This document provides information about lymphoma, including its definition, epidemiology, classification, causes, clinical manifestations, diagnosis, management, and prognosis. Lymphoma is cancer that develops in the lymphatic system. It can be classified as Hodgkin or non-Hodgkin lymphoma. The document outlines the specific objectives, types, diagnostic criteria, treatment approaches including chemotherapy regimens, complications, and prognostic factors for lymphoma.
2. z
General Objective
At the end of the session, participants will be
able to explain about lymphoma.
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3. z
Specific objectives
At the end of the session, participants will
be able to:
- Define lymphoma
- Explain the epidemiology of lymphomas
- Classify lymphoma
- List the causes of lymphoma
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4. z
Specific objectives
- Enlist the clinical manifestations of
lymphoma
- State the diagnostic criteria lymphoma
- Explain the management of lymphoma
- State the prognosis of lymphoma
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8. z
Lymphoma
Lymphoma is any cancer that starts in the
lymphatic system.
It can develop when a type of white blood
cell called a lymphocyte starts to grow in
an abnormal, uncontrolled way.
11. z
Hodgkin’s Lymphoma
Hodgkin’s disease (HD) is a
malignant disorder of
lymphoreticular system; a
lymphoreticular neoplasm
primarily of B cell lineage involving
lymph nodes.
12. z
Hodgkin disease:
Malignant process of lymphoreticular
system
6% of childhood cancer
5% of cancer in < 14 yr
15% in person 15-19 yr
Rare < 10 yr
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Epidemiology:
Bimodal incidence
Second peak after 50 y
Sex: Male > Female
Associated with specific HLA antigen
Infectious agents
Human herpes virus
6 Cytomegalovirus
Epstein – Barr virus
Immunodeficiency
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Clinical Manifestations
Lymphadenopathy cervical / supraclavicular
Painless, non tender, firm and rubbery
Hepatosplenomegaly
Cough, dyspnea, hypoxia
Pleural or pericardial effusion
Heptocellular dysfunction
Bone marrow infiltration (Anemia,
neutropenia, thrombocytopenia)
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Systemic Symptoms (B symptoms)
Important in staging
Unexplained fever > 390C
Weight loss > 10% in 3m
Drenching night sweats
Immune System abnormalities
Anergy to delayed-hypersensitivity skin test
Abnormal cellular immune response
Decreased CD4:CD8 ratio
Reduce natural killer cell cytotoxicity
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Ann Arbor Staging Classification for
Hodgkin Disease
▪ Stage I
Involvement of a single lymph node (1)
or of a single extra lymphatic site or
organ(1f)
• Stage II
Involvement of two or more lymph node
regions on the same side of the
diaphragm(II)
or localised involvement of an extra
lymphatic site or organ and one or more
lymph node regions on the same side of
the diaphragm (IIf)
22. z STAGING
Stage III
Involvement of lymph node regions on both
sides of the diaphragm (III) which may be
accompanied by the involvement of spleen
(IIIS) or by localized involvement of an extra
lymphatic site or organ ( IIIf) or both ( IIIsf)
Stage IV
Diffuse or disseminated involvement of one
or more extra lymphatic organs or tissues
with or without associated lymph node
involvement.
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Staging
The absence or presence of fever >
38C for three consecutive days ,
drenching night sweats , or
unexplained loss of > 10% body weight
in the 6 months preceding admission
are to be denoted in all cases by the
suffice letters A & B respectively.
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TREATMENT
Treatment depends on :
Stage of disease
Age at diagnosis
Presence / absence of B
symptoms
Presence of hilar
lymphadenopathy
Presence of bulky nodal disease
Current Treatment Regimen
Combined chemotherapy with or without low dose
involved field radiation therapy.
27. z LONG TERM
COMPLICATIONS
Secondary malignancy
Acute Myelogenous Leukemia
Non Hodgkin lymphoma
Carcinomas of breast , lungs & thyroid
Short stature
Hypothyroidism
Sterility
Dental caries
Sub clinical pulmonary dysfunction
Ischemic heart disease
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PROGNOSIS
Early Stage Disease
5 year survival ….95%
Advanced Stage Disease
5 year survival ….90%
Relapses common within first 3 years
from diagnosis
Relapses treated with Autologous Stem
Cell Transplantation
30. z EPIDEMIOLOGY
60% of all lymphomas in children
8-10% of all malignancies in children between 5-
19 yrs of age
Secondary causes of NHL include;
Inherited / acquired immune deficiencies
Viruses
HIV
EBV
Genetic Syndromes
Ataxia Telangiectasia
Bloom syndrome
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CLINICAL MANIFESTATIONS
Lymphadenopathy
Superior vena cava syndrome
Dyspnea
Abdominal Mass
Intestinal obstruction /
intussusception Ascites
Nasal Stuffiness
Earache
Tonsil enlargement
Localised bone involvement
Acute paraplegia secondary to CNS / spinal
cord compression
Tumor Lysis Syndrome
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Staging system for childhood
Non- Hodgkin lymphoma
Stage
I
II
Description
A single tumor (extranodal) or single
anatomic
area (nodal) with the exclusion of
mediastinum or abdomen
A single tumor (extranodal) with
regional node involvement
two or more nodes areas on the
same side of diaphragm
Two single (extranodal) tumors with or
without the regional node involvement
on same side of
diaphragm
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Stage II
A primary gastrointestinal tract tumor
usually in the ileocecal area, with or
without involvement of associated
mesenteric nodes, which may must
be grossly ( > 90%) resected
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Stage III
Two single tumors (extranodal)
on opposite side of the
diaphragm
Two more nodal areas above and
below the diaphragm
Any primary intarthoracic tumor
(mediastinal, pleural, or thymic)
Any extensive primary intra –
abdominal disease
IV
Any of the above, with initial
involvement of central nervous
system or bone marrow t time of
diagnosis
46. z
REFERENCES
• Ghai O, Paul V, Bagga A. Essential Pediatrics. 7th ed. CBS
Publisher & Distributers; 2008.
• Lymphoma in Children [Internet]. Stjude.org. 2021 [cited 16 May 2021]. Available
from:
https://www.stjude.org/disease/lymphoma.html#:~:text=Childhood%20lymphoma%2
0is%20rare.,under%20the%20age%20of%2014.
• Lymphoma Action | Lymphoma in children [Internet]. Lymphoma Action. 2021 [cited
16 May 2021]. Available from: https://lymphoma-action.org.uk/types-lymphoma-
lymphoma-children-and-young-people/lymphoma-children.
• https://doi.org/10.1093/ije/24.1.27
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Two broad categories
Hodgkin disease
Non- Hodgkin disease
and the lymphatic system has unique molecular, histologic, immune phenotypic and clinical features.
The lymphoreticular system consists of the spleen, lymphnodes, lymphatic vessels, thymus, and bone marrow
Bimodal: having 2 mode
4: 1 for 3-7 yr
3: 1 for 7-9 yr
1-3: 1 for > 10 yr
Human leukocyte Antigen
Disease below diaphragm is rare (only3%)
Drenching: soak
Anergy: absence of the normal immune response to a particular antigen or allergen.
A positron emission tomography (PET) scan is an imaging test that allows your doctor to check for diseases in your body. The scan uses a special dye containing radioactive tracers. These tracers are either swallowed, inhaled, or injected into a vein in your arm depending on what part of the body is being examined
fluorodeoxyglucose (FDG)-positron emission tomography (PET). The role of this procedure is to detect metabolically active .
Large (15-45 m) multiple / multilobulated nuclei.
Colonal in origin.
Arises from germinal center B cells.
Hilar lymphadenopathy is a common radiological finding associated with fungal infections, mycobacterial infections, and sarcoidosis. However, it is rarely seen in viral pneumonia.
Sometimes, despite the best care and significant progress made in treatment, cancer comes back. When this happens it is called a recurrence or relapse.
Ataxia Telangiectasia : Ataxia telangiectasia (A-T) is an autosomal recessive disorder primarily characterized by cerebellar degeneration, telangiectasia, immunodeficiency, cancer susceptibility and radiation sensitivity. A-T is often referred to as a genome instability or DNA damage response syndrome
Bloom syndrome (BSyn) is a rare genetic disorder characterized by short stature; a sun-sensitive, red rash that occurs primarily over the nose and cheeks; mild immune deficiency with increased susceptibility to infections; insulin resistance that resembles type 2 diabetes; and most importantly, a markedly increased
Superior vena cava syndrome (SVCS) happens when the superior vena cava is partially blocked or compressed.
Swelling of your face, neck, upper body, and arms
Trouble breathing or shortness of breath
Coughing
