Hodgkin - PowerPoint PPT Presentation

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Hodgkin

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A 25 year old lady ... Constitutional symptoms ( B symptoms ) Night sweats, sustained fever 38 ... Derived from B cell, at an early stage of differentiation ... – PowerPoint PPT presentation

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Title: Hodgkin


1
Hodgkins lymphoma
Rakesh Biswas MD, Professor, Department of
Medicine, People's College of Medical Sciences,
Bhanpur, Bhopal, India
2
A 25 year old lady1 month of evening rise of
temperature, night sweats and noticed a lump in
her neckOn examination pallor, generalized
lymphadenopathy, hepatosplenomegaly
3
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.
4
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.
5
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?
6
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

7
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

8
Etiology
  • ? Infection EBV
  • ? Environmental factors

9
REAL Classification
  • Classic
  • Nodular Sclerosis
  • Lymhocyte rich
  • Mixed Cellularity
  • Lymhocyte depleted
  • Non-Classic
  • Nodular Lymphocyte predominant

REAL Revised European,American,lymphoma
10
Clinical features
  • Bimodal age distribution
  • young adults ( 20-30 yrs) elderly (gt 50yrs)
    May occur at any age
  • M gt F
  • Lymphadenopathy
  • most often cervical region
  • asymmetrical, discrete
  • painless, non-tender
  • elastic character on palpation ( rubbery)
  • not adherent to skin
  • fluctuate in size

11
  • Contiguous spread via the lymphatic chain
    eg.involvement of abdominal thoracic LNs
  • Extra nodal disease - rare
  • Hepatospleenomegaly

12
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13
  • Constitutional symptoms ( B symptoms )
  • Night sweats,
  • sustained fever gt 38 degree celsius,
  • loss of weight gt10 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 (gt10 cm) disease

14
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.
15
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

16
  • 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

17
The operation was done on February third, my
boyfriend's birthday. I made him a card out of
paper I hadHard to celebrate your boyfriend's
birthday while getting ready to get operated on.
18
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20
REED-STERNBERG ( RS ) Cell
21
REED-STERNBERG ( RS) Cell
22
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23
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."
24
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
25
gt10 cm
Bulky disease
26
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28
  • Lymphangiography

29
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

30
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31
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.
32
Treatment
  • RT
  • Chemo
  • BMT / SCT
  • Antibody treatment Rituximab target CD-20
  • Supportive

33
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 gt3 areas involved
  • Stage III and IV disease

34
Treatment
  • Stage IA , Stage IIA with 3 or lt 3 areas
    involved Radiotherapy
  • Stage IB, Stage II A with gt 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)

35
Irradiation fields used in Hodgkins Lymphoma
36
Chemotherapy
  • MOPP

    Nitrogen Mustard,

    Vincristine (Oncovin),
    Procarbazine,

    Prednisolone
  • ABVD
    Adriamycin,

    Bleomycin,

    Vinblastine,
    Dacarbazine
  • Higher dose for relapse or younger pts with poor
    prognostic features

37
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.
38
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

39
International Prognostic Index (IPI)
  • Age
  • Advanced stage disease
  • Performance status
  • Elevated LDH
  • Presence of Extra nodal disease

40
Non Hodgkins lymphoma
  • Incidence is increasing
  • NHLgtHD
  • Median age of presentation is 65-70 yrs
  • MgtF
  • More often clinically disseminated at diagnosis
  • B-cell-70 T-cell-30

41
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42
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.
43
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

44
  • Systemic symptoms
  • Sweating, weight loss, itching
  • Metabolic complications
    hyperuricemia,
    hypercalcemia,
    renal failure
  • Compression syndrome
  • Gut obstruction
  • Ascites
  • SVC obstruction
  • S/C Compression

45
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46
'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
47
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48
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
  • CT / MRI

49
Classification
  • REAL
  • Clinical / Working Formulation
  • Low grade
  • Inermediate grade
  • High grade

50
Classification
  • Low grade
  • Proliferation Low
  • Course Indolent
  • Symptoms -ve
  • Treatment Not curable
  • High grade
  • High
  • Rapid, fatal(un-Rx)
  • ve
  • Potentially Curable

Staging
Similar to HD
51
Etiology
  • Cannot be attributed a single cause
  • Chromosomal translocations
    t (14, 18)
  • Infection
  • VirusEBV, HTLV,HHV-8, HIV
  • Bacteria H.Pylori - Gastric lymphoma
  • Immunology
  • Congenital immunodeficiency,
  • Immunocompromised patients - HIV, organ
    transplantation

52
'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.
53
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
54
'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.
55
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

56
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.
57
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.
58
  • Aggressive ( high / intermediate grade)
  • Chemotherapy mainstay
    CHOP
    -every 3
    weeks, at least 6 cycles
    Cyclophosphamide,
    Doxorubicin Hydrochloride,

    Vincristine,
    Prednisolonone

59
'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.
60
  • 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

61
Prognosis
  • Low grade Median survival 10 yrs
  • High Grade
  • Increasing age, advanced stage, concomitant
    disease, raised LDH,T- cell phenotype Poor
    prognosis
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