Hodgkin’s disease was initially described as an inflammatory
disease (hence the term “disease”), but is clearly
recognized and treated as a malignant lymphoma (hence the
more accurate term Hodgkin’s lymphoma (HL) is used
synonymously with Hodgkin’s disease).
The management of Hodgkin’s lymphoma has evolved from
extended-field radiation alone as the main therapy to a
combined-modality approach with
chemotherapy and radiation, or chemotherapy alone.
Painles lymphadenopathy
Systemic symptoms- unexplained fevers, drenching night sweats, weight loss, generalize pruritus, fatigue, and alcohol-induced pain in tissues involved by HD
Mediastinal mass on a routine chest radiograph
90% of patients present with contiguous sites of involvement or Extension from adjacent lymph nodes
Hematogenous (liver or multiple bony sites) Involvement of the bones may cause blastic changes, especially in the vertebrae (creating the classic “ivory vertebra” on plain radiographs), pelvis, sternum, or ribs
Nearly all patients with hepatic or bone marrow involvement by Hodgkin lymphoma have extensive involvement of the spleen
Rare- Gut-associated lymphoid tissues such as Waldeyer ring and Peyer patches, Upper aerodigestive tract, Central nervous system, and Skin
oth Hodgkin's lymphoma and non-Hodgkin's lymphoma are lymphomas — a type of cancer that begins in a subset of white blood cells called lymphocytes. Lymphocytes are an integral part of your immune system, which protects you from germs.
This is a lecture on Lymphoma, exploring the different types and subtypes of Lymphomas. It also discusses the epidemiology, stages, clinical features, diagnosis, treatment and prognosis.
This was presented to undergraduate medical students at University Teaching Hospital (UTH), department of Cancer Disease Hospital by Nghitukuhamba Tangi Elikana Kalipi (6th year medical student) at Cavendish University Zambia, School of Medicine.
Background
Definition
Lymphoma is a malignant proliferation of mature lymphocytes that accumulate in lymph nodes ± other tissue, often as a solid tumour. Differs from leukaemia, which arises in the bone marrow and is present in the blood.
Hodgkin's lymphoma represents 20% of all lymphomas, and is characterised by the presence of 'mirror-image' binucleated Reed-Sternberg cells on light microscopy.
Hodgkin's lymphoma is a B-cell cancer.
Sub-types
Common: nodular sclerosis (70%), usually in young patients, and mixed cellularity (25%), usually in old patients. Both have a good prognosis.
Rare (5%): lymphocyte-rich (excellent prognosis) or lymphocyte-depleted (bad prognosis).
Epidemiology
Lifetime risk: 1/400.
Occurs any age but peaks at 15-30 and 75-80.
Commoner in men.
Hodgkin’s disease was initially described as an inflammatory
disease (hence the term “disease”), but is clearly
recognized and treated as a malignant lymphoma (hence the
more accurate term Hodgkin’s lymphoma (HL) is used
synonymously with Hodgkin’s disease).
The management of Hodgkin’s lymphoma has evolved from
extended-field radiation alone as the main therapy to a
combined-modality approach with
chemotherapy and radiation, or chemotherapy alone.
Painles lymphadenopathy
Systemic symptoms- unexplained fevers, drenching night sweats, weight loss, generalize pruritus, fatigue, and alcohol-induced pain in tissues involved by HD
Mediastinal mass on a routine chest radiograph
90% of patients present with contiguous sites of involvement or Extension from adjacent lymph nodes
Hematogenous (liver or multiple bony sites) Involvement of the bones may cause blastic changes, especially in the vertebrae (creating the classic “ivory vertebra” on plain radiographs), pelvis, sternum, or ribs
Nearly all patients with hepatic or bone marrow involvement by Hodgkin lymphoma have extensive involvement of the spleen
Rare- Gut-associated lymphoid tissues such as Waldeyer ring and Peyer patches, Upper aerodigestive tract, Central nervous system, and Skin
oth Hodgkin's lymphoma and non-Hodgkin's lymphoma are lymphomas — a type of cancer that begins in a subset of white blood cells called lymphocytes. Lymphocytes are an integral part of your immune system, which protects you from germs.
This is a lecture on Lymphoma, exploring the different types and subtypes of Lymphomas. It also discusses the epidemiology, stages, clinical features, diagnosis, treatment and prognosis.
This was presented to undergraduate medical students at University Teaching Hospital (UTH), department of Cancer Disease Hospital by Nghitukuhamba Tangi Elikana Kalipi (6th year medical student) at Cavendish University Zambia, School of Medicine.
Background
Definition
Lymphoma is a malignant proliferation of mature lymphocytes that accumulate in lymph nodes ± other tissue, often as a solid tumour. Differs from leukaemia, which arises in the bone marrow and is present in the blood.
Hodgkin's lymphoma represents 20% of all lymphomas, and is characterised by the presence of 'mirror-image' binucleated Reed-Sternberg cells on light microscopy.
Hodgkin's lymphoma is a B-cell cancer.
Sub-types
Common: nodular sclerosis (70%), usually in young patients, and mixed cellularity (25%), usually in old patients. Both have a good prognosis.
Rare (5%): lymphocyte-rich (excellent prognosis) or lymphocyte-depleted (bad prognosis).
Epidemiology
Lifetime risk: 1/400.
Occurs any age but peaks at 15-30 and 75-80.
Commoner in men.
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2. Hodgkin disease
Definition:
A neoplastic transformation of lymphocytes particularly in lymph nodes.
Characterized by:
1) the presence of Reed-Sternberg cells on histology
2) spreading in an orderly fashion to contagious lymph nodes
( Forexample, Hodgkin lymphoma that starts in thecervical lymph nodes
may spread first to the supraclavicular nodes then to the axillary nodes )
⚫ 6% of childhood cancer
⚫ 5% of cancer in < 14 yr
⚫ 15% in person 15-19 yr
⚫ Rare < 10 yr
3. Red-Sternberg cells
Largecells ( >45um in diameter) with classically binucleate or
bilobed central nucleus each with a large acidophilic central
nucleoli surrounded byaclear halo. “owl’seyeappearance”
4. Epidemiology of Hodgkin’s lymphoma
⚫ Hodgkin disease has bimodal agedistribution--onepeak in the 20s
and 60s.
⚫ Early peak middle to late 20s
⚫ Second peak after50 yr
⚫ Sex Male : Female
4: 1 for3-7 yr
3: 1 for7-9 yr
1-3: 1 for > 10 yr
⚫ 100 folds risk forunaffected monozygotictwin of affected twin
⚫ Associated with specific HLA antigen
⚫ Infectiousagents
Human herpesvirus 6
CMV
Epstein – Barrvirus
⚫ Immunodeficiency
5. Etiology/Risk Factors
Doctors seldom know why one person develops
Hodgkin lymphomaand anotherdoes not. But
research shows thatcertain risk factors increase the
chance thata person will develop thisdisease.
Having one or more risk factors does not mean that a
person will develop Hodgkin lymphoma. Most people
who have risk factors neverdevelopcancer.
6. Risk Factors
1) Certain viruses:
Epstein-Barr virus (EBV)
Human immunodeficiency virus (HIV)
2) Weakened immunesystem:
inherited condition
certaindrugs used afteran organ transplant
3) Age:
Hodgkin lymphoma is mostcommonamong teensand adults aged 15 to
35 years and adults aged 55 years and older.
4) Family history:
Family members, especially brothers and sisters, of a person with
Hodgkin lymphoma orother lymphomas may havean increased chance
of developing thisdisease.
7. Lymphocyte Predominant
10-15% of patients
More common in male
Younger patients
Localized disease
Has best prognosis
Mixed cellularity
30% of patients
< 10 yr of age
Advanced disease
Extranodal extension
Lymphocyte depletion
Rare in children
Common with HIV
Has worst prognosis
Nodular sclerosis
Most common
40% of younger patients
70% of adolescents
Classification
Rye Classification System
8. REAL Classification
( Revised European – American Classification of Lymphoid Neoplasms )
⚫Nodular lymphocyte predominance
⚫Classical Hodgkin lymphoma
⚫Lymphocyte rich
⚫Mix cellularity
⚫Nodularsclerosis
⚫Lymphocyte depletion
⚫ Anaplastic large cell lymphoma Hodgkin like
9. Enlarged, painless, rubbery, non- erythematous, nontender lymph nodes are
the hallmarkof thedisease.
May become painful afterdrinking alcohol
Hepatosplenomegaly
Cough, dyspnea, hypoxia
Pleural orpericardial effusion
Heptocellulardysfunction
B.M infiltration(Anemia, neutropenia, thrombocytopenia)
25% have ''B'' symptoms
Although pruritus is common in thedisease it is not one of the ‘’B’’ symptoms.
Cervical, supraclavicularand axillary lymphadenopathyare the most common
initial signsof thedisease.
Disease belowdiaphragm is rare (only3%)
Clinical presentation
10. Systemic Symptoms (B symptoms)
⚫Important in staging
⚫Unexplained fever > 390C
⚫ Weight loss > 10% in 3m
⚫ Drenching night sweats
Immune System abnormalities
⚫Anergy to delayed-hypersensitivity skin test
⚫Abnormal cellular immune response
⚫ Decreased CD4:CD8 ratio
⚫ Reduce natural killer cell cytotoxicity
11. Extralymphaticsites may be involved such as:
# Spleen
# Liver
# Bone marrow
# Lung
# CNS
Extralymphatic involvement is morecommon with
non-hodgkin lymphoma.
Emergencypresentation:
Infections
SVC obstruction ( facial edema, increased JVP
and Dyspnea)
12. Thedoctorconsiders the following todetermine the
stageof Hodgkin lymphoma:
The numberof lymph nodes affected.
Whether these lymph nodes areon one or both sides of
thediaphragm.
Whether thedisease has spread to the bone marrow,
spleen, liver, or lung.
Each stage is divided into A or B symptomsaccording to
the presence of systemic symptoms.
Staging of Hodgkin’s Lymphoma
13. Ann Arbor Staging Classification for Hodgkin
Disease
StageI
Involvementof a single lymph node (1)
orof a singleextra lymphatic siteororgan(1f)
StageII
Involvementof twoor more lymph node regionson the same side of
thediaphragm(II)
or localised involvementof an extra lymphaticsite
ororganand oneor more lymph node regionson the same side of the
diaphragm (IIf)
14. Stage III
Involvement of lymph node regions on both sides of the
diaphragm (III) which may be accompanied by the involvement of
spleen (IIIS) or by localized involvement of an extra lymphatic site or
organ ( IIIf) or both ( IIIsf)
Stage IV
Diffuse or disseminated involvement of one or more
extra lymphatic organs or tissues with or without associated lymph node
involvement.
The absence or presence of fever > 38C for three consecutive days ,
drenching night sweats , or unexplained loss of > 10% body weight in
the 6 months preceding admission are to be denoted in all cases by
the suffice letters A & B respectively.
15.
16. DIAGNOSIS
Anexcisional lymph node biopsy is theessential first step in diagnosis.
A biopsy is theonlysureway todiagnose Hodgkin lymphoma.
⚫ Excisional Biopsy
⚫ Light Microscopy
⚫ Immunocytochemistry
⚫ Molecular Studies
⚫ ChestX – Ray
⚫ Mediastinal Mass
⚫ CT Scan
⚫ Chest
⚫ Abdomen
⚫ Pelvis
⚫ Blood CP & ESR
⚫ LFT’s
⚫ BoneMarrow Aspiration
⚫ Serum Copper& Ferritin
⚫ Bone Scan
⚫ Gallium 67 Scan / FDG/PET
19. Therapy isentirely based on thestage.
Localized disease ( stage IA and IIA ) is managed predominantlywith
radiation.
All patientswithevidenceof ‘’B’’ symptomsaswell as stage III and IV
are managed withchemotherapy.
The most effective combination chemotherapeutic regimen for
Hodgkin lymphoma is ABVD ( adriamycin, bleomycin, vinblastinand
dacarbazine).
ABVD is superior to MOP (meclorethamine, vincristin(oncovin) ,
prednisoloneand procarbazine) becauseABVD has fewersideeffects
suchas:
1) Permanentsterility
2) Secondarycancerformation
3) Aplasticanemia
4) Peripheral neuropathy
TREATMENT
20. International Prognostic Index
The International Prognostic Index (IPI) was first developed to help
doctors determine the prognosis for people with fast-growing
lymphomas. The indexdepends on 5 factors:
1) The patient’sage
2) Thestageof the lymphoma
3) Whetheror not the lymphoma is in organsoutside the lymph system
4) Performance status (PS) – howwell a person can complete normal
dailyactivities
5) The blood (serum) level of (LDH)
21.
22. LONG TERM COMPLICATIONS
⚫Secondary malignancy
⚫ Acute Myelogenous Leukemia
⚫ Non Hodgkin lymphoma
⚫ Carcinomasof breast , lungs & thyroid
⚫Shortstature
⚫Hypothyroidism
⚫Sterility
⚫Dental caries
⚫Subclinical pulmonary dysfunction
⚫Ischemic heartdisease
