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About
Lymphoma > Types of Lymphoma > Hodgkins
Lymphoma
Last update: 05/15/2008
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TOPIC SEARCH: PubMed: Diagnosis
| Review
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Overview
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Overview of
Lymphoma
What
is lymphoma? Briefly, lymphomas result when DNA
damage or changes occurs to an immune cell (a lymphocyte) that
alters the behavior of the cells.
The damage to DNA results in the
abnormal production of proteins that prevents the cells from dying
when they should, or causes sustained rapid cell division that
produces more of its kind.
These malignant cells then may accumulate
to form tumors that may enlarge the lymph nodes or spread to other
areas of the lymphatic system,
such as the spleen or bone marrow.
Lymphoma can also spread or first appear outside the lymphatic system
-- and is called extranodal
disease.
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Hodgkin's Lymphoma
A hallmark of cancer cells is that they have growth
and survival advantages over normal cells. Their cell division is not
balanced by cell death. The abnormal cells may eventually form lumps
called tumors.
All lymphomas involve lymphocytes (white blood cells) that have developed errors causing these cells to have growth and survival advantages over normal cells of the same type. Therefore these cells will accumulate to form tumors in the lymphatic system and blood. Most often the areas of presentation are limited to the lymphatic system, but not always.
What is the difference between Hodgkin's lymphoma and non-Hodgkin's lymphoma?
The difference is in the type of lymphocytes
involved - the cell of origin.
In Hodgkin's disease, the abnormal lymphocyte is the Reed-Sternberg cell
(a B lymphocyte). This particular lymphocyte isn't found in other types of
lymphomas. All other types of lymphomas are called non-Hodgkin's
(NHL). There are about 30 subtypes of NHL.
Identifying the correct type of lymphoma is important because treatment for Hodgkin's and non-Hodgkin's can be very different.
Pathologists can distinguish between Hodgkin's and non-Hodgkin's by examining the
cell sample from a biopsy under a microscope.
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Incidence
"The American Cancer Society estimates that
7,350 men and women (3,980 men and 3,370 women) will be diagnosed with
Hodgkin's lymphoma.
From 1998-2002, the median age at diagnosis for
Hodgkin's lymphoma was 37 years of age. Approximately 12.5% were
diagnosed under age 20; 32.9% between 20 and 34; 19.0% between 35 and
44; 12.0% between 45 and 54; 7.8% between 55 and 64; 8.4% between 65
and 74; 5.8% between 75 and 84; and 1.6% 85+ years of age."
Source: SEER
- Stat Fact Sheet 2006
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Symptoms of Hodgkin's Lymphoma
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painless swelling in the neck, armpits or
groin |
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night sweats |
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unexplained fever |
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unexplained weight loss |
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unexplained fatigue |
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cough or difficulty in breathing |
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persistent itch all over the body (pruritus) |
NOTE: These symptoms are common to many conditions other than Hodgkin's disease.
A definite diagnosis can only be made by removing an enlarged lymph
node or part of it and examining the cells. This test is known as a biopsy.
It is a very small operation and is commonly done under local
anesthesia.
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Staging
Staging
describes how wide spread the disease is. Hodgkin's disease is
characterized by contiguous (side-by-side) spread. Metastasis -
dissemination to all areas - is a late event.
Anne Arbor staging for Hodgkin's Lymphoma - Virginia.edu
click to enlarge illustration
Stage I
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Single node region (or extranodal) site
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Stage II
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Two or more lymph nodes on same side of diaphragm
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Stage III
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Lymph Nodes (sites) on both sides of the diaphragm
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Stage IV
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Multiple or disseminated spread
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Incidence of Hodgkin's
Lymphoma
Type
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New Cases
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Deaths per year
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Survival 5/10/15 year
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| Hodgkin's |
7,000 |
1,400 |
83% & 74% & 66% |
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Categories of Hodgkin's Lymphoma
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Nodular
Lymphocyte Predominant Hodgkin's Disease (NLPHD)
"B-cell lymphoproliferative disorder distinct from classical
HD
Uncommon, accounting for approximately 5-7% of all cases of
HD;
Most cases are clinically indolent; not associated with systemic B
symptoms
Close clinical follow-up and a low threshold for repeat biopsy is
not an unreasonable course for these patients. The subsequent
development of a hematolymphoid neoplasm in patients with
antecedent nodular LPHD should alert one to the possible presence
of a DLCL of B- lineage." hitchcock.org
 Also
see PubMed
| ClinicalTrials.gov
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Source: gucfm.georgetown.edu
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Infradiaphragmatic versus supradiaphragmatic Hodgkin lymphoma:
a retrospective review of 1114 patients. Leuk Lymphoma. 2005
Dec;46(12):1715-20. PMID:
16263573
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CD20 expression in Hodgkin's Lymphoma
| CD20 Expression in Hodgkin and Reed-Sternberg Cells of Classical Hodgkin’s Disease: Associations With Presenting Features and Clinical Outcome
jco.org
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Prognostic indicators in Hodgkin's Lymphoma
| NEW: Prognostic impact of bone involvement in Hodgkin lymphoma.
Neoplasma. 2008;55(2):96-100. PMID: 18237246 Medscape
... bone involvement is a relatively common finding in HL and is not an independent adverse prognostic factor. Key words: Hodgkin lymphoma - bone involvement - prognostic factors.
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| NEW: Better
Prognosis for Patients with Lymphocyte-predominant Hodgkin’s
Lymphoma patient.cancerconsultants.com
"In order to better understand characteristics of LPHL
[Lymphocyte-predominant HL], researchers from Germany conducted an
analysis of 8,298 HL patients treated within a German medical
trial to compare patient characteristics and treatment outcomes
among cHL [classical HL) patients and others diagnosed with LPHD .
"
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| Hodgkin's lymphoma in the elderly: The results of 10 years
of follow-up.
Leuk Lymphoma. 2006 Aug;47(8):1518-22. PMID:
16966262
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| Hodgkin disease survival in Europe and the U.S.: prognostic
significance of morphologic groups. Cancer. 2006 Jul
15;107(2):352-60. PMID:
16770772
Morphology distribution varied markedly across Europe and much
less in the U.S., with nodular sclerosis less common in Europe
(45.9%) than the U.S. (61.7%). The RER data showed that patients
who had lymphocyte depletion, NOS, and mixed cellularity had a
significantly worse prognoses compared with patients who had
nodular sclerosis, whereas patients who had lymphocyte
predominance had the best prognosis.
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| MAL [a gene also expressed in mediastinal (thymic) large
B-cell lymphoma] is expressed in a subset of Hodgkin lymphoma and
identifies a population of patients with poor prognosis. Am J Clin
Pathol. 2006 May;125(5):776-82. PMID:
16707382
"Expression correlated with nodular sclerosis subtype, and
within this subtype, with grade 2 histology."
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Resources
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Hodgkin’s Lymphoma: Evolving Concepts
with Implications for Practice asheducationbook.org
Ralph M. Meyer, Richard F. Ambinder and Sigrid Stroobants
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Risk Factors
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Risk Factors
What causes Hodgkin's
disease is not known. Risk factors associated with contracting
this kind of blood cancer include:
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Inborn immune deficiency
diseases |
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Acquired immunodeficiency from AIDS or immunosuppressive
drugs |
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Living in Western countries, being of higher social class, more
educated |
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Genetic pre-disposition, clusters are noted in siblings with similar HLA
genotypes |
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Infection with Epstein-Barr Virus (EBV) history is noted in up to 40% of patients developing Hodgkin's.
Elevated levels of the IgG and IgA immunoglobulins against the EBV capsid antigen are noted 3 months to 12 years prior to clinical Hodgkin's development.
Components of the EBV genome have been noted in the cellular DNA
of the Reed-Stemberg cell (Ref. Weiss NEJM 320: 502 1989).
However, the EBV is not noted in all patients and may be merely a
marker of the poorer cellular immunity (but intact humoral
immunity) seen in Hodgkin's patients. 1 |
"The most
important risk factors are: 1) genetic; 2) Epstein-Barr virus
(infectious mononucleosis); 3) congenital and acquired
immunodeficiency; 4) occupational exposure (the wood industry). 1
"Epstein Barr virus (EBV) is associated with
around one-third of Hodgkin's lymphoma (HL) cases and this association
is believed to be causal."
"The increased risk of NHL and HL among
individuals with a family history of hematopoietic malignancy was
approximately twofold for both lymphoma types."
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[Risk factors for Hodgkin's lymphomas] An Esp Pediatr. 2001
Sep;55(3):239-43. Review. Spanish. PMID:
11676899
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Risk factors for Hodgkin's lymphoma by EBV status and
significance of detection of EBV genomes in serum of patients with
EBV-associated Hodgkin's lymphoma.
Leuk Lymphoma. 2003;44 Suppl 3:S27-32. Review. PMID:
15202522 | Related
articles
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Characteristics of Hodgkin's lymphoma after infectious
mononucleosis.
N Engl J Med. 2003 Oct 2;349(14):1324-32. PMID:
14523140
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Family history of hematopoietic malignancy and risk of
lymphoma.
J Natl Cancer Inst. 2005 Oct 5;97(19):1466-74. PMID:
16204696 | Related
articles
Resources
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Epstein-Barr virus (EBV) associated lymphomas Related
abstracts
The three main types of EBV-associated B-cell lymphoma are
Burkitt lymphoma, Hodgkin lymphoma and post-transplant lymphomas
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Treatments & Long Term Side
Effects
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Generally, Hodgkins disease is treated with chemotherapy
or radiotherapy. Sometimes, both are given. Treatment depends on
the stage of the disease, it's location in the body, symptoms, and the
age and general health of the patient.
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TREATMENT - Standard of care
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| NEW:
Gemcitabine in the
treatment of Hodgkin lymphoma - Abstract
2008
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| A systematic overview of radiation therapy effects in
Hodgkin's lymphoma.
Acta Oncol. 2003;42(5-6):589-604. Review. PMID:
14596517
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| Hodgkin's Lymphoma: Basing the Treatment on the Evidence
(2001) asheducationbook.org
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Results of Conventional Therapy medscape.com
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Guidance for Parents on Childhood cancers by NCI
Large PDF
file | PDF-Help
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Articles on Refractory HD PubMed
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A parent's guide to children's cancer CancerBACUP
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Long Term Side Effects
TOPIC SEARCH:
PubMed
"Successfully treated children and adolescents
with Hodgkin's disease have a substantial risk for the occurrence of
subsequent neoplasms. The most frequent SMNs (skin, thyroid, and
breast) are readily detected by physical examination and available
screening procedures." 1
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Second Malignant Neoplasms After Treatment for Hodgkin's
Disease Medscape
free login req.
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A systematic overview of radiation therapy effects in
Hodgkin's lymphoma.
Acta Oncol. 2003;42(5-6):589-604. Review. PMID:
14596517
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A systematic overview of chemotherapy effects in Hodgkin's
disease.
Acta Oncol. 2001;40(2-3):185-97. Review. PMID:
11441931
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Late cardiotoxicity after treatment for Hodgkin's lymphoma.
Blood. 2006 Nov 21; PMID:
17119114
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Individualized estimates of second cancer risks after
contemporary radiation therapy for Hodgkin lymphoma. Cancer. 2007
Oct 16; PMID:
17941006
Contemporary IFRT is predicted to substantially reduce risk of
secondary breast and lung cancer compared with mantle RT, with
considerable variation in risk among individuals. Individualized
prospective risk estimates could facilitate patient-specific
counseling and the development of more effective RT techniques.
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Clinical Trials
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ClinicalTrials.gov
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Prognosis and Survival
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TOPIC
SEARCH : ASCO
| Medscape
| PubMed
| PubMed
- stage IV Bulky
About survival statistics:
Statistics cannot predict what will happen to you or a loved
one. Each patient and case is unique, and treatment outcomes can
vary from one person to another. Indeed, not even your doctor
can tell you for sure what will happen. The term '5 year
survival' is used often. It relates to the proportion of people
in research studies who were still alive 5 years after diagnosis.
Patients who live 6, 10, or 30 years after diagnosis are
also in this group. Also see Jay Gould's encouraging essay: The
Median isn't the Message
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Tumor microenvironment and mitotic checkpoint are key factors
in the outcome of classical Hodgkin lymphoma. Blood. 2006 Mar 21; PMID:
16551964
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Prognosis of bulky Hodgkin's disease treated with chemotherapy
alone or combined with radiotherapy. Cancer Surv.
1985;4(2):439-58. PMID:
2430700
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Research News
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 | NEW:
outcomes: min-SCT: Superiority of reduced-intensity allogeneic transplantation over
conventional treatment for relapse of Hodgkin's lymphoma following
autologous stem cell transplantation
This study compares outcome of reduced-intensity conditioned transplant
(RIT) with outcome of conventional non-transplant therapy in patients with
Hodgkin's lymphoma relapsing following autograft.
There were 72 patients in two groups who had relapsed, and received salvage
therapy with chemotherapy radiotherapy.
One group (n=38) then underwent alemtuzumab-containing RIT. The second
group-historical controls (n=34), relapsing before the advent of RIT-had no
further high-dose therapy.
This group was required to respond to salvage therapy and live for over 12
months post-relapse, demonstrating potential eligibility for RIT, had this
been available.
Overall survival (OS) from diagnosis was superior following RIT
(48% at 10 years versus 15% ; P=0.0014),
as was survival from autograft
(65% at 5 years versus 15% ; P0.0001).
For the RIT group, OS at 5 years from allograft was 51% , and in
chemoresponsive patients was 58% , with current progression-free survival of
42% .
Responses were seen in 8 of 15 patients receiving donor lymphocyte infusions
(DLI) for relapse/progression, with durable remission in five patients at
median follow-up from DLI of 45 months (28-55).
These data demonstrate the potential efficacy of RIT in heavily pre-treated
patients whose outlook with conventional therapy is dismal, and provide
evidence of a clinically relevant graft-versus-lymphoma effect.
full text: http://www.nature.com/bmt/journal/v41/n9/full/1705977a.html
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| Outcomes (small study): mTOR Inhibition
( everolimus) for Relapsed or Refractory Hodgkin Lymphoma: Promising Single Agent Activity with Everolimus (RAD001).
Session Type:
ASH Poster Session, Board #745-II
Oral everolimus has promising activity with acceptable toxicity in Hodgkin lymphoma. These results provide the rationale for additional studies with this novel class of agents and to integrate mTOR inhibitors into salvage treatment regimens for Hodgkin lymphoma.
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Outcomes: Rituxan in relapsed
lymphocyte-predominant Hodgkin Lymphoma: Long-term results of a
phase-II trial of the German Hodgkin Lymphoma Study Group (GHSG).
Blood. 2007 Oct 15; PMID:
17938252
Thus, rituximab is highly effective in relapsed and refractory
NLPHL. This study is registered at /www.klinisches-studienzentrum.de/trial/285
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| Promising
treatment target found in Hodgkin lymphoma eurekalert.org
Using gene microarray chips, the scientists looked for genes
that were active in Reed-Sternberg cells but not in cells of
another non-Hodgkin B-cell lymphoma. The comparison revealed
that a gene called Gal1 was up to 30 times more active in the
Reed-Sternberg cells, causing them to secrete large quantities of
a protein -- Gal1 or Galectin 1 -- that turns down the Th1 immune
response.
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| Stem
cell transplantation in Hodgkin lymphoma.
Expert Rev Anticancer Ther. 2007 Mar;7(3):297-306. Review. PMID:
17338650
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| Outcome
of Patients Experiencing Progression or Relapse After Primary
Treatment With Two Cycles of Chemotherapy and Radiotherapy for
Early-Stage Favorable Hodgkin's Lymphoma. J Clin Oncol. 2007 Apr
9; PMID:
17420510
Relapse after primary treatment with two cycles of doxorubicin,
bleomycin, vinblastine, and dacarbazine followed by RT is rare. In
our analysis, results were influenced by a high treatment-related
mortality rate. Additional studies are needed to define the
optimal salvage therapy.
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| Long-term
events in adult patients with clinical stage IA-IIA nonbulky
Hodgkin's lymphoma treated with four cycles of doxorubicin,
bleomycin, vinblastine, and dacarbazine and adjuvant radiotherapy:
a single-institution 15-year follow-up. PMID:
17085663
"Long-term events were mostly related to radiotherapy; the
role of short ABVD chemotherapy was very limited, as documented by
fertility preservation and lack of secondary
myelodysplasia/leukemia."
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LACE-conditioned autologous stem cell transplantation for
relapsed or refractory Hodgkin's lymphoma: treatment outcome and
risk factor analysis in 67 patients from a single centre.
Bone Marrow Transplant. 2006 Nov 20; PMID:
17115062
LACE followed by ASCT is an effective treatment for the
majority of patients with chemosensitive relapsed Hodgkin's
lymphoma and a proportion of chemorefractory patients also
benefit.
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Long-term outcome after radiotherapy alone for
lymphocyte-predominant Hodgkin lymphoma. Cancer. 2005 Aug 10; PMID:
16094666
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Vanishing Bile Duct Syndrome in Hodgkin's Disease:
case report ~ Department of Internal Medicine and Hematology and
Blood Transfusion Center, Universidade Estadual de Campinas,
Campinas, Brazil full
text | Related PubMed
articles
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Results of a prospective randomized clinical trial of
doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD)
followed by radiation therapy (RT) versus ABVD alone for stages I,
II, and IIIA nonbulky Hodgkin disease. Blood. 2004 Dec
1;104(12):3483-9. Epub 2004 Dec 1. PMID:
15315964 | Related
articles
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| Baseline Tumor Burden Predicts Clinical Outcome in Hodgkin
Lymphoma leukemia-lymphoma.org
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Prognostic factors in Hodgkin's disease.
Leuk Lymphoma. 2004 Jun;45(6):1133-9. PMID:
15359992 | Related
articles
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Long Term Outcome in Adolescents with Hodgkin's Lymphoma: Poor
Results using Regimens Designed for Adults. Leuk Lymphoma.
2004;45(8):1579-1585. PMID:
15370209
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The clinical value of tumor burden at diagnosis in Hodgkin
lymphoma.
Cancer. 2004 Sep 15 PMID:
15372482
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Strong impact of highly active antiretroviral therapy on
survival in patients with human immunodeficiency virus-associated
Hodgkin's disease. Br J Haematol. 2004 May;125(4):455-62. PMID:
15142115 | Related
articles
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Efficacy of
vinblastine, bleomycin, methotrexate (VBM) combination
chemotherapy with involved field radiotherapy in early stage (I-IIA)
Hodgkin disease patients. Leuk Lymphoma. 2003 Nov;44(11):1919-23.
Review. PMID:
14738143 | Related
articles
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Increased serum levels
of interleukin-9 correlate to negative prognostic factors in
Hodgkin's lymphoma. Leukemia. 2003 Oct 9 [Epub ahead of print] PMID:
14562126 | More
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Bispecific antibodies
- novel investigative treatment for refractory Hodgkin's
disease related
abstracts
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Involved-field
radiotherapy for advanced Hodgkin's lymphoma.
N Engl J Med. 2003 Jun 12;348(24):2396-406. PMID: 12802025 PubMed
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Epstein-Barr virus and
other candidate viruses in the pathogenesis of Hodgkin's disease.
Semin Hematol. 1999 Jul;36(3):260-9. Review.
PMID: 10462326 PubMed
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Abstracts
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Severe pruritus should be a B-symptom in Hodgkin's disease.
Cancer. 1983 May 15;51(10):1934-6. PMID: 6831358 PubMed
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Randomized comparison of ABVD and MOPP/ABV hybrid for the
treatment of advanced Hodgkin's disease: report of an intergroup
trial. J Clin Oncol. 2003 Feb 15;21(4):607-14.
PMID: 12586796 PubMed
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Treatment of relapsed
CD20+ Hodgkin lymphoma with the monoclonal antibody rituximab is
effective and well tolerated: results of a phase 2 trial of the
German Hodgkin Lymphoma Study Group. Blood 2003; 101: 420-424..
PubMed
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