期刊
AMERICAN JOURNAL OF SURGICAL PATHOLOGY
卷 37, 期 2, 页码 272-281出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/PAS.0b013e31826cb1d1
关键词
plasmablastic lymphoma; Richter syndrome; follicular lymphoma; chronic lymphocytic leukemia/small lymphocytic lymphoma; MYC; BCL2; plasma cell differentiation
资金
- Instituto de Salud Carlos III
- Fondo de Investigacion Sanitaria
- FEDER [PI11/00907]
- European Regional Development Fund (ERDF)
- European Union
- One way to Europe
- Spanish Comision Interministerial de Ciencia y Tecnologia (CICYT) [SAF08-3630]
- Red Tematica de Investigacion Cooperativa del Cancer (RTICC) [RD06/0020/0039]
- Premi Fi de Residencia Emili Letang, Hospital Clinic
Histologic transformation of low-grade B-cell lymphoma to diffuse large B-cell lymphoma is associated with poor prognosis. Although plasma cell differentiation is common in these lymphomas, an overt plasmablastic transformation (PBL-T) has been only rarely reported. We report 6 cases of PBL-T occurring in 3 chronic lymphocytic leukemias (CLL) and 3 follicular lymphomas. Five patients were men, and the mean age was 65 years (range, 52 to 72 y). None of them had history of immunodeficiency. In 3 cases the PBL-T occurred 34 to 85 months after the initial diagnosis, and in 3 it was detected simultaneously with the small cell component at diagnosis. All patients received chemotherapy after transformation, and 4 died 4 to 24 months after this diagnosis. In 3 cases, PBL-T occurred in an extranodal site. All PBL-Ts had immunoblastic morphology with admixed plasma cells, were CD20 and PAX5 negative, expressed l light chain, and 5 were CD138 positive. All cases were negative for HHV8, and only 1 PBL-T was Epstein-Barr virus positive. Evidence of a clonal relationship between the small cell and PBL-T components was found in 5 cases. In 2 CLL cases, both components had 13q deletions, and in all follicular lymphoma cases both components harbored the t(14;18) translocation. MYC translocations were observed in 2 cases transformed from a CLL. In conclusion, PBL-T expands the clinicopathologic spectrum of the transformation of low-grade B-cell lymphomas. These transformed tumors are clinically, histologically, and phenotypically similar to primary plasmablastic lymphomas, but they are not associated with immunodeficiency and rarely have Epstein-Barr virus infection or MYC alterations.
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