Genetic mechanism for the loss of PRAME in B cell lymphomas
| Authors | |
|---|---|
| Year of publication | 2022 |
| Type | Article in Periodical |
| Magazine / Source | Journal of Clinical Investigation |
| MU Faculty or unit | |
| Citation | |
| web | https://www.jci.org/articles/view/160983 |
| Doi | http://dx.doi.org/10.1172/JCI160983 |
| Keywords | CHRONIC LYMPHOCYTIC-LEUKEMIA22Q11 |
| Description | To the Editor: Takata et al. (1) reported that patients with diffuse large B cell lymphoma (DLBCL) relatively frequently (13% of patients) harbor a deletion at the 22q11.22 locus that involves the PRAME gene, and that PRAME loss is associated with poor outcomes and leads to cytotoxic T cell immune escape. The authors comment that “deletions...were located close to the Ig? gene.” I would like to bring to the attention of the authors and readers that the PRAME gene and neighboring ZNF280A, ZNF280B, and GGTLC2 genes are located between variable (V) subgenes for the immunoglobulin lambda (Ig?) light chain (Figure 1). The PRAME deletion is inevitable when a B lymphocyte (normal or malignant) rearranges the Ig? locus and utilizes one of the many V subgenes located more distantly from the J-C region. It is known that approximately 30% to 40% of B lymphocytes express Ig? (~60%–70% express Ig?, since this locus for the Ig light chain is rearranged before Ig?). Therefore, it is not surprising that the loss of PRAME has been previously noted in multiple B cell malignancies, especially chronic lymphocytic leukemia (2–4). Takata et al. (1) observed that patients with PRAME deletions more often have an Ig? rearrangement, but they also report cases of DLBCL with a PRAME deletion and rearranged Ig?. |