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Hum Pathol. 2015 Feb;46(2):260-71. doi: 10.1016/j.humpath.2014.10.021. Epub 2014 Nov 13.

Lymphoblastic transformation of follicular lymphoma: a clinicopathologic and molecular analysis of 7 patients.

Author information

1
Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, New York, NY 10065, USA. Electronic address: jut9021@med.cornell.edu.
2
Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, New York, NY 10065, USA.
3
Institute for Computational Biomedicine, Weill Cornell Medical College, New York, NY 10065, USA; Department of Medicine, Weill Cornell Medical College, New York, NY 10065, USA.
4
Institute for Computational Biomedicine, Weill Cornell Medical College, New York, NY 10065, USA.
5
Department of Pathology, Massachusetts General Hospital, Boston, MA 02114, USA.
6
Institut Universitaire de Pathologie, 1011 Lausanne, Switzerland.
7
Department of Pathology and Laboratory Medicine, Cleveland Clinic, Cleveland, OH 44195, USA.
8
Department of Medicine, Weill Cornell Medical College, New York, NY 10065, USA.

Abstract

Approximately 30% of patients with follicular lymphoma (FL) transform to a more aggressive malignancy, most commonly diffuse large B cell lymphoma. Rarely, FL transformation results in clinical findings, histology, and immunophenotype reminiscent of B-lymphoblastic leukemia/lymphoma. We report the largest series to date with detailed analysis of 7 such patients. Lymphoblastic transformation occurred on average 2 years after initial diagnosis of FL. Five patients had prior intensive chemotherapy. Two patients developed mature high-grade lymphoma, followed by the lymphoblastic transformation. FL had BCL2 gene rearrangement in 4 of 5 cases. High-grade transformation was accompanied by MYC gene rearrangement (5 of 5). Transformation was characterized by expression of TdT, loss of Bcl6, variable loss of immunoglobulin light chain, and persistence of Pax-5, Bcl2, and CD10. Whole-exome sequencing in 1 case revealed presence of several actionable mutations (CD79B, CCND3, CDK12). FL, aggressive mature B cell lymphoma, and lymphoblastic transformation were clonally related in 6 evaluable cases. After transformation, survival ranged from 1 to 14 months. Four patients died of disease, 2 were in remission after stem cell transplant, and 1 was alive with disease.

KEYWORDS:

Double hit lymphoma; Follicular lymphoma; Lymphoblastic transformation; MYC rearrangement; TdT staining

PMID:
25529125
DOI:
10.1016/j.humpath.2014.10.021
[Indexed for MEDLINE]

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