Published online May 26, 2024. doi: 10.12998/wjcc.v12.i15.2655
Revised: March 15, 2024
Accepted: April 9, 2024
Published online: May 26, 2024
Processing time: 96 Days and 1.4 Hours
High-grade B-cell lymphoma (HGBL) is an unusual malignancy that includes myelocytomatosis viral oncogene (MYC), B-cell lymphoma-2 (BCL-2), and/or BCL-6 rearrangements, termed double-hit or triple-hit lymphomas, and HGBL-not otherwise specific (HGBL-NOS), which are morphologically characteristic of HGBL but lack MYC, BCL-2, or BCL-6 rearrangements. HGBL is partially transformed by follicular lymphoma and other indolent lymphoma, with few cases of marginal zone lymphoma (MZL) transformation. HGBL often has a poor prognosis and intensive therapy is currently mainly advocated, but there is no good treatment for these patients who cannot tolerate chemotherapy.
We reported a case of MZL transformed into HGBL-NOS with TP53 mutation and terminal deoxynucleotidyl transferase expression. Gene analysis revealed the gene expression profile was identical in the pre- and post-transformed tissues, suggesting that the two diseases are homologous, not secondary tumors. The chemotherapy was ineffective and the side effect was severe, so we tried combination therapy including venetoclax and obinutuzumab. The patient tolerated treatment well, and reached partial response. The patient had recurrence of hepatocellular carcinoma and died of multifunctional organ failure. He survived for 12 months after diagnosis.
Venetoclax combined with obinutuzumab might improve the survival in some HGBL patients, who are unsuitable for chemotherapy.
Core Tip: Marginal zone lymphoma (MZL) are a group of B-cell malignant neoplastic diseases with the most common transformation to diffuse large B-cell lymphoma. Herein, we report one rare case of MZL converted to high-grade B-cell lymphoma, not otherwise specific (HGBL-NOS) and concomitant terminal deoxynucleotidyl transferase expression in a 63-year-old male for the first time. HGBL-NOS often has a poor prognosis. The current treatment advocates high-dose chemotherapy, but there is currently no effective treatment for patients who cannot tolerate chemotherapy. We have attempted targeted pharmacological combinations and achieved certain therapeutic effects, which may provide new treatment for these patients.