HODGKIN’S LYMPHOMA

I. Definition and brief overview of Hodgkin's Lymphoma

Hodgkin's lymphoma (HL) is a malignant neoplasm that arises from lymphoid tissue, specifically the B-
lymphocytes. HL is characterized by the presence of Reed-Sternberg cells, which are large, abnormal B-
lymphocytes that have multiple nuclei and are found in the lymph nodes.

II. Epidemiology and Etiology

A. Epidemiology of Hodgkin's Lymphoma

Hodgkin's lymphoma is a relatively rare cancer, accounting for approximately 0.5% of all new cancer
cases worldwide.

The incidence of Hodgkin's lymphoma varies by geographic region and age, with higher rates reported in
developed countries.

It is commoner in children and young adults.

The epidemiology of Hodgkin's lymphoma in Nigeria is not well-characterized due to limited data on the
incidence and prevalence of the disease in the country. However, studies suggest that the incidence of
Hodgkin's lymphoma in Nigeria is relatively low compared to other regions of the world, with an
estimated age-standardized incidence rate of 0.8 per 100,000 population

B. Etiology of Hodgkin's Lymphoma

The etiology of Hodgkin's lymphoma (HL) is multifactorial and involves a complex interplay between
genetic, environmental, and immunological factors.

1. Genetic factors may play a role in the development of HL, as certain mutations and
chromosomal abnormalities have been associated with an increased risk of the disease. For
example, alterations in the genes encoding for the TNF receptor and the HLA complex have been
implicated in HL susceptibility.
2. Infection with the Epstein-Barr virus (EBV) is a well-established risk factor for HL, with up to 40%
of HL cases being associated with EBV infection. Other viruses, such as the human
immunodeficiency virus (HIV) and human T-lymphotropic virus (HTLV), have also been linked to
an increased risk of HL.
3. Environmental factors, such as exposure to pesticides, herbicides, and other chemical toxins,
have been implicated in the development of HL. Some studies have also suggested an
association between HL risk and exposure to ionizing radiation, although the evidence is
inconsistent.
4. Immune system dysfunction, including autoimmune disorders and immunodeficiency
syndromes, may increase the risk of HL by impairing the immune system's ability to recognize
and eliminate abnormal cells.

The pathogenesis of HL involves the activation of abnormal B-cells, which give rise to the characteristic
Reed-Sternberg cells found in HL. The exact mechanisms underlying this process remain unclear, but
genetic and molecular studies are shedding new light on the pathogenesis of HL and may lead to the
development of new targeted therapies for the disease.
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III. Types of Hodgkin's Lymphoma

A. Classic Hodgkin's lymphoma (cHL): This is the most common type, accounting for about 95% of
cases. It is further divided into four subtypes: nodular sclerosis, mixed cellularity, lymphocyte-
rich, and lymphocyte-depleted.
B. Nodular lymphocyte-predominant Hodgkin's lymphoma (NLPHL): This is a less common
subtype, accounting for about 5% of cases. It is characterized by the presence of large, popcorn-
shaped cells called lymphocytic and histiocytic cells (L&H cells).

IV. Clinical presentation and symptoms

Children with Hodgkin's lymphoma may present with one or more of the following:

A. Enlarged lymph nodes: This is the most common symptom of Hodgkin's lymphoma. The lymph nodes
may be painless, firm, and rubbery to the touch. They are often located in the neck, armpit, or groin.

B. Fever: Children with Hodgkin's lymphoma may have an unexplained fever that comes and goes.

C. Night sweats: Children may experience drenching sweats at night, which can disrupt their sleep.

D. Weight loss: Children may lose weight without trying, which can be a sign of advanced disease.

E. Itching: Children may experience generalized itching, which is often worse at night.

F. Fatigue: Children may feel tired and lack energy, even after getting enough rest.

G. Pain: Children with advanced disease may experience pain, especially in the abdomen or chest.

V. Investigations

A. Imaging studies

A. Computed tomography (CT) scan: This noninvasive imaging modality can identify
lymphadenopathy, organ involvement, and extranodal disease.
B. Magnetic resonance imaging (MRI): This imaging test uses magnetic fields and radio waves to
produce detailed images of the body, and is particularly useful for assessing involvement of the
brain or spinal cord.
C. Positron emission tomography (PET) scan: This imaging test uses a radiotracer to detect
metabolic activity in cancer cells, which can help identify the extent of disease in the body and
guide treatment decisions.

B. Biopsy and histopathological examination

A. Excisional lymph node biopsy: This is the gold standard for diagnosis of Hodgkin's lymphoma. It
involves surgically removing an entire lymph node and examining it under the microscope for
the presence of Reed-Sternberg cells.
B. Fine-needle aspiration biopsy: This minimally invasive procedure involves inserting a thin needle
into a lymph node or other affected tissue and extracting a small sample for examination under
the microscope.
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C. Laboratory tests

A. Blood tests: These can help evaluate the child's overall health and identify abnormalities in
blood cell counts or other markers of disease.
1. Full blood count may show anemia, low white blood cell count (leukopenia), and/or low
platelet count (thrombocytopenia).
2. Erythrocyte sedimentation rate (ESR): This test measures the rate at which red blood
cells settle in a tube over a period of time. In Hodgkin's lymphoma, the ESR is often
elevated, indicating inflammation in the body.
3. Liver function tests: These tests measure levels of certain enzymes produced by the
liver. In Hodgkin's lymphoma, the liver may be affected, leading to elevated levels of
these enzymes.
4. Kidney function tests: These tests measure levels of certain waste products that are
filtered by the kidneys. In Hodgkin's lymphoma, the kidneys may be affected, leading to
elevated levels of these waste products.
5. Lactate dehydrogenase (LDH): This test measures the level of an enzyme that is released
into the bloodstream when cells are damaged or destroyed. In Hodgkin's lymphoma, the
LDH level may be elevated, indicating the presence of cancer cells.
B. Bone marrow biopsy: This invasive procedure involves taking a small sample of bone marrow
from the hipbone to check for the presence of Reed-Sternberg cells.

VI. Staging

Staging is a critical component of the diagnosis, treatment, and management of Hodgkin's lymphoma.
The Ann Arbor staging system is the most widely used staging system for Hodgkin's lymphoma, and it
classifies the disease into four stages based on the extent of involvement of lymph nodes and other
tissues. The stages are as follows:

1. Stage I: This stage involves a single lymph node or a single area of lymph nodes that are affected
by the cancer. For example, a patient may have cancer in one lymph node in their neck or groin.
2. Stage II: This stage involves two or more groups of lymph nodes that are affected by the cancer,
but all of the affected nodes are on the same side of the diaphragm. For example, a patient may
have cancer in lymph nodes in their neck and armpit on the same side of their body.
3. Stage III: This stage involves lymph nodes on both sides of the diaphragm that are affected by
the cancer. For example, a patient may have cancer in lymph nodes in their neck, armpit, and
groin.
4. Stage IV: This stage involves involvement of multiple organs or tissues. For example, a patient
may have cancer in their liver, lungs, or bone marrow, in addition to lymph nodes.

Each stage is further divided into sub-stages based on the presence or absence of "B symptoms," which
are systemic symptoms such as fever, night sweats, and weight loss. The presence of B symptoms
indicates more advanced disease and a poorer prognosis.
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VII. Treatment and Management

A. Treatment options for Hodgkin's Lymphoma

Hodgkin's lymphoma (HL) is a treatable malignancy with high cure rates.

The choice of treatment depends on various factors, including the stage of disease, the presence of risk
factors, and the child's overall health.

The main treatment modalities for HL include chemotherapy, radiation therapy, and stem cell
transplantation.

1. Chemotherapy
 Chemotherapy is the cornerstone of treatment for HL, and most children with HL receive
chemotherapy as part of their treatment regimen.
 The most commonly used chemotherapy regimens for HL include ABVD (doxorubicin,
bleomycin, vinblastine, and dacarbazine) and BEACOPP (bleomycin, etoposide, doxorubicin,
cyclophosphamide, vincristine, procarbazine, and prednisone).
 Chemotherapy can be given alone or in combination with other modalities, such as radiation
therapy or stem cell transplantation.
2. Radiation therapy
 Radiation therapy uses high-energy beams to kill cancer cells and is often used in combination
with chemotherapy.
 Radiation therapy is particularly effective for treating early-stage HL, and can also be used to
treat residual disease after chemotherapy.
 However, radiation therapy can cause long-term side effects, particularly in children, such as
growth and hormonal abnormalities, and an increased risk of secondary cancers.
3. Stem cell transplantation
 Stem cell transplantation is a treatment option for children with relapsed or refractory HL or
those who are at high risk of treatment failure.
 The two main types of stem cell transplantation are autologous (using the child's own stem
cells) and allogeneic (using stem cells from a donor).
 Stem cell transplantation can be associated with significant complications, including infection,
graft-versus-host disease, and organ damage.

B. Follow up Management of Hodgkin's Lymphoma

1. Supportive care

a. Management of treatment-related side effects

 Chemotherapy-related side effects: Nausea, vomiting, hair loss, and low blood counts can be
managed with antiemetics, scalp cooling, and growth factor support, respectively.
 Radiation-related side effects: Skin irritation, fatigue, and long-term complications can be
managed with topical treatments, rest, and long-term follow-up care.
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 Stem cell transplantation-related side effects: Infection prevention, graft-versus-host disease
management, and close monitoring for complications are essential.

a. Nutritional support

 Children with Hodgkin's lymphoma may experience weight loss, loss of appetite, and
malnutrition during treatment.
 Nutritional support, such as dietary counseling, enteral or parenteral nutrition, and appetite
stimulants, can improve the child's nutritional status and quality of life.

2. Palliative care

a. Symptom management

 Palliative care aims to relieve symptoms, such as pain, fatigue, and dyspnea that may arise
during advanced or refractory disease.
 Symptom management can be achieved through the use of pharmacologic and
nonpharmacologic interventions, such as opioids, nonsteroidal anti-inflammatory drugs, and
physical therapy.

b. Psychosocial support

 Children with Hodgkin's lymphoma may experience emotional and psychological distress,
including anxiety, depression, and social isolation.
 Psychosocial support, such as counseling, support groups, and play therapy, can help the child
and family cope with the emotional and psychological impact of the disease.

VIII. Prognosis and Follow-up

A. Prognosis for Hodgkin's Lymphoma

Factors affecting prognosis

Hodgkin's lymphoma has a relatively high cure rate, but the prognosis can vary depending on several
factors, including age, stage of the disease, subtype of the cancer, presence of B symptoms (fever, night
sweats, weight loss), and response to treatment.

Survival rates and outcomes

The five-year survival rate for Hodgkin's lymphoma is about 86%, and the ten-year survival rate is about
80%. However, these rates can vary depending on the stage of the disease, with early-stage Hodgkin's
lymphoma having a higher survival rate than advanced-stage disease.

B. Follow-up and monitoring for Hodgkin's Lymphoma

Surveillance testing

After completing treatment for Hodgkin's lymphoma, patients will undergo regular surveillance testing
to monitor for recurrence or relapse of the cancer. This may include physical exams, blood tests, imaging
studies, and bone marrow biopsies.
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Long-term follow-up and survivorship care

Patients who have completed treatment for Hodgkin's lymphoma will require long-term follow-up care
to manage any side effects of treatment and monitor for late effects of the cancer. This may involve
ongoing surveillance testing, as well as addressing any physical, emotional, or social needs of the patient
and their family. Survivorship care may also include strategies for maintaining a healthy lifestyle, such as
diet and exercise, and addressing any financial or logistical challenges that may arise as a result of the
cancer and its treatment