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Published on: March 17, 2014
Interferon regulatory factor 8 expression and features in blastic plasmacytoid dendritic cell neoplasm and extranodal
Yuejiao Lang1, Xiaoqin Dai1, Li Sun1
1Department of Pathology, the First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
Insights
Interferon regulatory factor 8 (IRF8) shows high expression in blastic plasmacytoid dendritic cell neoplasm (BPDCN) and extranodal NK/T-cell lymphoma, nasal type (ENKTL). This diagnostic marker is promising for identifying these specific hematolymphoid neoplasms.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Blastic plasmacytoid dendritic cell neoplasm (BPDCN) and extranodal NK/T-cell lymphoma, nasal type (ENKTL) are rare hematolymphoid neoplasms.
- Accurate diagnosis is crucial for appropriate treatment and patient outcomes.
- Interferon regulatory factor 8 (IRF8) is a transcription factor involved in immune cell development.
Purpose of the Study:
- To evaluate the diagnostic utility of IRF8 expression in BPDCN and ENKTL.
- To compare IRF8 expression patterns in BPDCN and ENKTL with other hematolymphoid neoplasms.
- To investigate the correlation between IRF8 expression, genetic variations, and clinical features in ENKTL.
Main Methods:
- Immunohistochemistry was employed to assess IRF8 protein expression in 19 BPDCN and 59 ENKTL cases.
- Expression levels were compared against a panel of other hematolymphoid malignancies, including myeloid sarcoma, lymphoblastic leukemia/lymphoma, and histiocytic sarcoma.
- DNA sequencing was performed to identify IRF8 genetic variations in a subset of BPDCN and ENKTL cases.
Main Results:
- IRF8 was highly expressed in 100% of BPDCN and 91.53% of ENKTL cases, with distinct staining patterns.
- Significantly higher IRF8 expression was observed in BPDCN and ENKTL compared to other evaluated neoplasms.
- IRF8 genetic point mutations were identified in 33.33% of BPDCN and 10% of ENKTL cases.
Conclusions:
- IRF8 demonstrates significant diagnostic value for differentiating BPDCN and ENKTL from other hematolymphoid neoplasms.
- The findings support IRF8 as a potential biomarker for these rare lymphomas.
- Further research into IRF8's role in ENKTL prognosis is warranted.
Aims:
To investigate the diagnostic value of Interferon regulatory factor 8 (IRF8) in blastic plasmacytoid dendritic cell neoplasm (BPDCN) and extranodal NK/T-cell lymphoma, nasal type (ENKTL).
Methods:
Immunohistochemistry staining was used to detect IRF8 expression in 19 cases of BPDCN and 59 cases of ENKTL. In addition, 21 cases of myeloid sarcoma, 30 of B-lymphoblastic leukemia/lymphoma (B-ALL/LBL), 30 of T-lymphoblastic leukemia/lymphoma (T-ALL/LBL), 10 of histiocytic sarcoma, 10 of Langerhans cell histiocytosis, and 9 of follicular dendritic cell sarcoma were also included. DNA sequencing detected IRF8 genetic variation in 6 cases of BPDCN and 20 cases of ENKTL.
Results:
IRF8 expression was detected in 100.00% (19/19) of BPDCN, exhibiting a strong and uniform staining pattern, and in 91.53% (54/59) of ENKTL, with varying degrees of staining intensity. Weak and focal staining was detected in 33.33% (7/21) of myeloid sarcoma, 13.33% (4/30) of B-ALL/LBL, and 11.11% (1/9) of follicular dendritic cell sarcoma. No expression was found in T-ALL/LBL, histiocytic sarcoma, or Langerhans cell histiocytosis. The proportion of IRF8 positive expression was higher in BPDCN and ENKTL than in other hematolymphoid neoplasms. In ENKTL, the average IRF8 expression was higher in nasal cases than in extranasal cases and in cases with mitosis figures of more than 4/10 high-power field (HPF). Predominantly large transformed cell morphology and extranasal involvement site might serve as independent prognostic factors of two-year survival in ENKTL. IRF8 genetic point mutations were found in 33.33% (2/6) of BPDCN and 10.00% (2/20) of ENKTL.
Conclusion:
The study demonstrated the promising value of IRF8 in the diagnosis of BPDCN and ENKTL.
