension, [[Pulmonary Hypertension]]): due to pulmonary vasculitis
Pneumonia-like illness: presents with dyspnea/ cough/ sputum
Endobronchial lesion: obstructive symptoms/ hemoptysis (may be massive)
Pneumothorax:
CNS Manifestations
Epidemiology: 25% of cases
Seizures/ asymmetric focal deficits
Dermatologic Manifestations
Epidemiology: 50% of cases
Patchy erythematous or papular rash (usually small, confluent and on the extremities)/ ulcerations/ subcutaneous nodules
Renal Mnaifestations
Clinical involvement is uncommon (despite autopsy evidence of GLN)
Clinical involvement is rare but autopsy involvement (focal necrosis and proliferative lesions without GLN) is common
Other Manifestations
Hepatic (unusual): involvement worsens prognosis
Lymph node/ spleen (rarely involved): involvement does not affect prognosis
Nasopharynx and upper airways (rarely involved, in contrast to Wegener’s)
Other: fever/ malaise
Aggressive Lymphoma: occurs in 15-25% of cases
Treatment
Asymptomatic Disease
Some patients do not require treatment (due to slowly progressive disease)
Symptomatic Disease
Steroids + Cytoxan
Responsive but recurrence/ refractory disease is frequent (and high-grade NHL complicates some cases)
Better long-term response with minimal disease and lack of CNS disease
50% of all patients receiving qOD steroids + Cytoxan had average survival of 4 years
XRT
Useful for localized lesions
Prognosis
Median survival is 17 months (despite treatment)/ death due to CNS or lung involvement
Virtually all patients that did not achieve remission developed lymphomas
>50% of patients die within first 5 years
Death may occur due to CNS etiology/ respiratory etiology (hemoptysis)/ progressive NHL
