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Hodgkins Lymphoma

Formally known as Hodgkins Disease


Unique neoplasm in which the malignant cell,
the Reed-Stenberg cell (RSC), represents only
a small proportion of cells constituting the bulk
of the tumor.

Hodgkins Lymphoma
Malignancy of lymphoid tissue
Unknown aetiology
Highly associated with Epstein-Barr Virus
Boys > Girls
Bimodal distribution
Peaks : 1) Adolescent/ young adult years
(Rare below 5 years of age)
2) After 50 years old

Pathophysiology

HL-RSC carry clonal immunoglobulin gene rearrangements,


which establishes their clonality and their B cell origin.
Germinal-center B-cells undergo clonal expansion and
activate the process of somatic hypermutation that
introduces mutations at a high rate into rearranged
immunoglobulin variable genes.
Germinal-center B-cells with unfavorable mutations are
functionally crippled and undergo programmed cell death
(apoptosis).
The reason for the apoptosis resistance of HL-RSC
precursors is unclear but may involve several distinct
transforming events, such as Epstein-Barr Virus (EBV)
infection.

Clinical manifestations
Painless ,firm lymphadenopathy
Confined to one or two lymph nodes areas
(supraclavicular and cervical lymph nodes
Mediastinal lymphadenopathy cough, shortness
of breadth, chest pain RISK of respiratory failure
May also cause superior vena cava syndrome
(dyspnoea, facial swelling, head fullness,cough,
arm swelling,chest pain, dysphagia, orthopnea,
distorted vision, hoarseness, stridor,headache,
nasal stuffiness, nausea, pleural effusions, and
light-headedness.)

Ann Arbor Staging system


Stage I - Single lymph node
region or single extranodal site
Stage II - Two or more lymph
node regions on the same side
of the diaphragm
Stage III - Lymph node regions
on both sides of the diaphragm
Stage IV - Diffuse or
disseminated involvement of
one or more extralymphatic
organs (liver, bone marrow,
lung) or tissues with or without
associated lymph node
involvement (The spleen is
considered a nodal site.)

A or B designations are also used. B


includes the presence of at least one
of the following symptoms:
1) Fever > 38 degree Celcius for 3
consecutive days
2) Drenching night sweats
3) Unintentional weight loss of 10% or more
within 6 months of diagnosis

Lab/ Imaging studies


Complete blood count (CBC)
1. Hemolytic anemia (Coombs positive), anemia of
chronic disease, or anemia secondary to involvement of
the bone marrow
2. Leukocytosis, lymphopenia, eosinophilia, monocytosis
3. Thrombocytopenia due to marrow infiltration or
idiopathic thrombocytopenia purpura
Esr, C-reactive protein, serum ferritin and copper:
Increased
Calcium, alkaline phosphatase (bony metastasis),
lactate dehydrogenase, uric acid

Chest X-ray: access mediastinal mass


CT scan: abdominal involvement
Lymph node Biopsy

Pathologic
confirmation: ReedSternberg cells

Binucleated or
multinucleated
giant cells. Bilobed
nucleus with 2 large
nucleoli that
produces the
characteristic "owls
eye" appearance.
The typical RSC is
characterized by
CD30 positivity,
absence of J chains

Histopathology subtypes:

1)
2)
3)
4)

Lymphocyte predominant: 10-15%


Nodular sclerosis : 50-60%
Mixed cellularity : 30%
Lymphocyte depletion: <5%

Differential diagnosis

Leukemia
Rhabdomysarcoma
Nasopharyngeal carcinoma
Germ cell tumour
Thymomas
Infectious mononucleosis
Branchial cleft, thyroglossal duct cysts
Cat scratch disease
Toxoplasmosis
Tinea capitis

Treatment
Chemotherapy (highly sensitive)
- Cyclophosphamide, vincristine,
procarbazine, doxorubicin,
bleomycin, vinblastine, prednisone,
etoposide
Low dose radiation therapy

Complication
From treatment:
Short term:
- Immunosuppresion: nausea, vomiting,
alopecia
Late adverse effects:
- Second malignant neoplasm( AML, thyroid
malignancies, breast cancer)
- Hypothyroid, impaired soft tissue and bone
growth, cardiac dysfunction, pulmonary
fibrosis

Prognosis
Excellent
90% - 5 years overall survival rate
Survival in developing countries may
be lower, depending on availability of
care and medications, distance to
the treating centers, and number of
patients who abandon therapy before
completion.