3. The lymph system and lymphoid tissue
• The lymph system (also known as the lymphatic system) is part of
the body’s immune system, which helps fight infections and some
other diseases. It also helps fluids move around within the body. The
lymph system is composed mainly of:
• Lymphoid tissue: includes the lymph nodes and related organs that
are part of the body’s immune and blood-forming systems
• Lymph: a clear fluid that travels through the lymph system, carrying
waste products and excess fluid from tissues, as well as lymphocytes
and other immune system cells
• Lymph vessels: small tubes, similar to blood vessels, through which
lymph travels to different parts of the lymph system
4. Lymphocytes: Lymphoid tissue is made up of cells
called lymphocytes, a type of white blood cell that fights infection.
There are 2 major types of lymphocytes: B lymphocytes (B cells) and
T lymphocytes (T cells). Normal T cells and B cells have different
jobs.
B lymphocytes: B cells help protect the body from germs (bacteria
and viruses). They do this by maturing into plasma cells, which make
antibodies (immune proteins). These antibodies attach to the germs,
marking them for destruction. Almost all cases of Hodgkin disease
start in B lymphocytes.
T lymphocytes: There are several types of T cells, and each has a
special job. Some T cells can directly destroy certain kinds of
bacteria or cells infected with viruses or fungi. Other types of T cells
play a role in either boosting or slowing the activity of other immune
system cells.
5. • Lymph nodes: Lymph nodes are small, bean-sized collections
of lymphocytes and other immune system cells found throughout
the body, including inside the chest, abdomen, and pelvis. They
can sometimes be felt under the skin in the neck, under the
arms, and in the groin. Lymph nodes are connected to each
other by a system of lymph vessels.
• Lymph nodes get bigger when they fight an infection. Lymph
nodes that grow in reaction to infection are called reactive nodes
or hyperplastic nodes. These often hurt when they are touched.
People with sore throats or colds might have enlarged neck
lymph nodes. An enlarged lymph node is not always a sign
of a serious problem, but it can be a sign of Hodgkin
disease.
6. • Bone marrow: The bone marrow is the spongy tissue inside
certain bones, which is where new white blood cells (including
some lymphocytes), red blood cells, and platelets are made.
• Thymus: The thymus is a small organ behind the upper part of
the breastbone and in front of the heart. It is important in the
development of T lymphocytes.
• Digestive tract: The back of the throat (adenoids and tonsils),
stomach, intestines, and many other organs also have lymphoid
tissue.
• Spleen: The spleen is an organ under the lower part of the rib
cage on the left side of the body. The spleen makes lymphocytes
and other immune system cells to help fight infection. It also
stores healthy blood cells and filters out damaged blood cells,
bacteria, and cell waste.
7. Hodgkin Lymphoma
• Hodgkin lymphoma is a cancer of the lymphoid system. The
lymphoid system is made up of various tissues and organs,
including the lymph nodes, tonsils, bone marrow, spleen, and
thymus. These organs produce, store and carry white blood
cells to fight infection and disease.
8.
9.
10. Lymphoma: Epidemiology
• 3rd most common cancer in children in the US (contrast: 2nd most
common cancer in Turkey)
– #1 leukemias
– #2 brain tumors
• Annual incidence of 13.2 per million children
• Major types include Hodgkin's and Non-Hodgkin's lymphoma
– 60% are NHL
– 40% Hodgkin’s Lymphoma
11. Types of Hodgkin disease
The 2 main types are:
1. Classic Hodgkin disease (which has several subtypes)
2. Nodular lymphocyte predominant Hodgkin disease
• All types of Hodgkin disease are malignant (cancerous)
because as they grow they may invade and destroy normal
tissue and spread to other tissues. There is no benign (non-
cancerous) form of Hodgkin disease
12. Classic Hodgkin disease
• Nodular sclerotic Hodgkin disease
• Mixed cellularity Hodgkin disease
• Lymphocytic rich Hodgkin disease
• Lymphocytic depleted Hodgkin disease
Reed–Sternberg cells (also known as lacunar histiocytes for
certain types) are different giant cells found with light
microscopy in biopsies from individuals with Hodgkin's
lymphoma (aka Hodgkin's disease; a type of lymphoma). 1229
13.
14. What causes it?
• Studies have suggested several infectious agents:
– EBV
– Human Herpes Virus
– CMV
• High EBV titers and the presence of EBV genomes in Reed-Sternberg
cells
• Surface markers suggest T cell or B cell lineage
17. Clinical Presentation
• Most common presentation in children is asymptomatic cervical
lymphadenopathy
– Painless, firm,not inflammatory
• Extension from one lymph node group to another
• 2/3 of patients have mediastinal adenopathy at presentation
– Cough or SOB if significant compression
• Infrequently presents as axillary or inguinal adenopathy
18. Extranodal Metastasis
• Hodgkin’s spreads through the lymphatic system
• Most frequent sites of extranodal involvement in decreasing order of
frequency
– bone marrow, bone,liver, lung, pericardium or pleura
• Paraneoplastic syndromes
– More likely seen in relapsing patients with widespread disease and NHL
– Hematologic, skin, nervous system, kidneys
19. Diagnostic Workup
• Tissue is needed for definitive histologic diagnosis
– Sample the node that is most accessible
• PE with careful attention and measurement of lymph nodes
• Labs
– CBC
– ESR
– LFT,Renal function
– Alkaline phosphatase; elevated
– (Immune response decreased,
20. Diagnostic & Staging Workup
• Cervical area US/CT/MR
• Thoracic imaging
– Chest Xray, CT scan of chest (ant/middle mediastinum)
• best visualization of lung parenchyma, pleura
• Abdominal imaging
– US/CT/MRI
– Lymphangiogram
• Most reliable method of detecting retroperitoneal lymph
nodes
• Rarely done in children
21. Diagnostic & Staging Workup
• Gallium Scan/ PET scan
– Search the body for other involvement
• Staging laparotomy
– Not used routinely any more
– Previously done routinely as part of staging
• Bone marrow biopsy
– Recommended for stage IIB or higher
– Bone marrow involvement at presentation is rare
• Bone scan
– Recommended for kids with bone pain, elevated alk phos, or extranodal disease
22. Sign and Symptoms
• Swollen lymph nodes: . The affected lymph nodes are usually
found in the neck or above the collarbone, and less
commonly under the arms or in the groin. The lymph nodes
are usually painless, firm, rubbery, and movable in the
surrounding tissue.
23. Conti…
• Poor appetite,
• Weight loss
• night sweats
• Fever
• fatigue may also be experienced by children with Hodgkin disease
• They are "systemic" symptoms with a more generalized affect on the
entire body.
• Itching (pruritus) is another symptom in patients with Hodgkin
disease. Itching can be mild or severe, and tends to occur more often
in patients with advanced disease.
24. Diagnosing Hodgkin Disease
• Evaluation begins with a complete medical history and physical
examination. Examine child's lymph nodes
and abdomen thoroughly.
• The presence of small, soft lymph nodes are less concerning
than nodes that have been increasing in size or are significantly
enlarged.
• An enlarged spleen or liver may indicate generalized disease.
• While a chest X-ray is usually performed prior to any biopsy, a
lymph node (or rarely other site) biopsy is needed to
diagnose the disease.
25. Diagnostic Findings
• Laboratory studies (do not establish stage of disease but are important)
– A complete blood count (CBC)
– Erythrocyte sedimentation rate (ESR)
– Kidney and liver function tests
• Imaging Studies (not all tests are needed for every patient)
– Chest X-ray
– Computer tomography (CT) scan
– Magnetic resonance imaging (MRI)
– Bone scan
– PET scan
– Bone marrow biopsy
26. Determining the Extent of Hodgkin Disease
• A standardized staging system (stages I-IV) developed for
Hodgkin disease is called the Ann Arbor staging system. The
stages are defined by the locations of lymph nodes in the
body that are infiltrated by the cancer. The higher the stage
number, the more the disease has spread throughout the
lymphoid system and into other parts of the body
27. Ann Arbor Staging Classification for Hodgkin
Disease
Stage I: Cancer is found in one location.
• Stage I: Cancer is found in a single lymph node region (most
commonly in the neck).
• Stage IE: Cancer is found in one organ or site outside the
lymph nodes.
28. Ann Arbor Staging Classification for Hodgkin
Disease
Stage II: Cancer is found in more than one location, but the
cancer sites are located either entirely above or below
the diaphragm (the muscle located below the lungs).
• Stage II: Cancer is limited to two or more lymph node regions
on the same side of the diaphragm.
• Stage IIE: Cancer is found in one or more lymph node
regions and a nearby organ or site on the same side of the
diaphragm.
29. Ann Arbor Staging Classification for Hodgkin
Disease
Stage III: Cancer is found both above and below the
diaphragm. Stage III: Cancer is limited to the lymph nodes,
but affected nodes are on both sides of the diaphragm.
• Stage IIIE: Cancer is found in lymph nodes on both sides of the
diaphragm, as well as in a nearby organ or site.
• Stage IIIS: Cancer is found in lymph nodes on both sides of the
diaphragm, as well as in the spleen.
• Stage IIIE+S: Cancer is found in lymph nodes on both sides of
the diaphragm, as well as in the spleen and a nearby organ or
site.
30. Ann Arbor Staging Classification for Hodgkin Disease
Stage IV: Cancer is located in sites usually outside of the
lymph nodes. When cancer is found in the lungs, liver,
bone or bone marrow it is classified as Stage IV.
31. Some Key Points
• At the time of diagnosis, approximately 60% of children have
stage I or II disease
• 30% have stage III disease
• 10% have stage IV disease
32. Causes of Hodgkin Disease
• Ionizing radiation
• Chemical exposures
• Epstein Barr Virus (EBV) the virus responsible for
mononucleosis, is seen in the tumors of 20-50% of Hodgkin
disease patients.
• Family history of Hodgkin disease: The risk of having Hodgkin
disease is two-to five-times greater for siblings, the likelihood that
a sibling will get the cancer remains extremely low. The clustering
of cases of Hodgkin disease within families and in certain ethnic
groups may suggest a genetic predisposition to the disease.
33. Conti…
• Immune system deficiencies: Hodgkin lymphoma is more
common in people whose immune system is weakened.
Immune deficiency can be caused by genetic conditions or
can be acquired. Therefore, people who have received organ
transplants and people with HIV infection are at increased
risk.
34. Determining Treatment and Likelihood of
Survival
• The type of treatment and likelihood of long-term survival
depend on many factors. Almost all children with stage I or II
disease can be cured; although about 1 in 10 children with early
stage disease may develop new disease and need additional
therapy. The recurrence of the disease decreases the chance of
long-term survival.
35. Conti…
• Advanced stage of disease (stage III or stage IV). About 8 in 10
children with stage III and IV disease can be cured.
• A large, cancerous lymph node or group of nodes, also referred
to as “tumor bulk."
• Histology –.
• Children with these findings may need more intense therapy
and radiation therapy to have the best chance of a cure.
37. Types
• There are four main types that occur in children:
• Burkitt Lymphoma (approximately 40% of childhood
lymphomas)
• Lymphoblastic lymphoma (approximately 30% of childhood
lymphomas)
• Diffuse large B-cell lymphoma (approximately 20% of
childhood lymphomas)
• Anaplastic large cell lymphoma (approximately 10 % of
childhood lymphomas)
38. Modified Ann Arbor Staging of
NHL
1255
The Non-Hodgkin’s Lymphoma Pathologic Classification Project. Cancer. 1982;49:2112.
Stage I
Stage II
Involvement of a single lymph node region
Involvement of 2 lymph node regions on the same side of the diaphragm
Stage III Involvement of lymph node regions on both sides
of the diaphragm
Stage IV Multifocal involvement of 1 extralymphatic sites
± associated lymph nodes or isolated extralymphatic
organ involvement with distant nodal involvement
40. Symptoms and Diagnosis
• Swelling of the lymph nodes in the neck, underarm, or groin.
Swelling is usually painless.
• Fever
• Weight loss
• Fatigue
• Night sweats
• Shortness of breath
41. A diagnosis is made after several tests are
conducted, including:
• Physical examination
• Chest X-ray
• Biopsy of a Lymph Node
• Bone Marrow Biopsy
• CT Scan
• Blood Tests
• Ultrasound
42. Treatments
• Typical NHL treatments include
• Chemotherapy
• Radiation
• Stem Cell Transplant
• Clinical Trials
• Monoclonal antibodies, such as Rituxan
43. Port – A- Cath or Central line
• Some children with medical problems need frequent IV
(intravenous) treatments. To avoid all those needle sticks,
the doctor may decide to insert a Port-a-Cath.
• Port-a-Caths may be used to give IV medicines, blood
transfusions, fluids, and nourishment, or draw blood samples.A
Port-a-Cath may also make it possible to do some of these
things at home.
44. Care for Port – A - Cath
• Preventing Bloodstream Infections. (If your client have a fever
over 100.5ºF or the skin around the central line becomes red or
painful, call the physician)
• Always keep the port-a-cath or IV tubing secured and away from
dirty surfaces.
• Store all supplies and medicines safely out of the reach of
children. Never reuse syringes, needles or any other supplies.
Do not use any supplies that have been dropped on the floor.
