Waldenström's Macroglobulinemia

Waldenström's Macroglobulinemia

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Waldenström's Macroglobulinemia is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • macroglobulinemia
  • Waldenstrom's syndrome

Disorder Subdivisions

  • None

General Discussion

Waldenström's macroglobulinemia (WMG) is a malignant disorder of the blood, a type of lymphoma and characterized by the presence of abnormally large numbers of a particular kind of white blood cell known as B lymphocytes. As these cells accumulate in the body, excessive quantities of an antibody known as IgM are produced. Large amounts of IgM causes the blood to become thick (hyperviscosity) and affects the flow of blood through the smaller blood vessels, leading to the symptoms of the disorder. The organs fed by these small blood vessels do not receive sufficient blood and oxygen, potentially resulting in partial or complete failure of the organ.

Symptoms

Symptoms of Waldenström's macroglobulinemia usually begin gradually. Common symptoms are fatigue and loss of energy due to anemia. Bleeding from the nose and gums may also occur, and tingling in the fingers and toes is often seen (peripheral neuropathy). Examination may also reveal enlarged liver, spleen, or lymph nodes. Blurring or vision impairment may also occur due to changes in blood flow through the blood vessels that serve the retina. The symptoms are quite variable depending on the effect of the thickened (viscous) blood on the organ involved.

Causes

The causes of Waldenström's macroglobulinemia are unknown. There is evidence of occurrence within families, but such occurrence is uncommon.

Affected Populations

Waldenström's macroglobulinemia is a very rare disorder affecting about 1 in 3.4 million American men and about half that number of American women. The incidence of WMG is estimated to be about 5 per 1,000,000 people over the age of 50. The median age at diagnosis is 63. However, cases have been diagnosed in patients in their twenties. This disorder is considerably less common among people of African descent.

Standard Therapies

Diagnosis

When patients show symptoms of an enlarged spleen and liver combined with bleeding of the retina, WMG is reasonably suspected. The results of a complete blood count (CBC) usually show low red blood cell counts as well as low platelet counts. In such circumstances, electrophoresis (subjecting blood plasma to an electric impulse) of serum samples will show a peak reading for IgM.



Treatment

With asymptomatic patients, a period of watchful waiting is considered the preferred course. To reduce the blood thickness, plasmapheresis, a method of blood exchange which filters out much of the IgM protein, has been shown to be effective in delaying the need for more aggressive treatment. The blood cells are separated from the liquid component (serum or plasma). The heavy IgM molecules that cause the trouble are discarded with the liquid fraction of the exchange. The red blood cells are "reconstituted" by adding previously donated plasma or a plasma substitute. If the disorder progresses, red blood cell transfusions and/or white blood cell transfusions and/or platelet infusions may be necessary.



Traditional chemotherapeutic agents, particularly chlorambucil (Leukeran), cyclophosphamide (Cytoxan), and/or melphalan (Alkeran) either as single agents or in combination with others, have been used in the management of this disease for over 40 years. The monoclonal antibody, rituximab, has been found to be effective. Ongoing research is examining combinations of these drugs. Many new therapies have been introduced in the past few years and include fludarabine, cladribine, bortezomib,lenalidomide, bendamustine and everolimus.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com



A search of the Clinical Trials database maintained by the National Institutes of Health at www.clinicaltrials.gov yields 55 trials for WMG.



Peripheral blood stem transplant and bone marrow transplant, using the patient's own stem cells or marrow and that of related or unrelated donors, has been used but the procedure remains highly controversial.

References

TEXTBOOKS

Kipps TJ. Macroglobulinemia. In Lichtman MA, Beutler E, Kipps TJ, Selisohn U, et al. eds. Williams Hematology. 7th ed. New York, NY: McGraw-Hill Companies; 2006:1549-60.



Longo DL, Anderson KC. Plasma cell disorders. In: Kasper, DL, Fauci AS, Longo DL, et al. Eds. Harrison's Principles of Internal Medicine. 16th ed. New York, NY: McGraw-Hill Companies; 2005:656, 661.



Gertz MA. Waldenström Macroglobulinemia. In: NORD Guide to Rare Disorders. Philadelphia, PA: Lippincott Williams & Wilkins; 2003:37-38.



Berkow R., ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:1009-1010.



Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:964-65.



JOURNAL ARTICLES

Gertz MA. Waldenström macroglobulinemia: a review of therapy. Am J Hematol. 2005;79(2):147-57.



Lin P, Medeiros LJ. Lymphoplasmacytic lymphoma/Waldenström macroglobulinemia: an eveolving concept. Adv Anat Pathol. 2005;12:246-55.



Robak T, Korycka A, Kasznicki M, et al. Purine nucleoside analogues for the treatment of hematological malignancies: pharmacology and clinical applications. Curr Cancer Drug Targets. 2005;5:421-44.



Dimopoulos MA, Anagnostopoulos A. Waldenström's macroglobulinemia. Best Pract Res Clin Haematol. 2005;18:689-707.



Gertz MA. Waldenström macroglobulinemia: a review of therapy. Am J Hematol. 2005;79:147-57.



Dimopoulos MA, Kyle RA, Anagnostopoulos A, et al. Diagnosis and management of Waldenström's macroglobulinemia. .J Clin Oncol. 2005;23:1564-77.



Bjorkholm M. Treatment options for Waldenström's macroglobulinemia. Clin Lymphoma. 2005;5:155-62.



INTERNET

American Cancer Society. Waldenström Macroglobulinemia. http://documents.cancer.org/acs/groups/cid/documents/webcontent/003148-pdf.pdf. Last Updated January 31, 2012. Accessed March 20, 2012.



Macroglobulinemia of Waldenström. Medical Encyclopedia. Medline Plus. http://www.nlm.nih.gov/medlineplus/ency/article/000588.htm. Updated April 30, 2010. Accessed March 20, 2012./



National Cancer Institute. Waldenström Macroglobulinemia: Questions and Answers..

www.cancer.gov/cancertopics/factsheet/Sites-Types/WM. Updated September 25, 2007. Accessed March 20, 2012.

Resources

American Cancer Society, Inc.

250 Williams NW St

Ste 6000

Atlanta, GA 30303

USA

Tel: (404)320-3333

Tel: (800)227-2345

TDD: (866)228-4327

Internet: http://www.cancer.org



NIH/National Heart, Lung and Blood Institute

P.O. Box 30105

Bethesda, MD 20892-0105

Tel: (301)592-8573

Fax: (301)251-1223

Email: nhlbiinfo@rover.nhlbi.nih.gov

Internet: http://www.nhlbi.nih.gov/



National Cancer Institute

6116 Executive Blvd Suite 300

Bethesda, MD 20892-8322

USA

Tel: (301)435-3848

Tel: (800)422-6237

TDD: (800)332-8615

Email: cancergovstaff@mail.nih.gov

Internet: http://www.cancer.gov



International Waldenstrom's Macroglobulinemia Foundation

6144 Clark Center Ave.

Sarasota, FL 34238

USA

Tel: (941)927-4963

Fax: (941)927-4467

Email: info@iwmf.com

Internet: http://www.iwmf.com



Rare Cancer Alliance

1649 North Pacana Way

Green Valley, AZ 85614

USA

Internet: http://www.rare-cancer.org



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Madisons Foundation

PO Box 241956

Los Angeles, CA 90024

Tel: (310)264-0826

Fax: (310)264-4766

Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



Friends of Cancer Research

1800 M Street NW

Suite 1050 South

Washington, DC 22202

Tel: (202)944-6700

Email: info@focr.org

Internet: http://www.focr.org



Cancer.Net

American Society of Clinical Oncology

2318 Mill Road Suite 800

Alexandria, VA 22314

Tel: (571)483-1780

Fax: (571)366-9537

Tel: (888)651-3038

Email: contactus@cancer.net

Internet: http://www.cancer.net/



Cancer Support Community

1050 17th St NW Suite 500

Washington, DC 20036

Tel: (202)659-9709

Fax: (202)974-7999

Tel: (888)793-9355

Internet: http://www.cancersupportcommunity.org/



Lance Armstrong Foundation

2201 E. Sixth Street

Austin, TX 78702

Tel: (512)236-8820

Fax: (512)236-8482

Tel: (877)236-8820

Email: media@livestrong.org

Internet: http://www.livestrong.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

This information does not replace the advice of a doctor. Healthwise, Incorporated disclaims any warranty or liability for your use of this information. Your use of this information means that you agree to the Terms of Use . How this information was developed to help you make better health decisions.

Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.