High grade B-cell lymphoma (HGBL)
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Based on the current guidelines and the current state of research, there are different diagnostic recommendations for patients with High grade B-cell Lymphoma. We have summarized the most important information on the classification and diagnostic methods at MLL. In addition, we provide further links on the prognosis and therapy of high grade B-cell lymphomas, so that you can inform yourself in more detail.
HGBL: Classification
In the WHO classification 2022, high grade B-cell lymphomas are assigned to mature B-cell neoplasms. We would like to present the following three high grade B-cell lymphomas in more detail:
- Diffuse large B-cell lymphoma/high grade B-cell lymphoma with MYC-/ and BCL2 rearrangements (DLBCL/HGBL-MYC/BCL2)
- HGBL with 11q aberrations
- HGBL, NOS
"Diffuse large B-cell lymphoma/high grade B-cell lymphoma with MYC and BCL2 rearrangements (DLBCL/HGBL-MYC/BCL2)" describes an aggressive lymphoma with structural chromosomal aberrations with breakpoints within the MYC and BCL2 loci. To this entity, in contrast to the 2017 WHO classification, are assigned only cases with MYC and BCL2 rearrangements, as these form a class with a clear morphologic spectrum and homogeneous genetic characteristics as well as gene expression profiles. Cases of this entity show large or intermediate/blastoid cells and thus - after confirmation of genetic markers - can be morphologically assigned to DLBCL with MYC and BCL2 rearrangements or HGBL with MYC and BCL2 rearrangements (WHO 2022).
"Burkitt-like lymphoma with 11q aberration" described in the previous 2017 WHO classification is listed as "HGBL with 11q aberrations (HGBL-11q)" in the new WHO classification 2022 and describes a MYC rearrangement-negative, aggressive mature B-cell lymphoma. Cytomorphology, immunophenotype, and gene expression profile are similar to Burkitt's lymphoma, but the mutational spectrum differs widely (WHO 2022).
Cases with MYC and BCL6 rearrangements are now classified as either "DLBCL, NOS" or "HGBL, NOS" according to their cytomorphologic characteristics due to their more diverse gene expression profiles and mutational spectra. HGBL, NOS represent a heterogeneous category of aggressive mature B-cell neoplasms with intermediate-sized or blastoid cells that cannot be assigned to any other defined lymphoma category (WHO 2022).
HGBL: Diagnostic methods and their significance
HGBL: Prognosis and therapy
DLBCL/HGBL-MYC/BCL2 are mostly aggressive lymphomas with unfavorable prognosis, as BCL2 and/or BCL6 additional rearrangements are an independent negative prognostic factor for survival in the first two years. Patients with DLBCL-like morphology seem to have a better prognosis than patients with Burkitt-like morphology.
Therapy is a major challenge. In order to establish guidelines for treatment in the future, large national and international studies are ongoing (Novo et al. 2019, Tisi et al. 2019, Ok & Medeiros 2020, Wang et al. 2020, Olszewski et al. 2022).
HGBL: Recommendation
Important notes on the test material
If enlarged lymph nodes are clinically prominent, one should be removed and histologically and immunohistologically processed. Since bone marrow involvement is common (59-94%), the diagnosis can often be made in the bone marrow. In addition, a CSF examination by immunophenotyping is recommended. Here, it is important to note that the CSF should be examined on the same day within four hours, if possible, in order to obtain a valid result.
Status: November 2023