Hodgkin's Disease - Human Lymphatic System
The "Bump" in the Road
You never know....," the start of the statement by Pennsylvania's Senator Arlen Specter (Republican) when he related the recurrence of Hodgkin's disease to the press during April 2008, aptly leads into this paper exploring Hodgkin's disease and its effects on an individual's body.
Senator Specter states one word best describes his response to the news during April 2008 that he was experiencing a recurrence of Hodgkin: "Surprise." At the time, Senator Specter reported that while living an active schedule, he had not noted any symptoms. "You never know," he stressed. "You deal with it as it comes up." ("Sen. Specter Comments...," 2008) Senator Specter states that he considers this current battle with Hodgkin "another bump in the road" and that he has "good shock absorbers." For treatment, Senator Specter's doctor prescribed a 12-week regimen of chemotherapy, once a week over 12 weeks. Prospects for a full recovery are reportedly excellent, he stressed. Approximately 90% of individuals diagnosed with Hodgkin's disease, Kaeseoglu; Senayli; Bicakci; Onuk-Filiz; Et al (2007) purport, are cured with chemotherapy and/or radiation. This form of cancer is reportedly one of the most curable ones, especially in children,
The Human Lymphatic System
The human lymphatic system, comprised of lymph nodes (or lymph glands) and lymphatics (small vessels connecting lymph nodes), returns excess fluid to the body's circulation, as it assists in combating infections (and cancer). The lymph nodes, usually occurring in clusters in a person's neck, armpits, and groin, produce a number of white blood cells and antibodies that help ward off infection. The right lymphatic duct, along with the thoracic duct, draws lymph fluids into two veins uniting to create the inferior vena cava. The vena cava passes into the heart. The cisterna chili, a broad portion of the thoracic duct, receives fluids from several lymph-collecting vessels. The spleen ultimately eradicates worn-out red blood cells; helping fight infection. (Kaeseoglu; Senayli; Bicakci; Onuk-Filiz; Et al, 2007)
The "Flow" of the Lymphatic System
The narrow lymphatic vessels carry lymphatic fluid from throughout the body. The lymph nodes are small organs that filter the lymphatic fluid and trap foreign substances, including viruses, bacteria, and cancer cells. The spleen, in the upper left abdomen, removes old cells and debris from the blood. The bone marrow, the tissue inside the bones, produces new red and white blood cells.
Lymphocytes are white blood cells that recognize and destroy disease-causing organisms. Lymphocytes are produced in the lymph nodes, spleen, and bone marrow. They circulate throughout the body in the blood and lymphatic fluid. Clusters of immune cells also exist in major organs. (Carson-DeWitt and Alic, 2002)
The following figure (1) depicts the human lymphatic system, part of a person's immune system which fights disease, as well as a component of the body's blood-producing system, which "includes the lymph vessels and nodes, and the spleen, bone marrow, and thymus": (Carson-DeWitt and Alic, 2002)
Figure 1: The Human Lymphatic System ("Atlas of the Body...," 1999)
Hodgkin's Disease: A Cancer of the Lymphatic System in 1832, Thomas Hodgkin, a British physician, initially described Hodgkin's disease, a rare lymphoma, a cancer of the lymphatic system, and distinguished this cancer from the more common non-Hodgkin's lymphomas. Today, most people diagnosed with Hodgkin's disease, Carson-DeWitt and Alic (2002) explain, can be cured. Prior to 1970, however, not many individuals survived Hodgkin's disease. In Hodgkin's disease, antibody-producing cells of the lymphatic system grow abnormally. Hodgkin usually develops in a lymph node and slowly spreads through the lymphatic vessels from one group of lymph nodes to the next in a generally predictable way. In some instances, the disease invades organs adjacent to the lymph nodes. When cancer cells spread to the blood, the disease can invade nearly every site in the body. "Advanced cases of Hodgkin's disease may involve the spleen, liver, bone marrow, and lungs." (Carson-DeWitt and Alic, 2002)
The following figure (2) denotes various subtypes of Hodgkin's disease:
Figure 2: Hodgkin's Disease Subtypes (adapted from Carson-DeWitt and Alic, 2002) Unclear Root Cause Although the root cause of Hodgkin has not been confirmed, abnormalities in a chest x-ray, due to nonspecific symptoms, frequently lead to the initial diagnosis of Hodgkin's disease. During a thorough physical examination, a physician not only notes the location of swollen lymph nodes, while examining their size, tenderness, and firmness, he/she considers whether the swollen nodes correlate with additional signs of infection. Hodgkin's disease more frequently affects lymph nodes of the neck, above the collarbone, under the arms, and in the chest above the diaphragm. Lymph nodes not shrinking after treatment with antibiotics may particularly present cause for concern. (Kaeseoglu; Senayli; Bicakci; Onuk-Filiz; Et al, 2007) to diagnose Hodgkin's disease, medical staff removes a complete enlarged lymph node (an excisional biopsy) or a tiny portion of a large tumor (an incisional biopsy).
To confirm a diagnosis of Hodgkin's disease, however, lab reports must confirm the presence of Reed-Sternberg cells (giant cells) in the sample of biopsied tissue. As affected lymph nodes may contain a minute number of Reed-Sternberg cells, sometimes difficult to recognize, other types of cells' characteristics in the biopsied tissue also help diagnose the subtype of Hodgkin's disease. Although instances are rare, at times to distinguish Hodgkin's disease from non-Hodgkin's lymphoma, diagnosticians use other tests which detect particular substances on cancer cells' surfaces or DNA cells' changes. (Kaeseoglu; Senayli; Bicakci; Onuk-Filiz; Et al, 2007) Most of the time, according to Kaeseoglu; Senayli; Bicakci; Onuk-Filiz; Et al (2007), Hodgkin's disease presents with lymph node or solid organ involvement, however, bone constitutes another potential origin of the disease. Radiographic evidence confirms bone involvement in 10-15% of cases. In 9% to 35% of Hodgkin's cases, particularly in later stages, bone is involved. Bone involvement, however, is rarely evident at presentation. In fact, Kaeseoglu; Senayli; Bicakci; Onuk-Filiz; Et al (2007) note that, to their knowledge, only 49 cases of Hodgkin's disease with bone involvement at the disease's onset have been reported. The prevalence of bone involvement occurs at later stages of the disease. The diagnosis of Hodgkin's disease, similar to numerous forms of cancer, includes two major components.
Identifying Hodgkin's lymphoma to be the origin of the individual's disease;
Staging of the disease to verify the area where the cancer spread. (Kaeseoglu; Senayli; Bicakci; Onuk-Filiz; Et al, 2007)
Suspected Causes and Symptoms of Hodgkin's Disease Although no single cause contributing to Hodgkin's disease has yet been confirmed, Kaeseoglu; Senayli; Bicakci; Onuk-Filiz; Et al, (2007) report that researchers suspect "some interaction between an individual's genetic makeup, environmental exposures, and infectious agents may be responsible. Immune system deficiencies also may be involved." Initial symptoms indicating Hodgkin's disease may resemble the flu and include:
fevers, night sweats, chills fatigue loss of appetite (anorexia) weight loss itching pain after drinking alcoholic beverages swelling of one or more lymph nodes (Kaeseoglu; Senayli; Bicakci; Onuk-Filiz; Et al, 2007)
Although some individuals present with similar symptoms, as many as 75% of individuals diagnosed with Hodgkin's disease, albeit, do not present with typical symptoms. (Kaeseoglu; Senayli; Bicakci; Onuk-Filiz; Et al, 2007) Some of the generalized symptoms denoting Hopkin's disease may sometimes be confused "with influenza, other infections, such as infectious mononucleosis ("mono"), tuberculosis (TB), or other cancers." (Hodgkin's Disease, 1998-2008) if a person experiences sudden high fever, loss of control of his/her bladder and/or bowel, and/or numbness in his/her arms and legs, and loss of strength, however, these symptoms should not be ignored as they may indicate emergency symptoms of Hodgkin's disease. (Kaeseoglu; Senayli; Bicakci; Onuk-Filiz; Et al, 2007) painless swelling in one or more of the neck, collarbone region, armpits, or groin lymph nodes depicts the most frequent symptom of Hodgkin's disease (HD), as well as, non-Hodgkin's lymphoma (NHL). When lymph nodes swell, in the majority of cases, particularly in children, an infection, not lymphoma, causes the swelling.
Generally, within a few weeks or months after an infection clears, affected lymph nodes return to their normal sizes. Frequently, physicians observe a patient's swollen lymph nodes during this time to determine changes in size following antibiotic treatment. If a lymph node(s) reaches more than one inch in size (> 1 in), however, accompanied by other suspicious symptoms, the physician sometimes performs an immediate biopsy to rule out or confirm a cancer diagnosis. (Hodgkin's Disease, 1998-2008) Effects on Abdomen When HD or NHL involves lymphatic tissue within a patient's abdomen, his/her belly may swell to the point in some females, it resembles pregnancy. Fluid may accumulate within the abdominal cavity, while swelling near the person's intestines may block his/her normal passage of feces, stimulating abdominal pressure or pain sensations. (Hodgkin's Disease, 1998-2008)
Effects on Chest When lymphoma involves thymus' lymphatic tissue, due to the thymus' location in front of the heart, chest pain may occur. An enlarged thymus may also exert pressure on nearby structures, "such as the trachea (windpipe) or superior vena cava (SVC), the large vein that carries blood from the head and arms back to the heart." (Hodgkin's Disease, 1998-2008) Effects on Trachea When swollen lymph nodes exert pressure on the trachea, the individual may develop a cough, become fatigues, be short of breath, and other respiratory difficulties. Pressure on the superior vena cava may produce SVC syndrome, a swelling of the head and arms. SVC syndrome involving the brain can be fatal and must be treated immediately. But enlarged lymphatic tissue in the chest cavity generally tends to displace -- rather than press upon or encase -- adjacent structures. Therefore, compromised breathing and SVC syndrome are relatively uncommon signs of lymphoma. (Hodgkin's Disease, 1998-2008)
Effects on Bone Marrow
Night sweats, fevers or anemia (a low red-blood-cell count), fevers may indicate Hodgkin's disease has spread to an individual's bone marrow. In these scenarios, a physician may order bone marrow aspiration and biopsy. In biopsy, medical staff uses a large needle to remove a narrow, cylindrical piece of the patient's bone. In another option, medical staff performs an aspiration, a process utilizing a needle to remove small bits of bone marrow. Generally, in both instances, to help determine cancer spread, physicians remove the marrow from the back of the patient's hip or other large bone. (Kaeseoglu; Senayli; Bicakci; Onuk-Filiz; Et al, 2007)
Other Extreme Effects Itching related to Hodgkin's disease produces much more severe sensations than those accompanying dry skin. In some reported cases, patients actually scratched through their skin with their fingernails attempting to secure some relief. Sometimes HD or NHL patients experience night sweats so severe they completely soak their sheets and blankets. (Hodgkin's Disease, 1998-2008)
Ages of Individuals and Prevalence of Hodgkin
Although most individuals do not develop Hodgkin's at any specific age, the majority are 15-40 years old and/or past 55 when diagnosed with this disease. Hodgkin is more prevalent in boys than girls, with approximately 10-15% of cases diagnosed in children under 17 years old. Hodgkin is rarely diagnosed in children under five years old. (Carson-DeWitt and Alic, 2002)
When Lymph Nodes Swell When the lymph nodes of a person with Hopkin's disease swell, they may exert pressure on other structures, potentially leading to a myriad of symptoms, such as:
pain due to pressure on nerve roots loss of function in muscle groups served by compressed nerves coughing or shortness of breath due to compression of the windpipe and/or airways, by swollen lymph nodes in the chest kidney failure from compression of the ureters, the tubes that carry urine from the kidneys to the bladder swelling in the face, neck, or legs, due to pressure on veins paralysis in the legs due to pressure on the spinal cord (Kaeseoglu; Senayli;
Bicakci; Onuk-Filiz; Et al, 2007)
As Hodgkin's disease advances, an individual's immune system weakens so that he/she can no longer effectively fight infection. In time, Hodgkin's lymphoma patients become more susceptible to bacteria-based infections, as well as, unusual (opportunistic) infections. In addition, symptomatic tumors later develop as Hodgkin's disease progresses. (Kaeseoglu; Senayli; Bicakci; Onuk-Filiz; Et al, 2007)
Hodgkin's Disease Stages Contemporary treatments for Hodgkin's disease reportedly possess serious short- and long-term side effects. With accurate staging, nevertheless, physicians and patients may frequently opt for minimum treatment that affectively cures this challenging disease. "The staging system for Hodgkin's disease is the Ann Arbor Staging Classification, also called the Cotswold System or the Revised Ann Arbor System." (Kaeseoglu; Senayli; Bicakci; Onuk-Filiz; Et al, 2007)
The following four stages denote stages and additional substages of Hodgkin's, along with effects from the disease:
Stage I: The disease is confined to one lymph node area
Stage IE: The disease extends from the one lymph node area to adjacent regions
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