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Waldenstrom Myeloma

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  • Waldenstrom Myeloma

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    اخواني الكرام ... اقدم لكم موضوع متواضع عن داء Waldenstrom Myeloma ارجوا ان يفيدكم




    Waldenstrom Myeloma
    In 1944 Jan Gosta Waldenstrom (1906-1996), a Swedish physician, identified a rare condition in which patients experienced a thickening of the serum (the liquid portion) of the blood. He described two patients who had bleeding in the area of the mouth and nose as well as in the retina of the eye, low red cell and platelet counts, high sedimentation rate, and lymph node involvement. Bone marrow biopsy showed excess lymphoid cells, yet there was no bone pain, so it wasn't multiple myeloma. They had a large amount of some single unknown globulin with an extremely high molecular weight (which we now call IgM). They had, as he described in 1961, a "monoclonal gammopathy."
    Description from the National Cancer Institute
    Waldenstrom's macroglobulinemia is a rare, chronic cancer that is classified as a plasma cell neoplasm. It affects plasma cells, which develop from white blood cells called B-lymphocytes, or B cells.
    B cells form in the lymph nodes and the bone marrow, the soft, spongy tissue inside bones. They are an important part of the body's immune (defense) system. Some B cells become plasma cells, which make, store, and release antibodies. Antibodies help the body fight viruses, bacteria, and other foreign substances.
    In Waldenstrom's macroglobulinemia, abnormal plasma cells multiply out of control. They invade the bone marrow, lymph nodes, and spleen and produce excessive amounts of an antibody called IgM. Excess IgM in the blood causes hyperviscosity (thickening) of the blood.
    Waldenstrom's macroglobulinemia usually occurs in people over age 65, but can occur in younger people. A review of cancer registries in the United States found that the disease is more common among men than women and among whites than blacks.
    Some patients do not experience symptoms. Others may have enlarged lymph nodes or spleen, and may experience fatigue, headaches, weight loss, a tendency to bleed easily, visual problems, confusion, dizziness, and loss of co-ordination. These symptoms are often due to the thickening of the blood. In extreme cases, the increased concentration of IgM in the blood can lead to heart failure.
    The diagnosis of Waldenstrom's macroglobulinemia generally depends on the results of blood and urine tests and a bone marrow biopsy. During this test, a needle is inserted into a bone and a small amount of bone marrow is withdrawn and examined under a microscope.
    The initial treatment of Waldenstrom's macroglobulinemia is determined mainly by the thickness of the patient's blood. Patients with hyperviscosity usually receive chemotherapy (anti-cancer drugs). A type of treatment called plasmapheresis may be performed to relieve symptoms such as excessive bleeding and dizziness. In this procedure, blood is removed from the patient and circulated through a machine that separates the plasma (which contains the antibody IgM) from the other parts of the blood (red blood cells, white blood cells, and platelets). The red and white blood cells and platelets are returned to the patient, along with a plasma substitute. Interferon alpha, a biological therapy (a type of treatment that stimulates the immune system to fight cancer) can also help to relieve symptoms. For long-term control of the disease, doctors generally combine plasmapheresis with chemotherapy.
    Researchers continue to look for more effective ways to treat Waldenstrom's macroglobulinemia by conducting clinical trials (research studies) of new anti-cancer drugs, combinations of drugs, and new biological therapies. Information about ongoing clinical trials is available from the Cancer Information Service (see below) or from the National Cancer Institute's Cancer Trials® website at
    This fact sheet, from National Cancer Institute Information Resources, was reviewed on 1/20/99.

    Symptoms
    Many WM patients really show no symptoms. If the diagnosis comes relatively early in the game, it may be made quite by accident from a blood test given for some completely different reason. Even when symptoms appear, they may be totally different from one person to another. There is, in other words, no single way in which WM shows itself to the outside world. That said, there are certain recurring problems that physicians find in WM patients and that are usually mentioned in the textbooks:
    • Increased size of the spleen, the liver, and some lymph nodes
    • Tiredness, usually the result of anemia (too few red blood cells)
    • A tendency to bleed easily and to bruise easily (too few platelets)
    • Night sweats
    • Headaches and dizziness
    • Various visual problems
    • Pain or numbness in the extremities
    Needless to say, those are symptoms of many other maladies as well. Only a thorough work-up by a hematologist, usually including a bone marrow biopsy, can determine whether one has WM or something else.

    Hyperviscosity syndrome
    At least 50% of individuals with WM have hyperviscosity syndrome, an increased viscosity or thickening of the blood caused by the accumulation of IgM in the serum. Hyperviscosity can cause a slowing in the circulation through small blood vessels. This condition can lead to a variety of symptoms:
    • Fatigue
    • Weakness
    • Rash
    • Bruising
    • Nose bleeds
    • Gastrointestinal bleeding
    • Weight loss
    • Night sweats
    • Increased and recurrent infections
    • Poor blood circulation in the extremities
    . Poor blood circulation, or Raynaud's phenomenon, can affect any part of the body, but particularly the fingers, toes, nose, and ears.
    Cold weather can cause additional circulatory problems, by further thickening the blood and slowing down circulation. In some cases, the excess blood protein may precipitate out of the blood in the cold, creating particles that can block small blood vessels. This is called cryoglobulinemia. The extremities may turn white, or a patchy red and white. The hands, feet, fingers, toes, ears, and nose may feel cold, numb, or painful.
    Hyperviscosity may affect the brain and nervous system, leading to additional symptoms. These symptoms include:
    • Peripheral neuropathy, caused by changes in the nerves, leading to pain or numbness in the extremities
    • Dizziness
    • Headaches
    • Vision problems or loss of vision
    • Mental confusion
    • Poor coordination
    • Temporary paralysis
    • Mental changes
    Hyperviscosity can clog the tubules that form the filtering system of the kidneys, leading to kidney damage or kidney failure. Existing heart conditions can be aggravated by WM. In extreme cases, WM may result in heart failure. Late-stage WM also may lead to mental changes that can progress to coma.
    Anemia
    The accumulation of IgM in the blood causes an increase in the volume of the blood plasma. This effectively dilutes out the red blood cells and other blood components. The lowered concentration of red blood cells can lead to anemia and cause serious fatigue. Likewise, a deficiency in platelets (thrombocytopenia), which cause the blood to clot, can result in easy bleeding and bruising. As the cancer progresses, there may be abnormal bleeding from the gums, nose, mouth, and intestinal tract. There may be bluish discoloration of the skin. In the later stages of the disease, leukopenia, a deficiency in white blood cells, also can develop.

    Organ involvement
    In 5-10% of WM cases, the IgM may be deposited in tissues. Thus, some individuals with WM have enlargement of the lymph nodes, the spleen, and/or the liver.
    If Bence-Jones proteins are produced by the malignant plasma cells, they may be deposited in the kidneys. There they can plug up the tiny tubules that form the filtering system of the kidneys. This can lead to kidney damage and kidney failure
    Prognosis
    As soon as one is told he or she has an incurable disease, especially if the word "cancer" appears in its description, the question arises, "How long have I got, Doc?" In the case of WM, we don't really know. Everyone's disease is different from every other case that has ever been seen. This is a disease of one's genetic makeup having gone awry and no two of us are built on the same genetic platform. It is also a treatable disease and new and better treatments are constantly being found. One of the purposes of the IWMF is to provide funding, direction, and a pool of willing experimental subjects to the end that development of better treatments will finally result in a true cure.
    Traditionally, the textbooks have predicted a life span of five to seven years after diagnosis. But that wisdom is changing. And this is, after all, a purely statistical figure. Let it be said that we have members in the IWMF who have known for 25 years that they have WM and who are still functioning normally. The wise physician, asked that question, may give you the five-to-seven year figure, but you may also be told that you are more likely to die with WM than from it.

    Treatment
    The IWMF does not prescribe or recommend specific treatment regimens; that is the job you pay your hematologist to perform. The fact is, there is no government-approved course of treatment. Waldenstrom's macroglobulinemia is what is often called an "orphan" disease. Because it is so rare, not enough research has been done specifically on WM to justify the establishment of a prescribed course of treatment. Instead, physicians look at what works in closely related diseases such as multiple myeloma and CLL, and then try to use similar approaches on WM. That is one of the reasons we have begun to give out research funds to those researchers who are willing to look specifically at Waldenstrom's macroglobulinemia.
    While we do not recommend any specific course of treatment, the IWMF has put together Treatment Options, A Handbook for Patients for the benefit of patients and their physicians that describes common methods used by doctors to treat WM patients. It can be obtained from our office at 3932D Swift Road, Sarasota, FL 34231.
    One more piece of advice: WM is so rare that many hematologists have never seen a case. That in itself may not be a negative. But be certain that the person treating you is willing to find and to compare notes with a physician who has had WM experience. The disease can be difficult to diagnose and challenging to treat. A second or even a third professional and expert opinion is always recommended prior to treatment.
    Summary
    Waldenstrom’s macroglobulinemia is an extremely rare blood cancer that is treatable, but with present medical technology, incurable. WM is a serious disease, but slow moving (indolent), and immediate treatment is seldom indicated. Unlike other cancers, which can be "staged," there is no scientific evidence that early treatment of WM will increase survival time. Thus, most treatments are directed at symptoms. Those of us who have the disease most often have time to study treatments and implications, to explore the newest of more effective clinical trials, to support research aimed at a cure, and to live a full life.



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    Dr: Nebal Metwali
    Syria - Damascus

  • #2
    جزاك الله خيرا موضوع مفيد و جديد بالنسبة لى
    :sm188:وفوق كل ذى علم عليم:sm188:

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    • #3
      المشاركة الأصلية بواسطة د.عبير مشاهدة المشاركة
      جزاك الله خيرا موضوع مفيد و جديد بالنسبة لى
      مشكورة على الدخول... والحمد الله انك انتفعت به

      والله الموفق
      Dr: Nebal Metwali
      Syria - Damascus

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