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Diffuse Large B-Cell Lymphoma (DLBCL): Still a pandora’s box?
IAP 2014 ABSTRACTS
Hodgkin lymphomas (NL Harris) and lymphoid hyperplasias and their differential diagnosis with lymphomas (JKC Chan). The goal is to provide guidance related to practicing state-of-the-art lymphoma pathology in 2014.
Hematopathology: LC14-1 DIFFUSE LARGE B-CELL LYMPHOMA (DLBCL): STILL A PANDORA’S BOX? Stefano A. Pileri Chair of Pathology and Haematopathology Unit, Department of Experimental, Diagnostic and Specialty Medicine, Bologna University School of Medicine, Bologna, Italy DLBCL is the most common lymphoid malignancy worldwide. It includes tumors that cannot be morphologically classified (NOS) and clinic-pathologic variants characterized by specific features (e.g., age, primary site, immunodeficiency, and/or relationship with infectious agents).Nowadays, the cell of origin and complex cytogenetic alterations deserve great attention by affecting the disease behavior and therapeutic decisions. Gene expression profiling (GEP) studies have subdivided DLBCL/NOS into two main categories, related to germinal center and activated blood B-lymphocytes. The former has a more favorable course also in thepresent immunochemotherapy era. The attempts to surrogate GEP by immunohistochemistry ensued in algorithms, none of which as effective as GEP. More recently, an approach based on 15 genes and Nanostring technology has revealed excellent accuracy, inter-lab reproducibility and affordable costs. Equally important is the search for double/triple hits involving BCL2, MYC and BCL6, which can be detected by FISH and confer protection against apoptosis and high proliferative activity. The search for the corresponding proteins does not represent an absolute surrogate of cytogenetics. However, BCL2/MYC double expression heralds a poor prognosis. Further relevant parameters are CD30 expression and EBV infection. Finally, next generation sequencing has highlighted a series of mutations that might represent the rationale for innovative-targeted therapies.
S21
lymphocytic leukemia/small lymphocytic lymphoma (CLL), follicular lymphoma (FL), nodal marginal zone lymphoma (MZL), MALT lymphoma, hairy cell leukemia, and lymphoplasmacytic lymphoma (LPL). An entity that should be included in the differential diagnosis of lymphomas composed mainly by small lymphocytes but with a rather aggressive behaviour is mantle cell lymphoma (MCL). As a consequence of new and better available techniques in routine diagnosis, an increased recognition of early and precursor lesions of lymphoid neoplasms has emerged. This talk aims to review the morphological and phenotypic characteristics of the most frequent small B-cell lymphomas and expand on emerging concepts like, BCL2 negative FL, grading of FL, Pediatric FL, and indolent and cyclin D1 negative MCL, differential diagnosis of CD5þ lowgrade lymphomas and more.
Hematopathology: SC14-1 CHALLENGES IN BONE MARROW PATHOLOGY Robert P. Hasserjian1 and Sa A. Wang2 1Department of Pathology, Massachusetts General Hospital, Boston, MA, and 2Department of Hematopathology, UT MD Anderson Cancer Center, Houston, TX, USA
SMALL B-CELL LYMPHOMAS. DIAGNOSIS OF MALIGNANT LYMPHOMAS IN 2014
Bone marrow diagnosis presents unique challenges: distinction between benign and neoplastic conditions may be subtle and the pathologist must effectively incorporate information from several morphologic modalities (trephine biopsy, aspirate smear, peripheral blood smear) as well as a myriad of ancillary testing results. In this Short Course, several difficult diagnostic scenarios will be explored using actual clinical cases that illustrate key points in the differential diagnosis of bone marrow diseases. These will include hypocellular bone marrow (in which distinction between hypoplastic myelodysplastic syndrome and aplastic anemia may be difficult), fibrotic bone marrow, and marrow lymphoid infiltrates, with discussion of both B-cell and T-cell lymphomas. Hypercellular bone marrow raises distinct differential diagnoses depending on whether the patient is cytopenic or presents with elevated counts, and can reflect a myeloid neoplasm or various reactive conditions in either situation. An accurate diagnosis relies on understanding the spectrum of diseases that can produce specific patterns in the bone marrow and awareness of clues that help distinguish among the differential diagnostic possibilities. Depending on the clinical context, the pathology may variably weigh morphology, clinical information, and ancillary test results in arriving at the final diagnosis.
Leticia Quintanilla-Fend University of Tuebingen, Germany
Hematopathology: SS14-1
Hematopathology: LC14-1
The 2008 WHO classification of tumors of hematopoietic and lymphoid tissues has adopted consensus guidelines for the definition of well-defined entities. The major principle of the classification is the recognition of distinct diseases according to a combination of morphology, immunophenotype, genetic, molecular and clinical features. These disease entities are stratified according to their cell lineage and their derivation from precursor or mature lymphoid cells. Although the new 2008 WHO classification intentionally does not divide lymphomas by grade, traditionally mature B-cell lymphomas composed mainly of small lymphocytes have been called low-grade lymphomas. These small B-cell lymphomas include chronic
T AND NK CELL NEOPLASMS, LESSONS FOR THE WORLD FROM ASIA Shigeo Nakamura Nagoya University Hospital, Nagoya, Japan The WHO classification of lymphoid neoplasms was published in 2001, then was updated in 2008, and will be evolving in the future. This classification is based on the disease discovery and accurate description by pathologists beyond the heterogeneity in the geographical distribution of the disease entities. It is now
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
Hodgkin lymphomas (NL Harris) and lymphoid hyperplasias and their differential diagnosis with lymphomas (JKC Chan). The goal is to provide guidance related to practicing state-of-the-art lymphoma pathology in 2014.
Hematopathology: LC14-1 DIFFUSE LARGE B-CELL LYMPHOMA (DLBCL): STILL A PANDORA’S BOX? Stefano A. Pileri Chair of Pathology and Haematopathology Unit, Department of Experimental, Diagnostic and Specialty Medicine, Bologna University School of Medicine, Bologna, Italy DLBCL is the most common lymphoid malignancy worldwide. It includes tumors that cannot be morphologically classified (NOS) and clinic-pathologic variants characterized by specific features (e.g., age, primary site, immunodeficiency, and/or relationship with infectious agents).Nowadays, the cell of origin and complex cytogenetic alterations deserve great attention by affecting the disease behavior and therapeutic decisions. Gene expression profiling (GEP) studies have subdivided DLBCL/NOS into two main categories, related to germinal center and activated blood B-lymphocytes. The former has a more favorable course also in thepresent immunochemotherapy era. The attempts to surrogate GEP by immunohistochemistry ensued in algorithms, none of which as effective as GEP. More recently, an approach based on 15 genes and Nanostring technology has revealed excellent accuracy, inter-lab reproducibility and affordable costs. Equally important is the search for double/triple hits involving BCL2, MYC and BCL6, which can be detected by FISH and confer protection against apoptosis and high proliferative activity. The search for the corresponding proteins does not represent an absolute surrogate of cytogenetics. However, BCL2/MYC double expression heralds a poor prognosis. Further relevant parameters are CD30 expression and EBV infection. Finally, next generation sequencing has highlighted a series of mutations that might represent the rationale for innovative-targeted therapies.
S21
lymphocytic leukemia/small lymphocytic lymphoma (CLL), follicular lymphoma (FL), nodal marginal zone lymphoma (MZL), MALT lymphoma, hairy cell leukemia, and lymphoplasmacytic lymphoma (LPL). An entity that should be included in the differential diagnosis of lymphomas composed mainly by small lymphocytes but with a rather aggressive behaviour is mantle cell lymphoma (MCL). As a consequence of new and better available techniques in routine diagnosis, an increased recognition of early and precursor lesions of lymphoid neoplasms has emerged. This talk aims to review the morphological and phenotypic characteristics of the most frequent small B-cell lymphomas and expand on emerging concepts like, BCL2 negative FL, grading of FL, Pediatric FL, and indolent and cyclin D1 negative MCL, differential diagnosis of CD5þ lowgrade lymphomas and more.
Hematopathology: SC14-1 CHALLENGES IN BONE MARROW PATHOLOGY Robert P. Hasserjian1 and Sa A. Wang2 1Department of Pathology, Massachusetts General Hospital, Boston, MA, and 2Department of Hematopathology, UT MD Anderson Cancer Center, Houston, TX, USA
SMALL B-CELL LYMPHOMAS. DIAGNOSIS OF MALIGNANT LYMPHOMAS IN 2014
Bone marrow diagnosis presents unique challenges: distinction between benign and neoplastic conditions may be subtle and the pathologist must effectively incorporate information from several morphologic modalities (trephine biopsy, aspirate smear, peripheral blood smear) as well as a myriad of ancillary testing results. In this Short Course, several difficult diagnostic scenarios will be explored using actual clinical cases that illustrate key points in the differential diagnosis of bone marrow diseases. These will include hypocellular bone marrow (in which distinction between hypoplastic myelodysplastic syndrome and aplastic anemia may be difficult), fibrotic bone marrow, and marrow lymphoid infiltrates, with discussion of both B-cell and T-cell lymphomas. Hypercellular bone marrow raises distinct differential diagnoses depending on whether the patient is cytopenic or presents with elevated counts, and can reflect a myeloid neoplasm or various reactive conditions in either situation. An accurate diagnosis relies on understanding the spectrum of diseases that can produce specific patterns in the bone marrow and awareness of clues that help distinguish among the differential diagnostic possibilities. Depending on the clinical context, the pathology may variably weigh morphology, clinical information, and ancillary test results in arriving at the final diagnosis.
Leticia Quintanilla-Fend University of Tuebingen, Germany
Hematopathology: SS14-1
Hematopathology: LC14-1
The 2008 WHO classification of tumors of hematopoietic and lymphoid tissues has adopted consensus guidelines for the definition of well-defined entities. The major principle of the classification is the recognition of distinct diseases according to a combination of morphology, immunophenotype, genetic, molecular and clinical features. These disease entities are stratified according to their cell lineage and their derivation from precursor or mature lymphoid cells. Although the new 2008 WHO classification intentionally does not divide lymphomas by grade, traditionally mature B-cell lymphomas composed mainly of small lymphocytes have been called low-grade lymphomas. These small B-cell lymphomas include chronic
T AND NK CELL NEOPLASMS, LESSONS FOR THE WORLD FROM ASIA Shigeo Nakamura Nagoya University Hospital, Nagoya, Japan The WHO classification of lymphoid neoplasms was published in 2001, then was updated in 2008, and will be evolving in the future. This classification is based on the disease discovery and accurate description by pathologists beyond the heterogeneity in the geographical distribution of the disease entities. It is now
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.