Hodgkins

lymphoma
Presented by
Dr FERMIZET RUDY SpPD-FINASIM
FK UNILA -2010
 A 25 year old lady

1 month of evening rise of temperature,

night sweats and noticed a lump in her
neck

On examination pallor, generalized

lymphadenopathy,
hepatosplenomegaly
I wasn't feeling well, still couldn't
shake the cold that had been
plaguing me for what seemed
like months.

But all in all, not feeling too bad,

either.
 It is required that anybody teaching in
the schools have a clear tuberculosis
record.

After waiting several minutes, the nurse

read my test site.

She said it seemed to her there was a

little swelling that shouldn't be there,
and she asked me to wait and see the
doctor.
 I started to get a little bit
nervous, I mean, Tuberculosis?
Was that possible?

The doctor brought me into her

room and she examined me,
and she said she could feel
tumors in my neck. Had I
noticed them?
 Lymphoma
Clonal malignant disorders that are derived
from lymphoid cells: either precursor or
mature T-cell or B-cell

Majority are of B- cell origin

Divided into 2 main types :

1. Hodgkins lymphoma
2. Non - Hodgkins lymphoma
 Hodgkins Disease

Histologically & clinically a distinct

malignant disease
Predominantly, B-cell disease
Course of the disease is variable,
but the prognosis has improved with
modern treatment
 Etiology

? Infection EBV

? Environmental factors
 REAL* Classification
Classic:
Nodular Sclerosis
Lymhocyte rich
Mixed Cellularity
Lymhocyte depleted

Non-Classic
Nodular Lymphocyte predominant

*REAL Revised European,American,lymphoma

 Clinical features
Bimodal age distribution :
young adults ( 20-30 yrs) & elderly (> 50yrs)
May occur at any age
M>F
Lymphadenopathy:
most often cervical region
asymmetrical, discrete
painless, non-tender
elastic character on palpation ( rubbery)
not adherent to skin
fluctuate in size
 Contiguous spread via the lymphatic chain
eg.involvement of abdominal & thoracic
LNs
Extra nodal disease - rare
Hepatospleenomegaly
 Constitutional symptoms ( B symptoms )
Night sweats,
sustained fever > 38 degree celsius,
loss of weight >10% of body weight in 6 mo
Fever sometimes cyclical (Pel-Ebstein fever)
Pain at the site of disease after drinking
alcohol
Pallor
Pruritis
Symptoms of Bulky (>10 cm) disease
A zillion tests were done, blood
drawn a zillion times, a zillion
questions by a million doctors.

Finally it was decided they

would have to perform a biopsy
on one of the tumors to get a
diagnosis.
 Investigations
CBP :
Anemia ( normochromic / normocytic), eosinophilia,
neutrophilia, lymphopenia
ESR -raised
LFT- (liver infil / obs at porta hepatis)
RFT- prior to treatment
Urate , Ca,
LDH - adverse prognosis
CXR- mediastinal mass
CT thorax / abdomen / pelvis-for staging
Other: Gallium scan, PET, Lymphangiography ,
Laporotomy
 LN FNAC / biopsy :

Malignant REED-STERNBERG ( RS) Cell: Bi-

nucleate cell with a prominent nucleolus. Derived
from B cell, at an early stage of differentiation

Reactive background of eosinophils,

lymphocytes, plasma cells

Fibrous tissue
The operation was done on February
third, my boyfriend's birthday.

I made him a card out of paper I had

Hard to celebrate your boyfriend's

birthday while getting ready to get
operated on.
REED-STERNBERG ( RS ) Cell
REED-STERNBERG ( RS) Cell
The X-ray technician came out again,
looking for me.

"We're not ready for you yet, the

Doctors are still reading your chart, but
we wanted to make sure you didn't
leave.

Just wait a few minutes and the doctor

will be out to talk to you."
I've read in novels the expression "my
heart sank" but I'm not sure I ever
really felt that sort of thing until just
about then.

I'd been telling myself there was

nothing to worry about, all was ok, but
this was a clear cut sign that something
was very. very wrong
 >10 cm

Bulky disease
Lymphangiography
 Staging
Stage I : Involvement of single LN region (I) or extra
lymphatic site (IAE )
Stage II : Two or more LN regions involved (II) or an
extra lymphatic site and lymph node regions on the
same side of diaphragm
Stage III : Involvement of lymph node regions on both
sides of diaphragm, with (IIIE) or without (III) localized
extra lymphatic involvement or involvement of the
spleen (IIS) or both (IISE)
Stage IV : Involvement outside LN areas (Liver, bone
marrow)

A : Absence of B symptoms
B : B symptoms present
I had Stage IVb Hodgkin's Disease, a form of
cancer of the lymph nodes.

Well, it's sort of hard to describe what hearing

something like that does.
It seemed impossible that the nodes in my
neck, and the masses that were supposedly
on my lungs were cancer.

How could that be? I just was amazed.

I said "this is unbelievable." and felt more or

less detached, numb.
 Treatment

RT
Chemo
BMT / SCT
Antibody treatment: Rituximab target CD-20
Supportive
 Treatment - Guidelines
Indications for RT:
Stage I disease
Stage II disease with 3 or lesser areas involved
For Bulky disease
For pressure problems

Indications for CT
All with B symptoms
Stage II disease with >3 areas involved
Stage III and IV disease
 Treatment
Stage IA , Stage IIA with 3 or < 3 areas involved:
Radiotherapy

Stage IB, Stage II A with > 3 areas , Stage IIB:

Chemotherapy every 3-4 weeks, 6-8 cycles;
either alone, or in combination with radiotherapy

Stage III & IV :

Chemotherapy + Radiotherapy ( for bulky
disease or palliation of symptoms)
Irradiation fields used in Hodgkins Lymphoma
 Chemotherapy
MOPP :
Nitrogen Mustard,
Vincristine (Oncovin),
Procarbazine,
Prednisolone

ABVD:
Adriamycin,
Bleomycin,
Vinblastine,
Dacarbazine

Higher dose for relapse or younger pts with poor

prognostic features
 After six cycles of chemotherapy, my
CT scans still show masses on my
chest and in my neck.

On July 7, I had a meeting with my

doctors, and was told that if I continued
with standard chemotherapy, my
chances of being cured stand at less
than ten per cent.
 Prognosis

Overall 10 yr survival 80%

In long term survivors there is a risk of

secondary malignancy: (leukemia , NHL), Solid
tumors- Lung, breast
Infections
Cardiac, pulmonary, endocrinal abnormalities
International Prognostic Index (IPI)
Age
Advanced stage disease
Performance status
Elevated LDH
Presence of Extra nodal disease
 Non Hodgkins lymphoma
Incidence is increasing
NHL>HD
Median age of presentation is 65-70 yrs
M>F
More often clinically disseminated at
diagnosis
B-cell-70% ; T-cell-30%
 1990Although I had been feeling
fine, no different from normal, I was
worried about this lump in my neck that
I had for several months.

I first thought it was just because I had

some sort of infection, but it didn't go
away.
 Clinical features
Widely disseminated at presentation
Nodal involvement:
Painless lymphadenopathy, often cervical region
is the most common presentation
Hepatospleenomegaly
Extranodal :
Intestinal lymphoma ( abdominal pain, anemia,
dysphagia);
CNS ( headache, cranial nerve palsies, spinal
cord compression) ;
Skin, Testis; Thyroid; Lung
Bone marrow (low grade): Pancytopenia
 Systemic symptoms
Sweating, weight loss, itching
Metabolic complications:
hyperuricemia,
hypercalcemia,
renal failure
Compression syndrome:
Gut obstruction
Ascites
SVC obstruction
S/C Compression
'The surgeon took a biopsy of the lump,
taking a few cells out with a needle to
be looked at under the microscope.

When the results came back a few

weeks later, he told me that they
showed I had non-Hodgkin's lymphoma
 Diagnosis and staging
Similar to HD plus,
Bone marrow aspirate & trephine
Immunophenotyping : Monoclonal antibodies
directed against specific lymphocyte
associated antigens
B cell antigens ( CD 19, 20, 22);
T cell antigens ( CD 2, 3, 5 & 7)
Immunoglobulin determination: Ig G / IgM
praprotein marker
HIV
 Classification
REAL
Clinical / Working Formulation
Low grade
Inermediate grade
High grade
 Classification
Low grade High grade

Proliferation: Low High

Course: Indolent Rapid, fatal(un-Rx)
Symptoms: -ve +ve
Treatment: Not curable Potentially Curable

Staging
Similar to HD
 Etiology
Cannot be attributed a single cause
Chromosomal translocations: t
(14, 18)

Infection:
Virus:EBV, HTLV,HHV-8, HIV
Bacteria: H.Pylori - Gastric lymphoma

Immunology:
Congenital immunodeficiency,
Immunocompromised patients - HIV, organ
transplantation
 'When I went back a couple of weeks
later, he said that the results showed I
had stage I indolent follicular non-
Hodgkin's lymphoma, which is a slow-
growing form of the disease.

He said that the good news was that

only one lymph node was affected and
that I had no B symptoms.
 So, he suggested that I have
radiotherapy on the swollen lymph
node in my neck to make it go
away.

Because I had heard so many

stories about how radiotherapy can
make you sick and your hair fall
out, I was quite worried
'In the end, I didn't have any real side
effects, apart from feeling a bit tired,
and the lump in my neck went away
completely.

After a check-up a couple of weeks

later, the haematologist told me that I
should come back every 6 months for
another CT scan to make sure that the
non-Hodgkin's lymphoma hadn't come
back.
 Management
Low grade:
Asymptomatic : No treatment ;
Radiotherapy for localised disease (Stage 1);
Chemotheraphy: mainstay is
Chlorambucil; Initial response good , but
repeated relapses, median survival 6-10 yrs;
Newer: Fludarabine, 2-CdA (Chlorodeoxyadenosine)
Monoclonal antibody: Rituximab
SCT/BMT
In 1994, when I was between my CT
appointments, I found another lump in
my neck, so I called up the specialist
hospital and they told me to come back
early for my next scan.

At the same time, they did the bone

marrow test and the LDH blood test
again.
When I went back to see the
haematologist, he told me that they had
found

swollen lymph nodes in my chest and

my armpit, as well as my neck,

which really worried me, although he

said the disease had not spread to my
bone marrow.
Aggressive ( high / intermediate grade):

Chemotherapy: mainstay
CHOP
-every 3 weeks, at least 6 cycles
Cyclophosphamide,
Doxorubicin Hydrochloride,
Vincristine,
Prednisolonone
 'We talked about what treatment I
should have, and the doctor said that,
as I had radiotherapy on my neck
before, and now other lymph nodes
were involved, I couldn't have the same
treatment again.

However, he offered me chemotherapy

instead, and I was given the treatment
over the next six months.
 High risk cases with poor prognostic
factors or relapse :
High dose chemotherapy combined with
autologous BMT / SCT

Monoclonal antibody

With CNS involvement / leukemic relapse :

Similar to ALL
 Prognosis
Low grade : Median survival 10 yrs
High Grade:
Increasing age, advanced stage, concomitant
disease, raised LDH,T- cell phenotype : Poor
prognosis
FINISH