Blood Matters Hodgkins Lymphoma
Hodgkins Lymphoma
Hodgkin’s lymphoma which is also called Hodgkin's disease; this is a type of lymphoma which is a cancer originating from white blood cells called lymphocytes. It was named after Thomas Hodgkin, who first described abnormalities in the lymph system in 1832, though he points out references to it going back to Marcello Malpighi in 1666.
Interesting History
While occupied as museum curator at Guy's Hospital, Hodgkin studied seven patients with painless lymph node enlargement. Of the seven cases, two were patients of Richard Bright, one was of Thomas Addison, and one was of Robert Carswell. Carswell' report of this seventh patient was accompanied by numerous illustrations that aided early descriptions of the disease. Hodgkin's report on these seven patients, entitled "On some morbid appearances of the absorbent glands and spleen", was presented to the Medical and Chirurgical Society in London in January 1832 and was subsequently published in the society's journal, Medical-Chirurgical Society Transactions. Hodgkin's paper went largely unnoticed, however, even despite Bright highlighting it in an 1838 publication. Indeed, Hodgkin himself did not view his contribution as particularly significant.
Tissue specimens from Hodgkin's seven patients remained at Guy's Hospital for a number of years. Nearly 100 years after Hodgkin's initial publication, histopathologic reexamination confirmed Hodgkin's lymphoma in only three of seven of these patients] The remaining cases included non-Hodgkin lymphoma, tuberculosis, and syphilis.
Hodgkin's lymphoma was one of the first cancers which could be treated using radiation therapy and, later, it was one of the first to be treated by combination chemotherapy
Hodgkins Today
Hodgkin’s lymphoma is characterized by the orderly spread of disease from one lymph node group to another and by the development of systemic symptoms with advanced disease. The disease usually starts in the lymph nodes in the neck, armpit or chest. Sometimes the cancer may spread to other lymph nodes nearby or cells may enter the blood stream and be carried to other organs.
Hodgkin's lymphoma must be distinguished from non-cancerous causes of lymph node swelling (such as various infections) and from other types of cancer. Definitive diagnosis is by lymph node biopsy (usually excisional biopsy with microscopic examination). Blood tests are also performed to assess function of major organs and to assess safety for chemotherapy. Positron emission tomography (PET) is used to detect small deposits that do not show on CT scanning. PET scans are also useful in functional imaging (by using a radiolabeled glucose to image tissues of high metabolism). In some cases a Gallium Scan may be used instead of a PET scan.
- There is a variant of Hodgkin’s Lymphoma called Hodgkin cell, it is an atypical mononuclear version of the Reed-Sternberg cells (RSC) and has the same characteristics, but is mononucleated.
- Hodgkin's lymphoma can be sub-classified by histological type. The cell histology in Hodgkin's lymphoma is not as important as it is in non-Hodgkin's lymphoma: the treatment and prognosis in classic Hodgkin's lymphoma usually depends on the stage of disease rather than the histotype.
Causes of Hodgkins
There are no guidelines for preventing Hodgkin's lymphoma because the cause is unknown or multifactorial. A risk factor is something that statistically increases your chance of getting a disease or condition. Risk factors include:
- Sex: male
- Ages: 15–40 and over 55
- Family history
- History of infectious mononucleosis or infection with Epstein-Barr virus, a causative agent of mononucleosis
- Weakened immune system, including infection with HIV or the presence of AIDS
- Prolonged use of human growth hormone
- Exotoxins, such as Agent Orange
After Hodgkin’s lymphoma is diagnosed, a patient will be staged, that is, they will undergo a series of tests and procedures that will determine what areas of the body are affected. These procedures will include documentation of their histology (study of the microscopic anatomy of cells and tissues), a physical examination, blood tests, chest x-ray and CT or MRI scans of the chest, abdomen and pelvis and a bone marrow biopsy.
When Hodgkin’s cells are examined microscopically there are cells with multiple nuclei (these are called multinucleated Reed-Sternberg cells, or RS cells.) These cells are the main characteristic in defining this disease. Classical Hodgkin's lymphoma (excluding nodular lymphocyte predominant Hodgkin's lymphoma) can be sub-classified into 4 subtypes based upon Reed-Sternberg cell morphology (that is the structure of the cells) and the composition of the reactive cell infiltrate seen in the lymph node biopsy specimen.
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Name |
Description |
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Is the most common subtype and is composed of large http://en.wikipedia.org/wiki/Tumor">tumor nodules showing scattered lacunar classical RS cells set in a background of reactive lymphocytes, eosinophils and plasma cells with varying degrees of collagen fibrosis/sclerosis. |
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Mixed-cellularity subtype |
Is a common subtype and is composed of numerous classic RS cells admixed with numerous inflammatory cells including lymphocytes, histiocytes, eosinophils, and plasma cells without sclerosis. This type is most often associated with EBV infection and may be confused with the early, so-called 'cellular' phase of nodular sclerosing CHL. |
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Is a rare subtype, show many features which may cause diagnostic confusion with nodular lymphocyte predominant B-cell Non-Hodgkin's Lymphoma (B-NHL). This form also has the most favorable prognosis. |
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Lymphocyte depleted |
Is a rare subtype, composed of large numbers of often pleomorphic RS cells with only few reactive lymphocytes which may easily be confused with diffuse large cell lymphoma. Many cases previously classified within this category would now be reclassified under anaplastic large cell lymphoma.[5] |
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Unspecified |
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On the basis of this staging, the patient will be classified according to a staging classification -the Ann Arbor staging classification scheme is a common one:
- Stage I is involvement of a single lymph node region (I) (mostly the cervical region) or single extralymphatic site (Ie); - Needs better explanation – not on wiki med
- Stage II is involvement of two or more lymph node regions on the same side of the diaphragm (II) or of one lymph node region and a contiguous extralymphatic site (IIe);
- Stage III is involvement of lymph node regions on both sides of the diaphragm, which may include the spleen (IIIs) and/or limited contiguous extralymphatic organ or site (IIIe, IIIes);
- Stage IV is disseminated involvement of one or more extralymphatic organs.
As well as giving each stage a number, doctors also use a letter code- either A or B to indicate symptoms such as fever, sweats or weight loss. If you have no symptoms your disease will be classified as A and if you have symptoms as B. For localized extranodal extension from mass of nodes that does not advance the stage, subscript 'E' is added.
Treatment
Hodgkin’s lymphoma may be treated with radiation therapy, chemotherapy or stem cell transplantation, the choice of treatment depending on the age and sex of the patient and the stage, bulk and histological subtype of the disease. These therapies may be used individually or together.
After Hodgkin’s lymphoma is diagnosed a patient will be staged. That is they will undergo a series of tests and procedures that will determine what areas of the body are affected. These procedures will include documentation of their histology, a physical examination, blood tests, chest X-ray, a CT or MRI scan and a bone marrow biopsy.
Patients with early stage disease (IA or IIA) are effectively treated with radiation therapy or chemotherapy. The choice of treatment depends on the age, sex, bulk and the histological subtype of the disease. Patients with later disease (III, IVA, or IVB) are treated with combination chemotherapy alone. Patients of any stage with a large mass in the chest are usually treated with combined chemotherapy and radiation therapy.
Radiotherapy
This is a treatment where high energy X-rays are directed at a cancer, in Hodgkin’s disease they are delivered through a machine called a Liner Accelerator. This form of treatment may be used on its own when the disease is confined to one or two groups of lymph nodes. It can also be used before after and during a course of chemotherapy.
Radiotherapy treatments are painless and similar to getting an X-ray. Treatments last less than 30 minutes each, and usually take place every week day during treatment. For lymphomas, there are a few different ways radiation oncologists target the cancer cells. Involved field radiation is when the radiation oncologists give radiation only to the parts of your body known to have the cancer. Very often, this is combined with chemotherapy. Radiation therapy directed above the diaphragm to the neck, chest and/or underarms is called mantle field radiation. Radiation to below the diaphragm to the abdomen, spleen and/or pelvis is called inverted-Y field radiation. Total nodal irradiation is when your doctor gives radiation to all the lymph nodes in the body to destroy cells that may have spread.
Prognosis
Treatment of Hodgkin's disease has been improving over the past few decades. Recent trials that have made use of new types of chemotherapy have indicated higher survival rates than have previously been seen. In one recent European trial, the 5-year survival rate for those patients with a favorable prognosis was 98%, while that for patients with worse outlooks was at least 85%.
In 1998, an international effort identified seven prognostic factors that accurately predict the success rate of conventional treatment in patients with locally extensive or advanced stage Hodgkin's lymphoma. Freedom from progression (FFP) at 5 years was directly related to the number of factors present in a patient. The 5-year FFP for patients with zero factors is 84%. Each additional factor lowers the 5-year FFP rate by 7%, such that the 5-year FFP for a patient with 5 or more factors is 42%.
The adverse prognostic factors identified in the international study are:
- Age ≥ 45 years
- Stage IV disease
- Hemoglobin < 10.5 g/dl
- Lymphocyte count < 600/µl or < 8%
- Male
- Albumin < 4.0 g/dl
- White blood count ≥ 15,000/µl
Other studies have reported the following to be the most important adverse prognostic factors: mixed-cellularity or lymphocyte-depleted histologies, male sex, large number of involved nodal sites, advanced stage, age of 40 years or more, the presence of B symptoms, high erythrocyte sedimentation rate, and bulky disease (widening of the mediastinum by more than one third, or the presence of a nodal mass measuring more than 10 cm in any dimension.)
Treatment
Treatment will vary depending on the exact type of lymphoma a person has, and how fast it is likely to grow and cause problems in the body. It will also depend on the extent of disease at diagnosis, the person's age, and their general health.
Some lymphomas grow slowly and cause few troubling symptoms, and may not need to be treated urgently. Others grow more quickly and need to be treated as soon as they are diagnosed. Treatment can involve chemotherapy, radiation therapy, and surgery. Cortico-steroid and immune therapies may also be used. Occasionally, a stem cell transplant is given to treat disease which has relapsed (come back), or where there is a high likelihood that the disease will relapse in the future.
Notable Cases
- Don Cohan, oldest U.S. Olympic bronze medalist at the age of 42, diagnosed with Stage 4B Hodgkins disease, defeated it twice, and then won a U.S. championship in sailing at the age of 72.
- Howard Carter, Egyptologist and discoverer of the Tomb of Tutankhamum, died in 1939 from Hodgkin's disease.
- Paul Allen, Microsoft co-founder, was diagnosed and treated for Hodgkin's lymphoma in 1983. He subsequently developed non-Hodgkin's lymphoma in November 2009
- Richard Harris, Irish actor, died from the condition in 2002
- Craig Wedren, lead singer of Shudder to Think and the lead for the newer pop-mash project, "BABY". In remission.
- Luke Menard, a finalist on the seventh season of American Idol, was diagnosed with the disease after being voted off the show.
- Starchild Abraham Cherrix, a teenager whose refusal to undergo further conventional treatment after relapsing resulted in a court battle and a change to Virginia laws about medical neglect.
- Michael C. Hall, American actor (Dexter, Six Feet Under), in remission as of April 2010.[44]
- Mamta Mohandas, Indian actress, survived the disease in November 2010.[46]
Resources
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Hodgkin Disease at American Cancer Society
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Hodgkin's Lymphoma at the American National Cancer Institute
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Information from the German Hodgkin Studygroup (English version)
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Timeline of discovery and treatment of Hodgkin's Lymphoma at hodgkinshistory.com
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Video and information booklet on Hodgkins Lymphoma
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Information on Hodgkins Lymphoma from Lymphoma Research Foundation
- Early Diagnosis and Referral of Lymphoma is Critical – article by Drs. Ken Bradstock and Peter Browett
- Is Lymphoma on Your Radar? – a decision support tool • Understanding Lymphoma – A4 tear off pad
- Understanding Lymphoma – wall poster • Tom has Lymphoma – children’s book
- Hodgkin and non-Hodgkin lymphoma patient information booklets
- Know your nodes – online quiz
