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Published on: October 14, 2021
Frequent immunoglobulin G4 expression in a common variant of primary cutaneous marginal zone B-cell lymphoma
Jing Ru Sun1, Lin Nong2, Xiao Qing Liu1
1Department of Dermatology and Venerology, Peking University First Hospital, Beijing, China.
Insights
Immunoglobulin G4 (IgG4) expression is linked to primary cutaneous marginal zone B-cell lymphoma (PCMZL). This study suggests a clonal progression between PCMZL and IgG4-related disease.
Area of Science:
- Immunology
- Dermatopathology
- Oncology
Background:
- Immunoglobulin G4 (IgG4)-related disease is a systemic condition characterized by specific histopathological features including lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis.
- Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is a type of non-Hodgkin lymphoma affecting the skin.
- Recent observations noted IgG4 expression in a subset of PCMZL cases with plasmacytic differentiation.
Observation:
- This report details three cases of common variants of PCMZL.
- These cases exhibited predominant and varied IgG4 expression.
- The clinical and histopathological presentations suggest a potential link between PCMZL and IgG4-related disease.
Findings:
- The study observed significant IgG4 expression in common variants of PCMZL.
- The varied IgG4 expression levels indicate a potential spectrum of disease.
- The findings support a relationship between PCMZL and IgG4-related disease.
Implications:
- The observed IgG4 expression in PCMZL suggests a potential shared pathway or clonal evolution.
- Understanding this link may refine diagnostic criteria for both PCMZL and IgG4-related disease.
- Further research is warranted to elucidate the clonal progression and underlying mechanisms connecting these entities.
Abstract:
Immunoglobulin (Ig)G4 expression was recently observed in a proportion of primary cutaneous marginal zone B-cell lymphoma (PCMZL) with plasmacytic differentiation. IgG4-related disease is characterised by polyclonal lymphoplasmacytic infiltration with IgG4 expression, storiform fibrosis and obliterative phlebitis in histopathology. Here we report three cases of common variants of PCMZL with predominant and varied IgG4 expression, suggesting there is an underlying clonal progression between these two entities.
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