Non-Hodgkin's Lymphoma

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Non-Hodgkin's Lymphoma
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• 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.

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Non-Hodgkin Lymphoma

• Two main types of Non-Hodgkins Lymphoma
• B-Cell and T-Cell Lymphomas
• B-Cell lymphomas (80)
• T-Cell lymphomas (15)

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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

• 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 ?d 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

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• 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

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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).
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Clinical Features

• Lymphadenopathy
• Cytopenias
• Systemic symptoms
• Hepatosplenomegaly
• Fever
• Night sweats

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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.

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B-Cell Cancers
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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

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• 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

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• Small cells lymphoma

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• 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

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• Follicular non-Hodgkin lymphoma (NHL) showing
  characteristic nodular pattern in a lymph node

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• 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.

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• 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.

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Burkitts Lymphoma

• African variety jaw tumor, strongly linked
  to Epstein-Barr 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.

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• 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.

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• Burkett lymphoma. Normal architecture is entirely
  replaced by lymphoma cells and evenly dispersed
  macrophages, starry sky