High-grade B cell lymphoma, unclassifiable, with blastoid features: an unusual morphological subgroup associated frequently with BCL2 and/or MYC gene rearrangements and a poor prognosis

Rashmi Kanagal-Shamanna, L Jeffrey Medeiros, Gary Lu, Sa A Wang, John T Manning, Pei Lin, Gerald M Penn, Ken H Young, M James You, Francisco Vega, Roland Bassett, Roberto N Miranda
Histopathology 2012, 61 (5): 945-54

AIMS: A subset of B cell lymphomas with blastoid features do not fit either as B lymphoblastic lymphoma/leukaemia or blastoid mantle cell lymphoma. Their classification is challenging, even with complete clinicopathological and genetic information. At a haematopathology workshop, experts had suggested the term 'high-grade B cell lymphoma, unclassifiable, with blastoid features', and recommended further studies.

METHODS AND RESULTS: We describe the clinicopathological, immunophenotypic and cytogenetic findings of 24 high-grade B cell lymphomas, unclassifiable, with blastoid features. Fifteen patients presented de novo and seven patients had a history of lymphoma. Twenty patients (83%) presented with nodal disease. All tumours expressed pan-B cell antigens and 17 (89%) of 19 tumours assessed had a germinal centre B cell immunophenotype. Ten (63%) of 16 tumours assessed by fluorescence in-situ hybridization (FISH) had MYC rearrangement, 13 of 18 (72%) carried IGH-BCL2 and nine of 15 (60%) had both (double-hit lymphoma). The median overall survival was 1.1 years. Using 2008 World Health Organization criteria, 15 cases were classified as B cell lymphoma, unclassifiable, with features intermediate between diffuse large B cell lymphoma (DLBCL) and Burkitt lymphoma, and nine as DLBCL, small centroblastic variant.

CONCLUSION: High-grade B cell lymphomas, unclassifiable, with blastoid features are clinically aggressive with poor survival. Most neoplasms have a germinal centre B cell phenotype. MYC rearrangements and IGH-BCL2 are common, and ~60% are double-hit lymphomas.


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