Journal Article

174 A Case of Follicular Lymphoma Transforming to CD5+/CD10+ Triple-Hit Lymphoma

Liron Barnea Slonim, Sherif Ibrahim, Virginia Donovan and Matthew Geller

in American Journal of Clinical Pathology

Published on behalf of American Society for Clinical Pathology

Volume 149, issue suppl_1, pages S74-S74
Published in print January 2018 | ISSN: 0002-9173
Published online January 2018 | e-ISSN: 1943-7722 | DOI: http://dx.doi.org/10.1093/ajcp/aqx121.173
174 A Case of Follicular Lymphoma Transforming to CD5+/CD10+ Triple-Hit Lymphoma

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Abstract

A 58-year-old man presented with abdominal pain, weight loss, and fatigue. Imaging showed a complex mass in the left lower quadrant involving the sigmoid colon, extensive lymphadenopathy and osseous metastases. A biopsy revealed grade 3A follicular lymphoma. Flow demonstrated bone marrow involvement, and FISH demonstrated a t(14;18)(q32;q21.3) translocation. Treatment was initiated, but three weeks later, he presented with headaches and neurologic symptoms. Intrathecal chemotherapy was started following a positive cerebrospinal fluid (CSF) evaluation. There was initial improvement; however, five months later, he had increased adenopathy and positive CSF cytology. Flow cytometry showed a CD5/CD10 double-positive large B-cell lymphoma. FISH identified a t(14;18) translocation involving the IGH-BCL2 genes, rearrangement of MYC, and rearrangement of BCL6, consistent with triple-hit diffuse large B-cell lymphoma. The patient’s course was further complicated with neutropenic sepsis and admission to the medical intensive care unit. Double-hit lymphomas (DHLs) are aggressive neoplasms with translocations involving the MYC gene combined with another event involving translocation of the BCL2 or BCL6 gene. The combination of MYC translocation with translocations of BCL2 and BCL6 are defined as triple-hit lymphomas (THLs). These neoplasms typically present in older men, with extranodal involvement, mostly in the head and neck region and gastrointestinal tract. They are usually CD10+, BCL6+, and BCL2+, with 50% being IRF4/MUM1+. A recent study observed that both BCL-2 and MYC gene gain/amplification were strong independent indicators of central nervous system involvement in DLBCL. It was reported as a predictor of poor clinical behavior and survival. To our knowledge, this is the first reported case of a double- or triple-hit DLBCL acquiring CD5 positivity as a secondary event. The aggressive features, resistance to therapy and leptomeningeal spread concur with previously reported characteristics. The field of the DHL/THL large B-cell lymphomas is becoming more complex. Future studies should help to their rapid recognition and improved patient outcomes.

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Subjects: Medical Skills ; Pathology

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