diffuse large b cell lymphoma | Diffuse large B-cell lymphoma

Diffuse large B-cell lymphoma


Summary
Lymphomas diffuse large cell B (DLBCL or DLBCL) are the most common form of malignant lymphomas not hodgkiniens. They account for about a third of all lymphomas. This entity includes aggressive forms, characterized both by a spontaneous evolution swift and harsh, and the possibility of healing after treatment. They are varied histopathology entities. In addition to this histological variety, the molecular biology of genes expressed in the DLBCL study to extract and isolate specific to subgroups of DLBCL from gene expression profiles. Therapeutic strategies developed so far are designed to be applicable to a homogeneous entity, although recently emerging specific therapeutic trials tested in a situation of relapse [1].

Diffuse Lymphoma large cell B 5
C. Thieblemont, M.D. Venon, v. Benet, J. Brière, N. Mounier
C. Thieblemont)
)-Hemato-Oncology Department, hospital Saint-Louis, 1, avenue Claude
Vellefaux, 75010 Paris
E-mail: catherine.thieblemont@sls.aphp.fr
C. Thieblemont, M. D. Venon - AP - HP, St. Louis hospital, Department of Hematology-Oncology,
University Paris VII-Diderot, INSERM U728
V. Benet, J. Brière - AP - HP, St. Louis hospital, pathological anatomy service, University
Paris VII-Diderot, INSERM U728
N. Mounier - CHU of Larchet, Hematology service, Nice
Under the direction of Pauline Brice and Catherine Thieblemont, therapeutic news in the
lymphomas.
ISBN: 978-2-8178-0370-8, © Springer-Verlag Paris 2013
Introduction
Lymphomas diffuse large cell B (DLBCL or DLBCL) are the
the most common form of malignant lymphomas not hodgkiniens. They represent
about a third of all lymphomas. This entity includes the
aggressive forms, characterized by a quick spontaneous evolution and
severe, and the possibility of healing after treatment. Histopathology entities
varied. In addition to this histological variety, the study in biology
Molecular genes expressed in the DLBCL enabled to extract and isolate
specific subgroups of DLBCL from gene expression profiles. Them
therapeutic strategies developed so far are designed to be
applicable to a homogeneous, even if entity recently emerging tests
tested in a situation of relapse [1] specific Therapeutics.

66 therapeutic news in lymphomas
-scalable variants on the one hand: this Lymphoma may be primitive (majority
cases), but can also develop during evolution of lymphoma
said such as follicular Lymphoma, marginal zone Lymphoma, indolent
the Lymphocytic / chronic lymphoid leukemia (Richter syndrome). In
General these less aggressive hemopathies are already known, or even already processed.
but sometimes the diagnosis of DLBCL linked to the transformation of a
indolent Lymphoma be established immediately in a "naïve" when patient is
found two cell components on the pathological piece;

-morphological variants on the other hand: the who classification of
lymphomas was updated in 2008 recognizes 11 subtypes [2], divided according to the
clinical, morphological, immunological and genetic features
(table I). There are three common morphological variants: centroblastique,.
immunoblastique and anaplastic, the immunoblastique being more
poor prognosis [3]. At the phenotypic level, more than 95% of the DLBCL
Express pan - B, such as the CD20 marker. Some markers as
FoxP1 and cyclin E are constantly associated with a bad survival [4]. The
prognostic value of BCL2 has been removed by the introduction of rituximab [5].
Table I - The DLBCL, with description of variants, subgroups and subtypes,
According to Jaffe and al.
Lymphomas diffuse large cell B 'unspecified '.
They represent the largest part of the DLBCL.
-3 common morphological variants: centroblastique, immunoblastique and
anaplastic, the immunoblastique being worse prognosis.
-Other rare morphological variants.
-Molecular subgroups (they will be detailed later): type Center
germinative 'GC-like' and type 'ABC-like' active B cell lymphoma
primitive of the mediastinum.
-Immunohistochemical subgroups: DLBCL CD5 +, GC-like or non-GC-like.
Lymphomas of specific subtypes
-Primitive nervous system Lymphoma central.
-Lymphoma T-cell rich B: infiltration by a small number of large
B cells associated with a reaction rate of T lymphocytes and histiocytes, which
is a problem of differential diagnosis with Hodgkin's disease.
-Primitive Cutaneous Lymphoma.
-Lymphoma. EBV + old.
Other large B-cell lymphomas
-Diffuse Lymphoma large cell B of the mediastinum (thymic): it has its origin
intrathymiques B cells. This Lymphoma affects the young form of subjects
anterior Mediastinal tumor. It has one distinct molecular signature of others
lymphomas