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

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

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 1.1years. 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.

Original languageEnglish
Pages (from-to)945-954
Number of pages10
JournalHistopathology
Volume61
Issue number5
DOIs
StatePublished - Nov 1 2012
Externally publishedYes

Fingerprint

Gene Rearrangement
B-Cell Lymphoma
Non-Hodgkin's Lymphoma
B-Lymphocytes
Lymphoma
Germinal Center
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Neoplasms
Mantle-Cell Lymphoma
Burkitt Lymphoma
Survival
Lymphoma, Large B-Cell, Diffuse
B-Cell Chronic Lymphocytic Leukemia
Fluorescence In Situ Hybridization
Cytogenetics
Phenotype
Education
Antigens

Keywords

  • BCL2 rearrangement
  • Blastoid
  • Double-hit
  • High-grade B cell lymphoma unclassifiable
  • MYC rearrangement

ASJC Scopus subject areas

  • Histology
  • Pathology and Forensic Medicine

Cite this

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. / Kanagal-Shamanna, Rashmi; Medeiros, L. Jeffrey; Lu, Gary; Wang, Sa A.; Manning, John T.; Lin, Pei; Penn, Gerald M.; Young, Ken H.; You, M. James; Vega, Francisco; Bassett, Roland; Miranda, Roberto N.

In: Histopathology, Vol. 61, No. 5, 01.11.2012, p. 945-954.

Research output: Contribution to journalArticle

Kanagal-Shamanna, Rashmi ; Medeiros, L. Jeffrey ; Lu, Gary ; Wang, Sa A. ; Manning, John T. ; Lin, Pei ; Penn, Gerald M. ; Young, Ken H. ; You, M. James ; Vega, Francisco ; Bassett, Roland ; Miranda, Roberto N. / 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. In: Histopathology. 2012 ; Vol. 61, No. 5. pp. 945-954.
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abstract = "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 1.1years. 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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T1 - High-grade B cell lymphoma, unclassifiable, with blastoid features

T2 - An unusual morphological subgroup associated frequently with BCL2 and/or MYC gene rearrangements and a poor prognosis

AU - Kanagal-Shamanna, Rashmi

AU - Medeiros, L. Jeffrey

AU - Lu, Gary

AU - Wang, Sa A.

AU - Manning, John T.

AU - Lin, Pei

AU - Penn, Gerald M.

AU - Young, Ken H.

AU - You, M. James

AU - Vega, Francisco

AU - Bassett, Roland

AU - Miranda, Roberto N.

PY - 2012/11/1

Y1 - 2012/11/1

N2 - 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 1.1years. 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.

AB - 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 1.1years. 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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KW - Blastoid

KW - Double-hit

KW - High-grade B cell lymphoma unclassifiable

KW - MYC rearrangement

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