Diffuse Large B-cell Lymphoma Associated with Chronic Inflammation
Wei Liu, MD, PhD
L. Jeffrey Medeiros, MD
Key Facts
Terminology
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DLBCL-CI is large B-cell neoplasm that occurs in context of longstanding chronic inflammation
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Most cases occur in body cavities or narrow spaces
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Pyothorax-associated lymphoma (PAL) is most common and prototype
Etiology/Pathogenesis
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Chronic pyothorax precedes PAL type of DLBCL-CI
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Very long latency period from onset of pyothorax until PAL
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Other causes of DLBCL-CI are rare and include
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Metallic implants in bones/joints, surgical mesh implant, longstanding hydrocele
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Chronic osteomyelitis; splenic false cyst, atrial myxoma
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Clinical Issues
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Patients with PAL present with
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Chest/back pain, tumor/swelling of chest wall
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B symptoms, cough, dyspnea, hemoptysis
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Other types of DLBCL-CI can present incidentally
Microscopic Pathology
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Diffuse large B-cell lymphoma
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Centroblastic, immunoblastic, or plasmablastic
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Ancillary Tests
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Nongerminal center B-cell immunophenotype
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EBV(+), HHV8(−)
Top Differential Diagnoses
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Primary effusion lymphoma
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Systemic lymphomas involving body cavity lining
TERMINOLOGY
Abbreviations
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Diffuse large B-cell lymphoma associated with chronic inflammation (DLBCL-CI)
Synonyms
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Pyothorax-associated lymphoma (PAL)
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PAL is not true synonym but represents > 90% of cases of DLBCL-CI
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Definitions
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DLBCL-CI is large B-cell neoplasm that occurs in context of longstanding chronic inflammation
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Most cases occur in body cavities or narrow spaces
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Pyothorax-associated lymphoma (PAL)
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Most common form of DLBCL-CI and prototype
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Develops in pleural cavity of patients with longstanding pyothorax
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ETIOLOGY/PATHOGENESIS
Infectious Agents
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Epstein-Barr virus (EBV) is present in most cases of DLBCL-CI reported
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EBV(+) in type III latency pattern in most cases
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Chronic Inflammation
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Chronic pyothorax precedes PAL type of DLBCL-CI in most cases
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Very long latency period from onset of pyothorax until PAL
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19-67 years; median 43 years
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Other causes of DLBCL-CI are rare including
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Metallic implants in bones and joints; surgical mesh implant
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Chronic osteomyelitis; longstanding hydrocele
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False cyst within spleen; atrial myxoma
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Causes of pyothorax in patients who develop PAL type of DLBCL-CI
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Result of surgical use of artificial pneumothorax to treat pulmonary tuberculosis
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Widely performed in Japan, especially from 1930s through 1950s
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˜ 15-20% of patients with PAL have no history of artificial pneumothorax
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Tuberculous pleuritis is another cause of chronic pyothorax
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Possible roles of chronic inflammation in pathogenesis
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Generation of reactive oxygen species
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“Local immunosuppression”
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EBV(+) B cells can secrete various cytokines; e.g., interleukin-10 inhibits T-cell proliferation
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Fibrosis surrounding area may limit access by cells involved in immunosurveillance
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Autocrine growth
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Interleukin-6 is autocrine growth factor that may be involved
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Patients with DLBCL-CI also may have systemic immunosuppression
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Not obvious clinically in most patients but possibility not excluded
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Possible Role of Gender
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PAL type of DLBCL-CI is much more common in men
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Men have substantially worse prognosis
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Unknown if related to hormonal status, environmental factors, patient behavior, or genetic component
Genetic Factors
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PAL type of DLBCL-CI appears to arise from post-germinal center B cells, often with crippling Ig gene mutations
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EBV may rescue cells from apoptosis (which would be expected physiologically)
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CLINICAL ISSUES
Epidemiology
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Incidence
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Rare
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Most cases of PAL type of DLBCL-CI are reported from Japan; rare in Western countries
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2% of patients with chronic pyothorax develop PAL in Japan
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Non-PAL types of DLBCL-CI are exceptionally rare, mostly case reports
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Age
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Adults
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Median 65-70 years; range 29-88 years
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Gender
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Marked male predominance
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