Lymphoma

Introduction

• The term lymphoma refers to cancers that originate in the

body's lymphatic tissues.
• Lymphatic tissues include the lymph nodes (also called
lymph glands), thymus, spleen, tonsils, adenoids, and bone
marrow, as well as the channels (called lymphatics or
lymph vessels) that connect them.
• Although many types of cancer eventually spread to parts
of the lymphatic system, lymphomas are distinct because
they actually originate there.
• Lymphoma is the third most common cancer in children.
Definition
• Lymphoma is a cancer that originates in the lymphocytes
(white blood cell) of the immune system of the body, a
malignant tumor may arise in any part of the lymphatic
system, like the lymph glands (or lymph nodes), spleen,
thymus, adenoids, etc. It originates only in the lymphatic
system.
• The etiology of Hodgkin lymphoma is believed to be
multifactorial.
• Siblings have a seven fold increase in the risk .
• A strong evidence for genetic susceptibility comes from a 100
fold increased risk in monozygotic twins compared with
dizygotic twins.
• Epidemiologic studies suggest link between Hodgkin
lymphoma and viral illnesses like Epstein-Barr virus (EBV).
• EBV viral DNA can be found in Reed-Sternberg cells
suggesting that monoclonal proliferation of the neoplastic
clone takes place after EBV infection.
Cause
• Unknown

Warning signs and risk factors

• Family history (though no hereditary pattern has been firmly
established)
• Autoimmune disease
• Receipt of an organ transplant
• Exposure to chemicals such as pesticides, fertilizers
• Infection with viruses such as Epstein-Barr, human T-
lymphotropic virus type 1, HIV, hepatitis C, or certain bacteria
such as Helicobacter pylori
Warning signs and risk factors for lymphoma are similar in
children and adolescents as well as in adults. Symptoms
include:
• Chills
• Swelling of the lymph nodes, which may or may not be
painless
• Fever
• Night sweats
• Unexplained weight loss
• Lack of energy
• Itchiness
 Types
Lymphomas are divided into two broad categories, depending on the
appearance of their cancerous cells.
These are known as Hodgkin's lymphoma and non-Hodgkin
lymphoma (NHL).
Together, they are the third most common type of cancer in children.
Lymphoma incorporates ICD-10 cancer codes C81 (Hodgkin’s
disease), C82 (follicular non-Hodgkin’s lymphoma), C83 (diffuse non-
Hodgkin’s lymphoma), C84 (peripheral and cutaneous T-cell
lymphomas) and C85–C86 (other and unspecified types of non-
Hodgkin’s lymphoma).
Hodgkin’s lymphoma
• It is defined by the presence of specific malignant
cells, called Reed-Sternberg cells, in the lymph nodes
or in some other lymphatic tissue.
• The malignancy originates in the lymphoid system &
primarily involves the lymph nodes.
• It predictably metastasizes to nonnodal or
extralymphatic sites, especially the spleen, liver, bone
marrow, lungs, & mediastinum, although no tissue is
exempt from involvement.
 Modified Ann Arbor staging for Hodgkin
lymphoma
Stage Involvement
I Single lymph node region (I) or one extralymphatic site (IE)*
II Two or more lymph node regions on same side of
diaphragm (II) or one or more lymph node regions on same side of diaphragm
plus local extralymphatic extension (IIE)*

III Lymph node regions on both sides of the diaphragm (III) which may be
accompanied by local extralymphatic extension (IIIE)*

IV Diffuse involvement of one or more extralymphatic*organ or sites

A No B symptoms
B Presence of at least one of the following B symptoms:
Unexplained weight loss >10% baseline during 6 months before staging
Recurrent unexplained fever >38°C
Recurrent night sweats

X Bulky tumor**
 Symptoms of Hodgkin’s lymphoma

• Painless cervical or supraclavicular lymphadenopathy; the nodes are

firm and rubbery in consistency .
• Cervical lymph nodes are the most frequent (80%)
• 50% patients may also have mediastinal adenopathy
• Less common, axillary or inguinal lymphadenopathy
• About 20-30% of children present with systemic 'B' symptoms, as
defined by the ann arbor staging criteria,
 Fever over 38°C
 Night sweats
 Unexplained weight loss of >10% body weight at presentation.
• The presence of unexplained pruritus should prompt complete
physical examination and chest radiography.
 Diagnosis

• Physical examination with measurement of lymph

nodes
• Complete hemogram with ESR, C reactive protein
• Liver and renal functions tests, alkaline phosphatase
• Lactate dehydrogenase
• Chest X-ray, mediastinal mass to thoracic cavity ratio
• CT scan of neck, chest and abdomen
• Bone marrow biopsy (all children except stages IA/IIA)
• Biopsy from lymph node or involved extranodal site
• Bone scan (if bone pain or raised serum
alkaline phosphatase)
• CT scan brain (if indicated)
• Cerebrospinal fluid examination (if indicated)
• PET-CT scan (higher sensitivity for stage and
residual mass than conventional imaging)
• Surgical staging with lymph node sampling
and lymphangiography (selected cases)
Therapeutic Management
• Radiation & Chemotherapy – Each may be used alone or
in combination based on the clinical staging.
• Radiation may involve only the involved field, an
extended field (involved areas plus adjacent nodes), or
total nodal irradiation, depending on the extent of
involvement.
• An effective combination of chemotherapy widely used
is MOPP (Mechlorethamine, Vincristine{Oncovin},
Procarbazine, Prednisolone) or ABVD (Adriamycin,
Bleomycin,Vinblastine, Dacarbazine).However, this
therapy combination has caused severe late effects,
especially secondary malignancies.
• COPP (Cyclophosphamide, Vincristine,
Prednisolone, Procarbazine) as a substitute for
MOPP have minimized late effects.
• Follow-up care of children no longer receiving
therapy is essential to identify relapse &
secondary cancers.
• In children with splenectomy, prophylactic
antibiotics are administered for an indefinite
period. Also, immunizations against pneumococci
& meningococci are recommended before the
splenectomy.
 Non-Hodgkin Lymphoma (NHL)
• Heterogeneous group of lymphoid neoplasms
derived from cells of the immune system.
• Most commonly occurs during the second
decade of life and occurs less frequently in
children less than three years of age.
• Characterized on basis of their t or b cell
nature.
• May follow previous chemotherapy for hodgkin
disease
• DNA repair deficiency syndromes (wiskott-aldrich
syndrome, x-linked a taxia-telangiectasia),
• Acquired immunodeficiency syndrome
• Organ transplantation (post-transplant
lymphoproliferative disease).
• Infection with malaria and eb virus are
considered risk factors for burkitt lymphoma.
Types of non-Hodgkin lymphoma
• Lymphoblastic lymphoma
• Burkitt lymphoma (small non-cleaved cell
lymphoma)
• Large cell lymphoma
Lymphoblastic lymphoma
• The cancer cells are very young lymphocytes
called lymphoblasts.
• They are the same cells as those seen in acute
lymphoblastic leukemia (ALL) in children.
• Most cases of lymphoblastic lymphoma
develop from T cells and are called precursor T-
lymphoblastic lymphomas. These lymphomas
often start in the thymus.
• Less often, this cancer may develop in the
tonsils, lymph nodes of the neck, or other
lymph nodes.
• It can spread very quickly to the bone marrow,
other lymph nodes, the surface of the brain,
and/or the membranes that surround the
lungs and heart.
Burkitt lymphoma
• Burkitt lymphoma, also known as small non-cleaved cell
lymphoma.
• In Burkitt lymphoma, a child will develop a large tumor in his
or her abdomen that can sometimes block the bowels
(intestines).
• This can cause belly pain, nausea, and vomiting.
• This lymphoma can also sometimes start in the neck or
tonsils, or rarely in other parts of the body.
• This lymphoma develops from B lymphocytes, and it is one
of the fastest growing cancers known.
• It may spread to other organs, including the surface of the
brain or inside the brain.
• Because of this, it must be diagnosed and treated quickly.
Large cell lymphomas
These lymphomas start in more mature forms of T
cells or B cells and can grow almost anywhere in the
body. There are 2 main subtypes of large cell
lymphoma.
• Anaplastic large cell lymphoma: It usually
develops from mature T cells. It may start in lymph
nodes in the neck or other areas, and may be
found in the skin, lungs, bone, digestive tract, or
other organs.
• Diffuse large B-cell lymphoma:
It starts in B cells. These lymphomas often grow
as large masses in the mediastinum (the space
between the lungs), in which case they are
referred to as primary mediastinal B-cell
lymphomas. But they are also sometimes found
in other areas such as lymphoid tissue in the
neck or abdomen, or in the bones.
Symptoms of Non Hodgkin’s lymphoma
– Swelling of glands in the neck, underarms, and groin
or above the collar bone.
– The lymphoma might grow in the abdomen, causing
blockages and abdominal pain.
– Sometimes cause the spleen to become enlarged
and press on the stomach & feel full after eating
only a small amount of food.
– Swelling in the chest, which causes difficulty
breathing.
– SVC syndrome: Lymphomas may push on the
superior vena cava (SVC), which can cause the blood
to back up in the veins. This lead to swelling in the
face, neck, arms, and upper chest. It can also cause
trouble breathing, as well as headaches, dizziness,
and a change in consciousness if it affects the brain.
– Fever, weight loss and night sweats might occur.
– May also block urine from leaving the kidney lead to
kidney problems, which may cause low urine output
– Headache, vision changes, facial numbness, and
trouble speaking- if lymphoma can spread to the
area around the brain and spinal cord.
– Itchy, red or purple lumps or nodules under the
skin.
– Easy bruising or bleeding (from lack of blood
platelets), frequent infections (from a lack of
white blood cells), or fatigue and pale skin (from
a lack of red blood cells) – if Lymphomas spread
to the bone marrow
 Diagnosis
Biopsy
• Fine needle aspiration (FNA) or core needle biopsy
• Excisional or incisional biopsy: a surgeon cuts through the skin
to remove either the entire node (excisional biopsy) or a small
part of a large tumor (incisional biopsy).

• Bone marrow aspiration and biopsy: These tests help

determine if a lymphoma has reached the bone marrow. The
biopsy samples are usually taken from the back of the pelvic
(hip) bones, although in some cases they may be taken from
the front of the hip bones or from other bones.
• Lumbar puncture (spinal tap): This test is used to look for
lymphoma cells in the cerebrospinal fluid
• Pleural or peritoneal fluid sampling
• Blood tests - the complete blood count (CBC) low blood cell
counts might mean that the lymphoma is growing in the
bone marrow and damaging new blood cell production.
• Blood levels of a chemical called LDH will often be
abnormally high in patients with fast-growing lymphomas
• Blood chemistry tests can help detect liver or kidney
problems caused by the spread of lymphoma cells or
certain chemotherapy drugs
Imaging tests to diagnose and stage non-Hodgkin lymphoma
• Chest x-ray
A chest x-ray may be done to look for enlarged lymph nodes
inside the chest.
• Computed tomography (CT or CAT) scan
CT scans can be used to look for enlarged lymph nodes or
other masses in the chest, abdomen, pelvis, head, and neck.
• Ultrasound (sonogram)
USG to look at lymph nodes near the surface of the body or
to look inside abdomen for enlarged lymph nodes or organs
such as the liver and spleen.
• Magnetic resonance imaging (MRI) scan
Provide detailed images of soft tissues in the body.
• Therapeutic Management
Aggressive use of irradiation & chemotherapy.
Several antineoplastic agents used in the treatment
of Non-Hodgkin Lymphoma include vincristine,
prednisolone, L-asparaginase, methotrexate, 6-
mercaptopurine, cytarabine, cyclophosphamide,
anthracyclines, & teniposide or etoposide.
Nursing Management
1. Prepare child & family for diagnostic & therapeutic procedures
2. Relieve pain
3. Prevent complications of Myelosuppression
- Infection
- Hemorrhage
- Anemia
4. Use precautions in administering and handling Chemotherapeutic
Agents
5. Manage problem of drug toxicity
- Nausea & Vomiting
- Anorexia
- Mucosal Ulceration
- Neuropathy
- Hemorrhagic Cystitis
- Alopecia
- Moon Face
- Mood change
6. Provide emotional support
Prognosis
• The prognosis is excellent for children with
localized disease, & long- term remissions are
possible in many patients, even in those with
disseminated disease. Because relapse after 2
years is rare, survival after 24 months is
considered a cure.
Difference between Hodgkin’s and non- Hodgink’s lymphoma
Prevalence
• Hodgkin's Disease is most prevalent among people between 15 and
35 and over 50. Older adults (age 60 and over) are more susceptible
to non-Hodgkin's Lymphoma.
Pathophysiology
• Hodgkin's Disease tends to arise from what is called the Epstein-Barr
virus and contain Reed-Sternberg Cells, which are necessary for
diagnosis. The malignant cells that form usually remain localized in
one lymph node or a surrounding chain in the neck, shoulder, and
chest. Non-Hodgkin's Lymphoma do not contain Reed-Sternberg
cells and tend to develop in peripheral lymph nodes and spread
throughout the body.
Signs and symptoms
• Usually the itchy, dry skin that occurs with Hodgkin's. The itching
sensation is usually more severe than a patch of dry skin. A patient
may feel the need to relieve the symptom by scratching at the
surface of the skin, sometimes trying to break the skin to relieve the
symptom. People with Hodgkin's disease often have fevers, night
sweats, and weight loss. Other signs and symptoms include fatigue,
itching, anemia, and an enlarged spleen.
• People who suffer from non-Hodgkin's Lymphoma can develop
fevers and night sweats as the disease progresses. More common
symptoms include abdominal pain, nausea, vomiting, cough, and
signs of central nervous system involvement (seizures and altered
mental status.)
Prognosis
• Hodgkin's Disease is one of the most curable
cancers with a 'cure' rate (5 years or more cancer
free) between 60 and 90%. However, the prognosis
for non-Hodgkin's Lymphoma can range from
excellent to poor depending on the grade of tumor
involved. Low grade tumors are most differentiated
(specialized in function) and least malignant, while
high grade tumors are least differentiated and the
most malignant.