Transcript Slide 1

Non-Hodgkin's Lymphoma
Non-Hodgkin lymphoma (NHL).
NHL is a heterogeneous group of lymphoproliferative
malignancies with differing patterns of behavior and responses to
treatment.
NHL usually originates in the lymphoid tissues and can spread to
other organs. However, unlike Hodgkin lymphoma, NHL is much
less predictable and has a far greater predilection to disseminate
to extranodal sites.
Non-Hodgkin Lymphoma
Two main types of Non-Hodgkin’s Lymphoma:
B-Cell and T-Cell Lymphomas
–B-Cell lymphomas (80%)
–T-Cell lymphomas (15%)
WHO/REAL Classification of Lymphoid Neoplasms
B-Cell Neoplasms
Precursor B-cell neoplasm
Precursor B-lymphoblastic leukemia/lymphoma
(precursor B-acute lymphoblastic leukemia)
Mature (peripheral) B-neoplasms
B-cell chronic lymphocytic leukemia / small lymphocytic
lymphoma
B-cell prolymphocytic leukemia
Lymphoplasmacytic lymphoma‡
Splenic marginal zone B-cell lymphoma
(+ villous lymphocytes)*
Hairy cell leukemia
Plasma cell myeloma/plasmacytoma
Extranodal marginal zone B-cell lymphoma of MALT type
Nodal marginal zone B-cell lymphoma
(+ monocytoid B cells)*
Follicular lymphoma
Mantle cell lymphoma
Diffuse large B-cell lymphoma
Mediastinal large B-cell lymphoma
Primary effusion lymphoma†
Burkitt’s lymphoma/Burkitt cell leukemia§
T and NK-Cell Neoplasms
Precursor T-cell neoplasm
Precursor T-lymphoblastic leukemia/lymphoma
(precursor T-acute lymphoblastic leukemia
‡ Formerly known as lymphoplasmacytoid lymphoma or
immunocytoma
II Entities formally grouped under the heading large granular
lymphocyte
leukemia of T- and NK-cell types
* Provisional entities in the REAL classification
Mature (peripheral) T neoplasms
T-cell chronic lymphocytic leukemia / small
lymphocytic lymphoma
T-cell prolymphocytic leukemia
T-cell granular lymphocytic leukemiaII
Aggressive NK leukemia
Adult T-cell lymphoma/leukemia (HTLV-1+)
Extranodal NK/T-cell lymphoma, nasal type#
Enteropathy-like T-cell lymphoma**
Hepatosplenic γδ T-cell lymphoma*
Subcutaneous panniculitis-like T-cell lymphoma*
Mycosis fungoides/Sézary syndrome
Anaplastic large cell lymphoma, T/null cell,
primary cutaneous type
Peripheral T-cell lymphoma, not otherwise characterized
Angioimmunoblastic T-cell lymphoma
Anaplastic large cell lymphoma, T/null cell,
primary systemic type
Predisposing factors of NHL.
1- Chromosomal translocations and molecular rearrangements.
2- Environmental factors .
3- Chemicals that have been linked to development of NHL include
a variety of pesticides and herbicides
4- chemotherapy and/or radiation therapy are at increased risk
of developing NHL.
5- Viruses have been implicated in the pathogenesis of NHL,
including the Epstein-Barr virus in Burkett lymphoma
6- Immunodeficiency states that seem to predispose to NHL include
congenital immunodeficiency states (e.g., ataxia telangiectasia,
Wiskott-Aldrich syndrome,
7- Increased incidence of GI lymphomas is observed in patients with
celiac sprue and inflammatory bowel disease
Staging of NHL:
The stage of NHL describes how many groups of lymph nodes are affected, where they are
in the body, and whether other organs such as the bone marrow or liver are involved.
Stage 1 The lymphoma is only in one group of lymph nodes, in one particular area of the
body.
Stage 2 More than one group of lymph nodes is affected, but all the affected nodes are
contained within either the upper half or lower half of the body. The upper half
of the body is above the sheet of muscle underneath the lungs (the diaphragm),
and the lower half is below the diaphragm.
Stage 3 Lymphoma is present in lymph nodes in both the upper and the lower parts of the
body (i.e. in lymph nodes both above and below the diaphragm).
Stage 4 The lymphoma has spread beyond lymph nodes to other lymphatic organs – for
example, to sites such as the bone marrow, liver or lungs.
The stage usually includes the letter A or B, which describes whether the B symptoms are
present or not (e.g. stage 2B).
Clinical Features
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Lymphadenopathy
Cytopenias
Systemic symptoms
Hepatosplenomegaly
Fever
Night sweats
B-Cell Lymphoma (80%)
B-Cells help make antibodies, which are proteins that attach to and help
destroy antigens.
Lymphomas are caused when a mutation arises during the B-cell life cycle.
Various different lymphomas can occur during several different stages of
the cycle
Follicular lymphoma, which is a type of B-cell lymphoma is caused by a
gene translocation which results in an over expressed gene called BCL-2,
which blocks apoptosis.
B-Cell Cancers
T-Cell Lymphoma (15%)
The T-cells are born from stem cells, similar to that of B-cells, but mature
in the thymus.
They help the immune system work in a coordinated fashion.
These types of lymphomas are categorized by how the cell is affected.
Anaplastic Large cell Lymphoma, t-cell lymphoma caused by a gene
translocation in chromosome 5
Small lymphocytic lymphoma
(B-SLL) represents only about 4% of adult NHL.
It usually occurs in adults with generalized lymph node
disease (lymphadenopathy).
This disease manifests more often as B-cell chronic
lymphocytic leukemia (B-CLL) than as B-SLL
Small cells lymphoma
Follicular lymphoma
It represents 70% of all the low-grade B-cell lymphomas.
It is the second most common lymphoma in the United States
and comprises 20% of all NHL.
Men and women are affected in nearly equal numbers, and the
average age of onset is 55 years.
Most patients tend to have widespread, advanced-stage disease
at diagnosis. Despite this fact, the disease is slow to progress
Follicular non-Hodgkin lymphoma (NHL) showing characteristic
nodular pattern in a lymph node
Diffuse large cell lymphoma
It is the most common type of lymphoma, accounting for
approximately one-third of all NHLs, regardless of grade.
Patients typically report a history of a rapidly enlarging,
symptomatic mass.
Approximately two-thirds of all patients have widespread
disease at diagnosis.
Diffuse large B-cell non-Hodgkin lymphoma (NHL). Large cells with
abundant cytoplasm and large round-ovoid nuclei with thick nuclear
membrane and multiple prominent nucleoli.
Burkitt’s Lymphoma
African variety: jaw tumor, strongly linked to EpsteinBarr Virus infection.
In U.S., about 50% EBV infection.
May present as abdominal mass.
Most rapidly growing human tumor.
Typical chromosome abnormality: c-myc oncogene
linked to one of the immunoglobulin genes.
Burkitt's lymphoma
tends to occur most often in children and in adults with
compromised immune systems.
There are three distinct forms of this disease: endemic, sporadic,
and immunodeficient.
Endemic Burkitt's lymphoma is seen among the young of
equatorial Africa.
Sporadic Burkitt's lymphoma often involves the abdomen, and it
spreads to the bone marrow in about 20% of patients.
Burkett lymphoma. Normal architecture is entirely replaced by
lymphoma cells and evenly dispersed macrophages, starry sky