LYMPHOMA

1. . Lymphomas
• Lymphomas account for 10–15% of childhood cancers.
• Lymphomas, are cancer of called lymphocytes, classified as
Hodgkin’s and non-Hodgkin’s depending on the immune cell
type, are characterized by abnormal proliferation of
lymphocytes.
• The two forms of lymphoma are:
i. Hodgkin ’s disease
ii. Non -Hodgkin ’s lymphoma

2. Hodgkin ’s disease:
• Is a cancer of the antigen-processing cells found within the
lymph nodes or spleen and sometimes tonsils and bone
marrow
• It makes up about 11% of all lymphomas.
• It is a malignant condition characterized by proliferation of
abnormal giant, multinucleated cells, called Reed-Sternberg
cells, which are located in lymph nodes.
• It’s a malignancy of the lymph nodes that originates in a
single lymph node or a chain of nodes.

3. Cont’d
• The disease has a bimodal age-specific incidence, occurring most
frequently in persons from 15 to 35 years of age and above 50 years of
age.
• In adults, it is twice as prevalent in men as in women.
• Each year, approximately 9060 new cases of Hodgkin’s lymphoma are
diagnosed and approximately 1200 deaths occur.
• However, long-term survival exceeds 85% for all stages
• Metastasis occurs to other, adjacent lymph structures and eventually
invades nonlymphoid tissue.

4. Etiology & Pathophysiology of Hodgkin’s disease
• The cause of Hodgkin’s lymphoma remains unknown,
• several key factors are thought to play a role in its development.
• The main interacting factors include:
• infection with Epstein-Barr virus (EBV),
• genetic predisposition, and
• exposure to occupational toxins.
• The incidence of Hodgkin’s lymphoma is increased among patients who have HIV
infection
Clinical features.
Most children with Hodgkin’s disease present with painless lymphadenopathy

5. CONT’D
• Normally, the lymph nodes are composed of connective tissues that
surround a fine mesh of reticular fibers and cells.
• In Hodgkin’s lymphoma the normal structure of lymph nodes is destroyed
by hyperplasia of monocytes and macrophages.
• The main diagnostic feature of Hodgkin’s lymphoma is the presence of
Reed-Sternberg cells in lymph node biopsy specimens.
• The disease is believed to arise in a single location (it originates in cervical
lymph nodes in 70% of patients) and then spreads along adjacent
lymphatics.
• However, in recurrent disease, it may be more diffuse and not necessarily
contiguous.
• It eventually infiltrates other organs, especially lungs, spleen, and liver.
When the disease begins above the diaphragm, it remains confined to
lymph nodes for a variable time.
• Disease originating below the diaphragm frequently spreads to
extralymphoid sites such as the liver.

6. Hodgkin’s lymphoma (stage IIA). This patient
has enlargement of the cervical lymph nodes

7. Main areas of lymphadenopathy and organ
involvement in Hodgkin’s disease

8. Cont’d
• The disease predictably metastasizes to nonnodal or extralymphatic
sites, especially the spleen, liver, bone marrow, lungs, and
mediastinum.
• Hodgkin’s disease is characterized by the presence of Reed-Sternberg
cells noted in a lymph node biopsy specimen.
• Peak incidence is in mid-adolescence.
• Possible causes include viral infections and previous exposure to
alkylating chemical agents.
• The prognosis is excellent, with long-term survival rates depending on
the stage of the disease.
• The primary treatment modalities are radiation and chemotherapy;
each may be used alone or in combination, depending on the clinical
stage of the disease.

9. Clin ical Fe atu re Hodgkin’s Disease Non-Hodgkin ’s
Lymphom a
Symptom onset
Common location
Systemic
symptoms(fever, night
sweets and weight loss)
Abdominal
findings(abdominal pain,
abd mass, obstruction)
Painless adenopathy
SVC syndrome
Airway compression
Slow, indolent
Cervical and
supraclavicular nodes
Relatively common
(30%)
Rare
common
rare
rare
Rapid
Abdominal, mediastinal,
and supraclavicular
nodes
Uncommon
common
common
common
common

12. Assessment: signs and symptoms
• Painless enlargement of lymph nodes
• Enlarged, firm, non-tender, movable nodes in the supraclavicular
area; in children, the “sentinel” node located near the left clavicle
may be the first enlarged node
• Nonproductive cough as a result of mediastinal lymphadenopathy
• Abdominal pain as a result of enlarged retroperitoneal nodes
• Advanced lymph node and extralymphatic involvement that may
cause systemic symptoms, such as a low-grade or intermittent fever,
anorexia, nausea, weight loss, night sweats, and pruritus
• Positive biopsy specimen of a lymph node (presence of Reed-
Sternberg cells) and positive bone marrow biopsy specimen
• Computed tomography scan of the liver, spleen, and bone marrow
may be done to detect metastasis.

13. CLINICAL MANIFESTATION
• The onset of symptoms in Hodgkin’s lymphoma is usually insidious.
• The initial development is most often enlargement of cervical, axillary,
or inguinal lymph nodes; a mediastinal node mass is the second most
common location. This lymphadenopathy affects discrete nodes that
remain movable and nontender.
• The enlarged nodes are not painful unless they exert pressure on
adjacent nerves.

14. CONT’D
• The patient may notice weight loss, fatigue, weakness, fever, chills,
tachycardia, or night sweats.
• A group of initial findings including fever (in excess of 100.4° F [38°
C]), drenching night sweats, and weight loss (exceeding 10% in 6
months) are termed B symptoms and correlate with a worse
prognosis.
• After the ingestion of even small amounts of alcohol, individuals with
Hodgkin’s lymphoma may complain of a rapid onset of pain at the site
of disease. The cause for the alcohol-induced pain is unknown.
• Generalized pruritus without skin lesions may develop.
• Cough, dyspnea, stridor, and dysphagia may all reflect mediastinal
node involvement.

15. CONT’D
• In more advanced disease, there may be hepatomegaly and
splenomegaly.
• Anemia results from increased destruction and decreased production
of erythrocytes.
• Other physical signs vary depending on where the disease is located.
For example, intrathoracic involvement may lead to superior vena
cava syndrome, enlarged retroperitoneal nodes may cause palpable
abdominal masses or interfere with renal function, jaundice may
occur from liver involvement, spinal cord compression leading to
paraplegia may occur with extradural involvement, and bone pain
occurs as a result of bone involvement.

16. Diagnosis
• The basis of diagnosis is histologic review of tissue obtained by
lymphnode biopsy. The hallmark histologic feature is the Reed
Sternberg cell, a large multinucleated cell with abundant cytoplasm.
Staging
• Classification by the Ann Arbor system is the basis for treatment and
provides prognostic information. There are four basic stages, and each
stage is sub-classifie d into “A” or “B,” reflecting clinical symptoms.
• A - refers to lack of systemic symptoms.
• B - refers to the presence of systemic symptoms, such as fever, night sweats,
or > 10% weight loss

17. Stages
• Stage I: involvement of a single lymph node or
extralymphatic site
• Stage II: involvement of two or more lymph node regions on
the sameside of the diaphragm, or extension to an
extralymphatic site and one or more lymph node regions on
the same side of the diaphragm
• Stage III: involvement of lymph nodes on both sides of the
diaphragm (in this case, the spleen is considered a
lymphnode)
• Stage IV: diffuse or disseminated involvement of one or more
extralymphatic organs or tissues

18. . Management
• Treatment is based on the child’s age, disease stage, and
• tumor burden. Treatment most commonly includes chemotherapy and
radiation therapy. Late complications of therapy include the following:
• Growth retardation as a result of radiation therapy
• Secondary malignancies, including breast cancer, AML, and
nonHodgkin’s lymphoma
• Hypothyroidism (10–20%)
• Male sterility (very common)

19. Interventions
• For early stages without mediastinal node involvement, the treatment of
choice is extensive external radiation of the involved lymph node regions.
• With more extensive disease, radiation and multidrug chemotherapy are
used.
• Monitor for medication-induced pancytopenia and an abnormal
depression of all cellular components of the blood, which increases the risk
for infection, bleeding, and anemia.
• Monitor for signs of infection and bleeding.
• Protect the child from infection.
• Monitor for adverse effects related to chemotherapy or radiation; the most
common adverse effect of extensive irradiation is malaise, which can be
difficult for older children and adolescents to tolerate physically and
psychologically
• Monitor for nausea and vomiting, and administer antiemetics as prescribed

20. Non-Hodgkin’s lymphoma
• is a very aggressive cancer and is 1.5 times more
common than Hodgkin’s disease.
Epidemiology
• Male predominance
• Associated with immunodeficiency states, such as
HIV, Wiskott Aldrich syndrome, ataxia
telangiectasia syndrome, and prior EBV infection.
Increasing incidence after 5 years of age

21. Non-Hodgkin’s lymphomas (NHLs)
• Non-Hodgkin’s lymphomas (NHLs) are a heterogeneous group of
malignant neoplasms of primarily B-, T-, or natural killer (NK) cell
origin affecting all ages.
• B-cell lymphomas constitute about 85% of all NHLs.
• They are categorized by the level of differentiation, cell of origin, and
rate of cellular proliferation.
• A variety of clinical presentations and courses are recognized, from
indolent (slowly developing) to rapidly progressive disease.
• NHL is the most commonly occurring hematologic cancer and the fifth
leading cause of cancer death.
• Each year approximately 66,360 new cases of NHL are diagnosed and
approximately 19,320 deaths occur

22. Etiology and Pathophysiology
• The cause of NHL is usually unknown.
• NHLs may result from chromosomal translocations, infections,
environmental factors, and immunodeficiency states.
• Chromosomal translocations have an important role in the pathogenesis of
many NHLs.
• Some viruses and bacteria are implicated in the pathogenesis of NHL,
including HTLV-1, EBV, human herpesvirus 8, hepatitis B and C, Helicobacter
pylori, Chlamydophila psittaci, Campylobacter jejuni, and Borrelia
burgdorferi.
• Environmental factors linked to the development of NHL include chemicals
(e.g., pesticides, herbicides, solvents, organic chemicals, wood
preservatives).
• NHL is also more common in individuals who have inherited
immunodeficiency syndromes and who have used immunosuppressive
medications (e.g., to prevent rejection after an organ transplant or to treat
autoimmune disorders) or received chemotherapy or radiation therapy.

23. CONT’D
• NHLs involve lymphocytes arrested in various stages of development
and may mimic a leukemia. For example, small lymphocytic
lymphoma (SLL) and chronic lymphocytic leukemia (CLL) result from
malignant proliferation of small B lymphocytes, with CLL having the
majority of disease within the bone marrow (versus the lymph
nodes).
• Diffuse large B-cell lymphoma, the most common aggressive
lymphoma, is a neoplasm that originates in the lymph nodes, usually
in the neck or abdomen.
• Burkitt’s lymphoma is the most highly aggressive disease and is
thought to originate from B-cell blasts in the lymph nodes.

24. CLASSIFICATION OF NON-HODGKIN’S LYMPHOMA

25. Clinical Manifestations Lymphomas
• NHLs can originate outside the lymph nodes, the method of spread can be
unpredictable, and the majority of patients have widely disseminated
disease at the time of diagnosis
• The primary clinical manifestation is painless lymph node enlargement.
• The lymphadenopathy can wax and wane in indolent disease.
• Because the disease is usually disseminated when it is diagnosed, other
symptoms are present depending on where the disease has spread (e.g.,
hepatomegaly with liver involvement, neurologic symptoms with CNS
disease).
• NHL can also manifest in nonspecific ways, such as an airway obstruction,
hyperuricemia and renal failure from tumor lysis syndrome, pericardial
tamponade, and GI complaints.

26. • Patients with high-grade lymphomas may have lymphadenopathy and
constitutional symptoms (B symptoms) such as fever, night sweats,
and weight loss.
• The peripheral blood is usually normal, but some lymphomas
manifest in a “leukemic” phase.

27. Diagnostic
• Diagnostic studies used for NHL resemble those used for Hodgkin’s
lymphoma.
• However, because NHL is more often in extranodal sites, more
diagnostic studies may be done, such as an MRI to rule out CNS or
bone marrow infiltration, or a barium enema, upper endoscopy, or CT
to visualize suspected GI involvement.