Hodgkin’s lymphoma Rakesh Biswas MD, Professor, Department of Medicine, People's College of Medical Sciences, Bhanpur, Bhopal, India
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?
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. Hodgkin’s lymphoma
2. Non - Hodgkin’s lymphoma
Histologically & clinically a distinct malignant disease
Predominantly, B-cell disease
Course of the disease is variable, but the prognosis has improved with modern treatment
? Infection – EBV
? Environmental factors
Nodular Lymphocyte predominant
*REAL – Revised European,American,lymphoma
Bimodal age distribution :
young adults ( 20-30 yrs) & elderly (> 50yrs) M ay occur at any age
M > F
most often cervical region
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
Constitutional symptoms ( B symptoms )
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
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.
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
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
Stage I : Involvement of single LN region (I) or extra lymphatic site (IA E )
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 (III E ) or without (III) localized extra lymphatic involvement or involvement of the spleen (II S ) or both (IIS E )
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.
BMT / SCT
Antibody treatment: Rituximab target CD-20
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
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 Hodgkin’s Lymphoma
MOPP : Nitrogen Mustard, V incristine (Oncovin), P rocarbazine, Pr ednisolone
ABVD : A driamycin, B leomycin, V inblastine, D acarbazine
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.
Overall 10 yr survival – 80%
In long term survivors there is a risk of
secondary malignancy: ( leukemia , NHL ), Solid tumors- Lung, breast
Cardiac, pulmonary, endocrinal abnormalities
International Prognostic Index (IPI)
Advanced stage disease
Presence of Extra nodal disease
Non Hodgkin’s lymphoma
Incidence is increasing
Median age of presentation is 65-70 yrs
More often clinically disseminated at diagnosis
B-cell-70% ; T-cell-30%
‘ 1990…Although 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.
Widely disseminated at presentation
Nodal involvement : Painless lymphadenopathy , often cervical region is the most common presentation
'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
CT / MRI
Clinical / Working Formulation
Treatment: Not curable
Staging Similar to HD
Cannot be attributed a single cause
Chromosomal translocations : t (14, 18)
Virus: EBV, HTLV,HHV-8, HIV
Bacteria: H.Pylori - Gastric lymphoma
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.
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
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 C yclophosphamide, D oxorubicin H ydrochloride, V incristine, P rednisolonone
'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
With CNS involvement / leukemic relapse : Similar to ALL