Transcript hodgkin disease

LYMPHOMAS
By
DR : Ramy A. Samy
INTRODUCTION
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Neoplastic lymphoid proliferation
Two types – Hodgkins & Non-Hodgkins.
Fever, lymphadenopathy,
Immunodeficiency / autoimmune syndrome
Viral, genetic, unknown etiology.
Lack of programmed cell death - Apoptosis
• Lymphomas are a
cancer of the
lymphatic system
– Lymphatic vessels
– Lymph nodes
(underarms, groin,
neck, spleen, tonsils
and bone marrow)
• The Lymphatic system is our bodies main fight
against infection
• Lymphocytes (B-cell and T-cell)
– Carried through our lymphatic system and help
our bodies fight infection
– Lymphocytes are carried through the lymph
vessels as well as the blood stream, so cancer can
start in nodes and spread anywhere throughout
the body.
Lymphoma
Row of
enlarged
lymph
nodes
Lymphoma - Gross
Lymphoma Intestine
Lymphoma Intestine
Mediastinal Lymphnodes-NHL
NON-HODGKIN LYMPHOMAS
• Essentials of Diagnosis
• Often
presents
with
painless
lymphadenopathy.
• Pathologic diagnosis of lymphoma is made by
pathologic examination of tissue.
General Considerations
• The non-Hodgkin lymphomas are
heterogeneous group of cancers
lymphocytes.
a
of
• The disorders vary in clinical presentation and
course from indolent to rapidly progressive.
Associated with Non-H lymphoma
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SLE
Celiac Disease
AIDS
Organ transplant patients
Rheumatoid Arthritis
• Classification of the lymphomas is a
controversial area still undergoing evolution
• Eighty-five percent (85 %)of non-Hodgkin
lymphomas are B-cell, 15% are T-cell.
• These lymphomas are either indolent (low
grade) and incurable or aggressive
(intermediate and high grade) and potentially
curable.
Classification
• Usually classified by how the cells look under
a microscope and how quickly they grow and
spread
– Aggressive lymphomas (high-grade lymphomas)
– Indolent Lymphomas (low-grade lymphomas)
Classification of Malignant Lymphomas
• Low Grade: small lymphoid cells, nodular
growth
• Intermediate Grade: large cells, follicular and
diffuse patterns
• High Grade: immunoblastic, lymphoblastic,
Burkitt’s disease
• T-cell lymphomas: peripheral, cutaneous
World Health Organization proposed
classification of non-Hodgkin lymphomas.
 Precursor B
 B cell lymphoblastic lymphoma
 Mature B
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1)
2)
3)
Diffuse large B cell lymphoma
Mediastinal large B cell lymphoma
Follicular center cell lymphoma
Small lymphocytic lymphoma
Lymphoplasmacytic lymphoma
Mantle cell lymphoma Burkitt lymphoma
Marginal zone lymphoma
MALT type
Nodal type
Splenic type
NHL- Histologic types
Diffuse - & - Follicular
NHL- Histologic types
Small – Intermed. – Large
Precursor T
T cell lymphoblastic lymphoma
Mature T (and NK cell)
Anaplastic T cell lymphoma
Peripheral T cell lymphoma
Cutaneous
T-cell
lymphoma
fungoides)
(mycosis
Lymphoma spread to Spleen
Clinical Findings
• Symptoms and Signs
• Patients with indolent lymphomas usually
present with :
 painless lymphadenopathy, which may be
isolated or widespread.
 lymph nodes may be peripherally or centrally
 The indolent lymphomas are usually disseminated
at the time of diagnosis, and bone marrow
involvement is frequent.
 constitutional symptoms such as fever, drenching
night sweats, and weight loss (referred to as "B"
symptoms).
• On examination,
• lymphadenopathy may be isolated, or
extranodal
sites
of
disease
(skin,
gastrointestinal tract, liver) may be found.
• Patients with Burkitt lymphoma are noted to
have abdominal pain or abdominal fullness
because of the predilection of the disease for
the abdomen.
Laboratory Findings
• The peripheral blood is usually normal even
with extensive bone marrow involvement by
lymphoma.
• Circulating lymphoma cells in the blood is
uncommon.
• Bone marrow involvement is manifested as
paratrabecular monoclonal lymphoid aggregates
• In some high-grade lymphomas, the meninges
are involved and malignant cells are found with
cerebrospinal fluid cytology.
• The chest radiograph may show a mediastinal
mass in some lymphomas.
• The serum LDH has been shown to be a useful
prognostic marker and is now incorporated in risk
stratification of treatment.
• The diagnosis of lymphoma is made by tissue
biopsy.
Treatment
• The treatment of indolent lymphoma depends
on the stage of disease and the clinical status
of the patient.
• A small number of patients have limited
disease with only one abnormal lymph node
group and may be treated with localized
irradiation with curative intent.
• Most patients with indolent lymphoma have
disseminated disease at the time of diagnosis
and are not curable.
• When the disease is not bulky and the patient
not symptomatic, no initial therapy is required.
• Some patients
remissions.
will
have
spontaneous
• Treatment with the anti-CD20 monoclonal
antibody rituximab (intravenous) is a
commonly used treatment because of its very
low toxicity and avoidance of chemotherapy.
• Combinations of rituximab with chemotherapy
may also be used.
• Common chemotherapy regimens include
fludarabine;
the
combination
of
cyclophosphamide,
vincristine,
and
prednisone (R-CVP); and cyclophosphamide,
doxorubicin, vincristine, prednisone (R-CHOP).
Prognosis
• The median survival of patients with indolent
lymphomas is 10–15 years..
HODGKIN DISEASE
Essentials of Diagnosis :
Painless lymphadenopathy.
Constitutional symptoms may or may not be
present.
Pathologic diagnosis by lymph node biopsy.
General Considerations
• Hodgkin disease is a group of cancers
characterized by Reed–Sternberg cells in an
appropriate reactive cellular background
• The malignant cell is derived from B
lymphocytes of germinal center origin.
Reed-Sternberg Cell
Hodgkins lymphoma
Hodgkins lymphoma cells
Clinical Findings
• There is a bimodal age distribution,
• with one peak in the 20s and a second over age
50 years.
• painless mass, commonly in the neck.
• constitutional symptoms such as fever, weight
loss, or drenching night sweats, or generalized
pruritus.
• An unusual symptom of Hodgkin disease is pain
in an involved lymph node following alcohol
ingestion.
• An important feature of Hodgkin disease is its
tendency to arise within single lymph node
areas and spread in an orderly fashion to
contiguous areas of lymph nodes.
• Only late in the course of the disease will
vascular invasion lead to widespread
hematogenous dissemination.
Hodgkin disease is divided into several subtypes:
 classic Hodgkin (nodular sclerosis, mixed
cellularity, and lymphocyte depleted) and
 non-classic Hodgkin (lymphocyte predominant).
• Hodgkin disease should be distinguished
pathologically from other malignant lymphomas
and may occasionally be confused with reactive
lymph nodes seen in infectious mononucleosis,
cat-scratch disease, or drug reactions (eg,
phenytoin).
STAGING
 The staging nomenclature (Ann Arbor) is as follows:
I. stage I, one lymph node region involved;
II. stage II, involvement of two or more lymph node
regions on one side of the diaphragm;
III. stage III, lymph node regions involved on both sides
of the diaphragm; and
IV. stage IV, disseminated disease with extranodal
involvement.
• In addition, patients’ stages are qualified as "A" if they
lack constitutional symptoms or as "B" if 10% weight loss
over 6 months, fever, or drenching night sweats are
present.
Staging of Lymphoma
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