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Last modified on
Tue Mar 12 2002 21:47:53 PST
What is Hodgkin's Disease
2. What is Hodgkin's Disease?
Without treatment, Hodgkin's is a fatal disease. Luckily, Hodgkin's generally responds well to modern chemotherapy and/or radiation treatments.
2.1 Hodgkin's Disease
Hodgkin's usually starts in a lymph node. Left untreated, the lymph circulating through the lymphatic system spreads Hodgkin's to adjacent lymph nodes as well as other parts of the lymphatic system such as the spleen, liver, and bone marrow. Hodgkin's can also invade tissues outside of the lymphatic system such as the lungs. Since lymph tissue is widespread in the body, Hodgkin's disease can start almost anywhere.
Hodgkin's disease is rare. The incidence is about 3 cases per 100,000 people per year, and it accounts for less than 1 percent of all cases of cancer in the United States. It is most often seen in people aged 15 to 34 or over the age of 60. It afflicts a slightly higher percentage of men and whites than women and blacks. Hodgkin's is one of the most treatable cancers and with treatment it has a high survival rate.
2.2 The lymphatic system
Connected along this network of vessels are groups of small, bean shaped and sized organs called lymph nodes. The lymph nodes filter the lymph as it passes through the nodes. Lymph nodes are found in the neck, armpits, abdomen, and groin. The lymphatic system also includes the tonsils, thymus (important to the development of T-cells, a type of white cells), spleen (which is a gland that filters bacteria and old red blood cells from the body), and bone marrow (spongy tissue inside the bones).
2.3 Reed Sternberg cell
The R-S cell is a very unusual and very large cell with more than one large nuclei. Each nucleus encloses a large nucleolus with an unusual clear space around it.
Click here to view a R-S cell from my pathology.
2.4 History of Hodgkin's Disease
This was a very important event for us though. This was the first time that lymphoma was clinically identified as separate from other diseases. This paper, written by a 34 year old Thomas Hodgkin described what was finally named Hodgkin's disease years after his 1866 death.
Hodgkin's disease has been called the "great white whale of hematopathology." For over a hundred years the mysteries of this illness proved illusive. Initially, it wasn't even known if Hodgkin's was cancer, an infection, or an inflammation. If fact, it's only in the last few years that modern molecular techniques have identified that the Reed Sterberg cell is probably an abnormal B lymphocyte.
So who was this man who was the first to get his hook into the whale? Hodgkin began his life as an English Quaker in 1798. He earned his medical degree in 1823 and spent his life pursuing both medical and social causes.
On the medical front he helped introduce the stethoscope to English medicine, helped produce the first accurate description of red bloods cells, did diabetes research, and spoke out about the medical ramifications of smoking.
Hodgkin was deeply religous and fervently worked on causes such as the better treatment of Canadian Indians and the abolishment of slavery.
Although Hodgkin's was the first to begin to understand Hodgkin's disease, he was not the only one to make important contributions to the understanding of this disease. Two other people who will forever have their names associated with Hodgkin's Disease are Reed and Sternberg. In 1898, Sternberg described 15 similar cases, which he thought were a kind of tuberculosis of the lymphatic system. However, he identified microscopically the cells that differentiate Hodgkin's from other lymphomas. Four years later, Reed described theses cells in detail. They are now called Reed-Sternberg (R-S) cells.
2.5 Symptoms and warning signs
The erythrocyte sedimentation (SED) rate is commonly high in Hodgkin's patients, and may be the only indication that active disease is present. Many Hodgkin's patients have normal SED rated though.
Also, since Hodgkin's disease reduces the body's ability to fight disease, patients have a tendency to develop various kinds of infections.
It may last a lifetime, but usually goes on for about 10 years before becoming lymphoma. The strangely good news, is that if it turns to lymphoma, it is treatable. -- Paul McMullen <firstname.lastname@example.org>
A system called the Ann Arbor Staging Classification is used to give a rough idea of the extent of the disease. The four stages are as follows:
Note, the E suffix is used when Hodgkin's extends to tissues beyond, but near, the major lymphatic sites. Stage IV refers to disease that is diffusely spread throughout a site outside the lymphatic system, such as the liver.
The stage is also given an A or B suffix. An A suffix is used unless one of the following specific "B" symptoms is present:
Note that the most significant "B" symptoms are fevers and weight loss. Night sweats alone do not imply an adverse prognosis.
Itching as a Hodgkin's symptom remains controversial and it is not considered a B symptom. However, when the itching is recurrent, generalized, and it comes and goes along with disease activity, it may be the equivalent of a B symptom.
If pathology proves involvement of one or more sites outside the lymphatic system, the symbol for the site of involvement, followed by a plus sign (+), is listed. Sites are identified by the following notations: N=nodes, H=liver, L=lung, M=bone marrow, S=spleen, P=pleura, O=bone, and D=skin.
Subtypes are determined by the variants of the Reed Sternberg cells present as well as the structure of the inflammatory background.
There are four different subtypes of Hodgkin's Disease. A system called the Rye modification of the Lukes and Butler classification is used to delineate the four subtypes:
2.8 What causes Hodgkin's Disease?
However, research continues and there are some clues which may eventually help understand the causes of Hodgkin's Disease: