B Lymphoproliferative Neoplasms of Uncertain Biological Significance: Report from the IV Workshop of the Italian Group of Hematopathology and Review of the Literature
Abstract
:1. Introduction
2. Submitted Cases of ISFN and ISMCN Associated with Overt Lymphoma
2.1. Case n. 1
2.2. Case n. 2
2.3. Case n. 3
3. Submitted Cases of Early Lymphoproliferative Lesions Originating from the Germinal Center Other Than ISFN and ISMCN
3.1. Case n. 4
3.2. Case n. 5
4. Submitted Cases of Early Lymphoproliferative Lesions Arising in the Setting of Monocytoid Hyperplasia
Case n. 6
5. Submitted Cases of Early Lymphoproliferative Lesions Associated with Infectious Diseases and Chronic Inflammation
5.1. Case n. 7
5.2. Case n. 8
6. Conclusions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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GC Involvement | Non-GC Involvement | Histologic Characteristics | Suggested Procedures When Lymphoproliferative Neoplasms of Uncertain Biological Significance Are Encountered | |
---|---|---|---|---|
Entities accepted in the 4th WHO edition | ||||
“In situ” follicular neoplasia (ISFN) | Yes | No | Lack of polarization, closely packed centrocytes with few centroblast. | Exclude pFL and a composite form with appropriate immunohistochemistry. Suggest a complete staging workup to rule out an overt lymphoma in another site. |
“In situ” mantle cell neoplasia (ISMCN) | No | Yes | Cyclin D1-expressing B-cells in non-expanded mantle zones of otherwise morphologically reactive lymph node. | Exclude a MCLGP and composite form with appropriate immunohistochemistry. Suggest a complete staging workup to rule out an overt lymphoma in another site. |
Entities not included in the in the 4th WHO edition | ||||
Atypical GC BCL2-negative | Yes | No | Isolated follicles with GC composed of aggregates of large centroblast with atypical mitosis without centrocytes and BCL2 negative by immunohistochemistry and FISH analysis. | Apply immunohistochemestry to confirm the presence of follicular dendritic networks and the GC origin of the B cell population. Occurrence of strong and diffuse immunoreactivity for IRF4, BCL2, and c-MYC must be assessed. In addition, FISH analysis for IRF4, BCL2, c-MYC rearrangements is advisable. Exclusion of an overt disease with a complete staging workup is mandatory. |
Early pattern of large B-cell lymphoma (LBCL) with IRF4 rearrangement | Yes | No | Atypical GC enriched with centroblasts, without tingible body macrophages or polarization with strong IRF4 positivity and IRF4 rearrangement. | |
Single extranodal involvement of “double hit” Follicular lymphoma (DH-FL) | Yes | No | Atypical GC B-cell population with follicular dendritic networks and BCL-2 + c-MYC rearrangements. | |
Early Burkitt lymphoma (BL) | No | Yes | Aggregates composed of medium-sized lymphoid cells morphologically and phenotipically consistent with BL cells along with mitotic figures and apoptotic bodies | Apply immunohistochemical analysis to characterize the medium size population with BL features added to FISH to identify c-MYC rearrengement |
Early classic Hodgkin lymphoma (cHL) | No | Yes | Presence of RS and HL cells in perifollicular areas with preserved lymph node architecture. | Evaluation of the preserved lymph node architecture. Investigate the scattered atypical RS- and HL-cells by immunohistochemistry. Exclusion of an overt disease with a complete staging workup is mandatory. |
Case | Gender | Age | Diagnosis | Presence of Overt Lymphoma | Type of Overt Lymphoma |
---|---|---|---|---|---|
Case n. 1 | F | 75 y | FL and ISMCN | Yes | FL |
Case n. 2 | M | 45 y | ISMCN and SLL | Yes | MCL in previous biopsy |
Case n. 3 | M | 45 y | CLL with atypical phenotype and ISFN | Yes | CLL on bone marrow |
Case n. 4 | M | 77 y | FL, 3A with focal localization on the contralateral lymph node | Yes | FL, 3A |
Case n. 5 | F | 72 y | Extranodal FL with BCL2 and c-MYC rearrangement | No | |
Case n. 6 | F | 61 y | Early cHL in monocytoid hyperplasia | Yes | cHL, mixed cellularity |
Case n. 7 | M | 76 y | Necrotizing granulomatous lymphadenitis associated with ISFN | No | |
Case n. 8 | M | 77 y | IgG4-RD with ISFN | No |
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Di Stefano, G.; Magnoli, F.; Granai, M.; Vittone, F.; Santi, R.; Ferrara, D.; Boveri, E.; Florena, A.M.; Fend, F.; Sabattini, E.; et al. B Lymphoproliferative Neoplasms of Uncertain Biological Significance: Report from the IV Workshop of the Italian Group of Hematopathology and Review of the Literature. Hemato 2022, 3, 634-649. https://doi.org/10.3390/hemato3040043
Di Stefano G, Magnoli F, Granai M, Vittone F, Santi R, Ferrara D, Boveri E, Florena AM, Fend F, Sabattini E, et al. B Lymphoproliferative Neoplasms of Uncertain Biological Significance: Report from the IV Workshop of the Italian Group of Hematopathology and Review of the Literature. Hemato. 2022; 3(4):634-649. https://doi.org/10.3390/hemato3040043
Chicago/Turabian StyleDi Stefano, Gioia, Francesca Magnoli, Massimo Granai, Federico Vittone, Raffaella Santi, Domenico Ferrara, Emanuela Boveri, Ada M. Florena, Falko Fend, Elena Sabattini, and et al. 2022. "B Lymphoproliferative Neoplasms of Uncertain Biological Significance: Report from the IV Workshop of the Italian Group of Hematopathology and Review of the Literature" Hemato 3, no. 4: 634-649. https://doi.org/10.3390/hemato3040043
APA StyleDi Stefano, G., Magnoli, F., Granai, M., Vittone, F., Santi, R., Ferrara, D., Boveri, E., Florena, A. M., Fend, F., Sabattini, E., Paulli, M., Ponzoni, M., Lazzi, S., Pileri, S. A., Leoncini, L., & the Italian Group of Hematopathology. (2022). B Lymphoproliferative Neoplasms of Uncertain Biological Significance: Report from the IV Workshop of the Italian Group of Hematopathology and Review of the Literature. Hemato, 3(4), 634-649. https://doi.org/10.3390/hemato3040043