33. Triple Hit Follicular Lymphoma with MYC, BCL-2, and BCL-6 rearrangements, a case report

      Germinal center type B-cell lymphomas include a spectrum of lesions ranging from low-grade follicular lymphomas to diffuse large B-cell lymphoma (DLBCL). A subset of DLBCL cases harbor MYC rearrangements in combination with BCL-2 and/or BCL-6 translocations and are commonly called double- or triple-hit lymphomas. This entity is both clinically and histologically aggressive with poor outcomes and in 2016, WHO classified this as High-grade B-cell lymphoma (HGBL) with MYC and BCL-2 and/or BCL-6 rearrangements. Rarely documented are cases that display morphology of follicular lymphoma (DLBCL) but are cytogenetically consistent with HGBL. We present the case of a 67-year-old male diagnosed with low-grade follicular lymphoma (grade 1), follicular pattern of the left axilla. Over the next several months, a biopsy of retroperitoneal adenopathy showed a low-grade follicular lymphoma (grade 2), diffuse pattern and a pleural effusion contained an atypical CD10 positive B-cell population on flow cytometry consistent with involvement. He received multiple rounds of chemotherapy and approximately one year later developed a right neck mass which was excised. Morphologically, this appeared to be a low-grade diffuse pattern follicular lymphoma with a low mitotic index and predominantly small cells. Cytogenetic studies revealed rearrangements of MYC, BCL2, and BCL6, consistent with HGBL. This discordance between the morphology and the high-grade findings on FISH makes classification of these lesions difficult as a double- or triple-hit follicular lymphoma and is not currently recognized in the WHO classification. This in turn complicates treatment as therapy protocols for HGBL and follicular lymphoma are significantly different. Recent literature suggests that this is an aggressive disease with improved response to intensive chemotherapy despite the low-grade morphology.
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