Hodgkin's Disease

Hodgkin’s disease was first described in 1832. It is a disease of the immune system and is a very unique type of lymphoma. The exact cell of origin is unknown but is suspected to be a fused T and B cell. (Visit section on Cancer and The Immune System to understand these terms) The disease is diagnosed by the presence of a large double-nucleated cell with pink staining nucleoli, called the Reed-Sternberg cell. No cause for Hodgkin’s disease has been identified. The disease starts in the immune system and some researchers have argued that Hodgkin’s disease may be an atypical response to an infectious agent. The lymph nodes are the factory houses of the immune system. In these way stations, the immune system responds to an attack by the lymphocytes proliferating and producing new clones of cells. Some of these clones, called T-Lymphocytes, become active defense cells and others, called B-Lymphocytes, produce antibodies. Lymph nodes are distributed thought out the body, but are more plentiful in a line that runs, like a hedge, from the base of skull, down the neck and all the way down the backbone to the groin. Lymph nodes are plentiful in the neck and in either arm pith. The liver, bone marrow and spleen are also part of the immune system and can become involved in this disease. Presentation Patients who get this disease are usually between the ages of fifteen and thirty-five. It rarely occurs younger and seldom older. This disease starts by producing fevers and night sweats without obvious cause. They may have painless enlarged nodes in the neck, arm-pith or groin. The abdomen may feel distended due to an enlarging spleen. The skin may be diffusely itchy and dermatologists cannot tell why. Patients complain that when they drink alcohol, it hurts as it hits the stomach. Patients may loose weight for no obvious reason. How To Diagnose Hodgkin’s Disease The diagnosis is made under a microscope. A lymph node needs to be biopsied, removed, processed and examined by an experienced pathologist. Hodgkin’s can be confused with other types of lymphoma and other diseases, i.e.: sarcoma; Castleman’s disease; sarcoidosis; tuberculosis and mononucleosis. Once a diagnosis is made, the patient must next be staged. Staging tells how widespread the lymphoma is. Accurate staging is important because the treatment patients get is directly dependent on their stage. Staging starts with the physician doing a physical examination and then ordering radiological tests, i.e. PET Scans; Cat Scans, Bone Scans, and blood tests, i.e. complete blood count; sma-20; gamma-globulin levels, immune-flow cytometry and bone marrow evaluation. Occasionally, it may be necessary to do a laparotomy and remove the spleen to more accurately stage Hodgkin’s disease. When all these tests have been done, the patient can be accurately staged. The Staging of Hodgkin’s Disease Patients are categorized into four different stages and each stage is subclassifed into either A or B. A - is without symptoms. B - is with fevers or weight loss (more than 10%) or drenching night sweats. 1. In Stage 1, the lymph nodes in one part of the body are involved and the rest of the body’s lymph nodes are free of disease. 2. In stage 2, two different sets of lymph nodes on either side of the body’s middle i.e. the diaphragm, are involved. 3. In stage 3, a group of lymph nodes on either side of the diaphragm are involved. This may involved the spleen. 4. In stage 4, the disease is disseminated all over the body. 5. E-Stage. If the disease involves an organ outside the lymphoid tissue. 6. X-Stage. The term X is added to the stage if there is one area that has bulky disease. i.e. If the spleen is very large, or the center of the chest is packed with nodes, then X is added to the stage. There are four different pathological types of Hodgkin’s disease. The presence of the Reed- Sternberg cell in a lymph node or in lymphoid tissue makes the diagnosis. The distribution of the lymphocytes and their pattern of presentation around the Reed-Sternberg cells helps to sub-classify disease into four different categories. Type I. Nodular Lymphocyte Predominant Hodgkin’s Disease. (NLPHD) This variety makes up about 6% of all Hodgkin’s case. They present with enlarged nodes, without any fever, weight loss or itching. The lymph nodes involved are usually the peripheral ones and the nodes in the middle of the chest and those lying along the backbone are usually not involved. Some researchers believe that this disease may be a type of non-Hodgkin’s lymphoma because a percentage of them relapse as non-Hodgkin’s disease. They respond very well to treatment and most do not relapse at all. Type 2. Nodular Sclerosing Hodgkin’s Disease This type is so classified because, under the microscope, the lymph nodes have a particular nodular pattern and the Reed-Sternberg cells have a lacunar appearance --the cell’s nucleus looks like an island in a lake. Patients may present with enlarged lymph nodes in the neck or arm piths. Fevers, dry cough, and itching are usually present; night sweats and weight loss are usually absent. The lymph nodes in the center of the body (the mediastinun and the retro peritoneal space) are usually involved and become enlarged. Sometimes they occupy so much of the upper neck that they press on the veins that drain the face, obstruct them causing the face to swell.( This is called The Superior Mediastinal Syndrome) Type 3. Mixed Cellular Hodgkin’s disease In this type, the lymph nodes are fibrotic (rubbery) and the number of lymphocytes is decreased. The Reed-Sternberg cells are large and classically bilobar with a pink inclusion body in the nucleus. Thirty percent of Hodgkin’s cases present with this type. They can have fever, itching, night sweats, and pain on swallowing or weight loss. The lymph nodes that lie along the backbone and the spleen itself are frequently involved and can become quite large. Type 4. Lymphocyte Depleted Hodgkin’s disease In this type, under the microscope, the lymph nodes are sclerotic and most of the lymphocytes are absent. The Reed-Sternberg cells are plentiful and have the classical appearance. This is the least common variety and tends to occur in older people. The lymph nodes in the abdomen are usually involved, along with the liver, spleen and bone marrow. This is the most difficult to treat. The Immunology of Hodgkin’s Disease Antigens that appear on their cell walls distinguish lymphocytes from each other. B-Lymphocytes have gammo-globulins and special receptors; T-Cells have particular antigens, which distinguishes one population from another. These antigens are labeled with a CD-acronym. In Hodgkin’s disease, the lymphocytes have antigens, CD-15, CD-30 but are lacking CD-45. (See the Section entitled The Immune System and Cancer) The lymphocytes lack the common antigens of T and B cells. These particular immune markers help pathologists distinguish Hodgkin’s disease from other diseases. Treatment of Hodgkin’s Disease The treatment for Hodgkin’s disease depends on the stage. There are two main modalities of treatment. 1. Radiation therapy. 2. Chemotherapy. With these two modalities, most cases of Hodgkin’s disease are curable. Each patient is carefully staged as described above and then once the exact stage is known, a conference is held between the radiation therapist, the medical oncologist and the family doctor and a plan of action is taken. Early Stage 1A disease may be treated with radiation therapy alone; advanced Stage 4 b may be treated with radiation plus chemotherapy or chemotherapy alone. Since each treatment modality has long-term consequences, an attempt is made to treat the patient with the minimum of treatments. A discussion on what treatments to use for what stage of disease is beyond this discussion. If you wish to know more about this disease or the treatment modalities used to treat it, give my office a call for an appointment and I will be happy to further discuss, clarify and manage your particular disease.