Waldenström's macroglobulinemia
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{{DiseaseDisorder infobox |
Name = Waldenström's macroglobulinemia |
ICD10 = {{ICD10|C|88|0|c|81}} |
ICDO = {{ICDO|9761|3}} |
ICD9 = {{ICD9|273.3}} |
OMIM = 153600 |
MedlinePlus = 000588 |
eMedicineSubj = med |
eMedicineTopic = 2395 |
DiseasesDB = 14030 |
MeshID = D008258 |
}}
'''Waldenström's macroglobulinemia''' (WM, also known as lymphoplasmacytic lymphoma) is cancer involving a subtype of white blood cells called [[lymphocytes]]. The main attributing antibody is [[IgM]]. It is a type of [[lymphoproliferative disease]], and shares clinical characteristics with the indolent [[non-Hodgkin lymphoma]]s.<ref name="Cheson">{{cite book | author = Cheson BD | year = 2006 | title = ACP Medicine | chapter = Chronic Lymphoid Leukemias and Plasma Cell Disorders | editor = Dale DD, Federman DD | publisher = WebMD Professional Publishing | location = New York, NY | id = ISBN 0974832715 }}</ref>
It is named after the Swedish physician [[Jan G. Waldenström]] (1906-1996), who first identified the condition.
==History and classification==
WM was first described by [[Jan G. Waldenström]] (1906-1996) in 1944 in two patients with bleeding from the nose and mouth, [[anemia]], decreased levels of [[fibrinogen]] in the blood (hypofibrinogenemia), [[lymphadenopathy|swollen lymph nodes]], neoplastic plasma cells in bone marrow, and increased [[viscosity]] of the blood due to increased levels of a class of heavy proteins called [[macroglobulins]].<ref name="Waldenstrom1944">{{cite journal | author= Waldenstrom J | title=Incipient myelomatosis or "essential" hyperglobulinemia with fibrinognenopenia-a new syndrome? | journal=Acta Med Scand| year=1944 | pages=216–247 | volume=117}} </ref>
For a period of time, WM was considered to be related to [[multiple myeloma]] due to the presence of monoclonal gammopathy and infiltration of the bone marrow and other organs by plasmacytoid lymphocytes. The new [[World Health Organization]] (WHO) classification, however, places WM under the category of lymphoplasmacytic lymphomas, itself a subcategory of the indolent (low-grade) non-Hodgkin lymphomas. <ref name="Harris">{{cite journal | author=Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, Lister TA, Bloomfield CD | title=The World Health Organization classification of neoplastic diseases of the haematopoietic and lymphoid tissues: Report of the Clinical Advisory Committee Meeting, Airlie House, Virginia, November 1997 | journal=Histopathology | year=2000 | pages=69–86 | volume=36 | issue=1 | pmid=10632755 | doi=10.1046/j.1365-2559.2000.00895.x}}</ref>
==Causes==
The underlying cause is not yet known. However, there has been an association demonstrated with the locus 6p21.3 on [[chromosome 6]].<ref name="pmid16938573">{{cite journal |author=Schop RF, Van Wier SA, Xu R, ''et al'' |title=6q deletion discriminates Waldenström macroglobulinemia from IgM monoclonal gammopathy of undetermined significance |journal=Cancer Genet. Cytogenet. |volume=169 |issue=2 |pages=150–3 |year=2006 |pmid=16938573 |doi=10.1016/j.cancergencyto.2006.04.009}}</ref>
==Epidemiology==
Of all [[lymphoproliferative disorders|cancers involving the same class of blood cell]], 1% of cases are WM.<ref name="isbn0-7817-5007-5">{{cite book
|author=Turgeon, Mary Louise
|title=Clinical hematology: theory and procedures
|publisher=Lippincott Williams & Wilkins
|location=Hagerstwon, MD
|year=2005
|pages=283
|isbn=0-7817-5007-5
|quote=Frequency of lymphoid neoplasms. (Source: Modified from WHO Blue Book on Tumour of Hematopoietic and Lymphoid Tissues. 2001, p. 2001.)
}}</ref>
WM is a rare disorder, with fewer than 1,500 cases occurring in the United States annually.<ref name="Cheson"> </ref> The median age of onset of WM is between 60 and 65 years, with some cases occurring in late teens.<ref name="Raje">{{cite book | author = Raje N, Hideshima T, Anderson KC | year = 2003 | title = Holland-Frei Cancer Medicine | chapter = Plasma Cell Tumors | edition=6th edition | editor = Kufe DW, Pollock RE, Weichselbaum RR, Bast RC, Gansler TS| publisher = B.C. Decker | location = New York, NY | id = ISBN 1550092138 }}</ref><ref name="Cheson"> </ref>
==Symptoms==
Symptoms of WM include [[weakness]], [[fatigue (physical)|fatigue]], [[weight loss]] and chronic oozing of blood from the nose and gums.<ref name="Kyle1988">{{cite book | author =Kyle RA | year = 1998 | title = Internal Medicine | chapter = Chapter 94: Multiple Myeloma and the Dysproteinemias | editor = Stein JH | edition = 5th ed. | publisher = C.V.Mosby | location = New York | id = ISBN 0815186983 }}</ref> [[Peripheral neuropathy]] can occur in 10% of patients. Lymphadenopathy, [[splenomegaly]], and/or [[hepatomegaly]] are present in 30-40% of cases.<ref name="Raje"> </ref> Some symptoms are due to the effects of the [[IgM]] [[paraprotein]], which may cause [[autoimmune]] phenomenon or [[cryoglobulinemia]]. Other symptoms of WM are due to the [[hyperviscosity syndrome]], which is present in 6-20% of patients.<ref name="Owen">{{cite journal | author=Owen RG, Barrans SL, Richards SJ, O'Connor SJ, Child JA, Parapia LA, Morgan GJ, Jack AS | title=Waldenstrom macroglobulinemia. Development of diagnostic criteria and identification of prognostic factors | journal=Am J Clin Pathol | year=2001 | pages=420–8 | volume=116 | issue=3 | pmid=11554171 | doi=10.1309/4LCN-JMPG-5U71-UWQB}}</ref><ref name="SanMiguel">{{cite journal | author=San Miguel JF, Vidriales MB, Ocio E, Mateo G, Sanchez-Guijo F, Sanchez ML, Escribano L, Barez A, Moro MJ, Hernandez J, Aguilera C, Cuello R, Garcia-Frade J, Lopez R, Portero J, Orfao A | title=Immunophenotypic analysis of Waldenstrom's macroglobulinemia | journal=Semin Oncol | year=2003 | pages=187–95 | volume=30 | issue=2 | pmid=12720134 | doi=10.1053/sonc.2003.50074}}</ref><ref name="Ghobrial">{{cite journal | author=Ghobrial IM, Witzig TE | title=Waldenstrom macroglobulinemia | journal=Curr Treat Options Oncol | year=2004 | pages=239–47 | volume=5 | issue=3 | pmid=15115652 | doi=10.1007/s11864-004-0015-5}}</ref><ref name="Dimopoulos">{{cite journal | author=Dimopoulos MA, Kyle RA, Anagnostopoulos A, Treon SP | title=Diagnosis and management of Waldenstrom's macroglobulinemia | journal=J Clin Oncol | year=2005 | pages=1564–77 | volume=23 | issue=7 | pmid=15735132 | doi=10.1200/JCO.2005.03.144}}</ref> This is attributed to the IgM monoclonal protein increasing the viscosity of the blood. Symptoms of this are mainly neurologic and can include blurring or loss of vision, headache, and (rarely) [[stroke]] or [[coma]].
==Diagnosis==
A distinguishing feature of WM is the presence of an IgM monoclonal protein (or paraprotein) that is produced by the cancer cells, and a concurrent decrease in levels of uninvolved [[immunoglobulins]] (i.e., [[IgG]] and [[IgA]]).
==Prognosis==
Current medical treatments result in survival some longer than 10 years. In part this is because better diagnostic testing means early diagnosis and treatments. Older diagnosis and treatments resulted in published reports of median survival of approximately 5 years from time of diagnosis.<ref name="Cheson"> </ref> New treatments have made longer term survival a reality for many with this condition. In rare instances, WM progresses to [[multiple myeloma]].<ref name="Johansson">{{cite journal | author=Johansson B, Waldenstrom J, Hasselblom S, Mitelman F | title=Waldenstrom's macroglobulinemia with the AML/MDS-associated t(1;3)(p36;q21) | journal=Leukemia | year=1995 | pages=1136–8 | volume=9 | issue=7 | pmid=7630185}}</ref>
==Treatment==
There is no single accepted treatment for WM. Indeed, in 1991, Waldenström himself raised the question of the need for effective therapy.<ref>{{cite journal | author=Waldenstrom J | title=To treat or not to treat, this is the real question | journal=Leuk Res | year=1991 | pages=407–8 | volume=15 | issue=6 | pmid=1907339 | doi=10.1016/0145-2126(91)90049-Y}}</ref> In the absence of symptoms, many clinicians will recommend simply monitoring the patient.
In 2002, a panel at the International Workshop on Waldenstrom Macroglobulinemia agreed on criteria for the initiation of therapy. They recommended starting therapy in patients with constitutional symptoms such as recurrent [[fever]], [[night sweats]], [[Fatigue (medical)|fatigue]] due to [[anemia]], [[weight loss]], progressive symptomatic [[lymphadenopathy]] or [[splenomegaly]], and [[anemia]] due to marrow infiltration. Complications such as hyperviscosity syndrome, symptomatic sensorimotor peripheral neuropathy, systemic [[amyloidosis]], [[renal insufficiency]], or symptomatic [[cryoglobulinemia]] were also suggested as indications for therapy.<ref name="Kyel2003">{{cite journal | author=Kyle RA, Treon SP, Alexanian R, Barlogie B, Bjorkholm M, Dhodapkar M, Lister TA, Merlini G, Morel P, Stone M, Branagan AR, Leblond V | title=Prognostic markers and criteria to initiate therapy in Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia | journal=Semin Oncol | year=2003 | pages=116–20 | volume=30 | issue=2 | pmid=12720119 | doi=10.1053/sonc.2003.50038}}</ref>
Treatment includes the monoclonal antibody [[rituximab]], sometimes in combination with chemotherapeutic drugs such as [[chlorambucil]], [[cyclophosphamide]], or [[vincristine]]. [[Corticosteroid]]s may also be used in combination. [[Plasmapheresis]] can be used to treat the hyperviscosity syndrome by removing the paraprotein from the blood, although it does not address the underlying disease.<ref name="Gertz">{{cite journal | author=Gertz MA | title=Waldenstrom macroglobulinemia: a review of therapy | journal=Am J Hematol | year=2005 | pages=147–57 | volume=79 | issue=2 | pmid=15929102 | doi=10.1002/ajh.20363}}</ref>
Recently, autologous [[bone marrow transplantation]] has been added to the available treatment options.<ref name="Yang">{{cite journal | author=Yang L, Wen B, Li H, Yang M, Jin Y, Yang S, Tao J | title=Autologous peripheral blood stem cell transplantation for Waldenstrom's macroglobulinemia | journal=Bone Marrow Transplant | year=1999 | pages=929–30 | volume=24 | issue=8 | pmid=10516708 | doi=10.1038/sj.bmt.1701992}}</ref><ref name="Martino">{{cite journal | author=Martino R, Shah A, Romero P, Brunet S, Sierra J, Domingo-Albos A, Fruchtman S, Isola L | title=Allogeneic bone marrow transplantation for advanced Waldenstrom's macroglobulinemia | journal=Bone Marrow Transplant | year=1999 | pages=747–9 | volume=23 | issue=7 | pmid=10218857 | doi=10.1038/sj.bmt.1701633}}</ref><ref name="Anagnostopoulos">{{cite journal | author=Anagnostopoulos A, Dimopoulos MA, Aleman A, Weber D, Alexanian R, Champlin R, Giralt S | title=High-dose chemotherapy followed by stem cell transplantation in patients with resistant Waldenstrom's macroglobulinemia | journal=Bone Marrow Transplant | year=2001 | pages=1027–9 | volume=27 | issue=10 | pmid=11438816 | doi=10.1038/sj.bmt.1703041}}</ref><ref name="Tournilhac">{{cite journal | author=Tournilhac O, Leblond V, Tabrizi R, Gressin R, Senecal D, Milpied N, Cazin B, Divine M, Dreyfus B, Cahn JY, Pignon B, Desablens B, Perrier JF, Bay JO, Travade P | title=Transplantation in Waldenstrom's macroglobulinemia--the French experience | journal=Semin Oncol | year=2003 | pages=291–6 | volume=30 | issue=2 | pmid=12720155 | doi=10.1053/sonc.2003.50048}}</ref>
==References==
{{Reflist|2}}
==External links==
*[http://www.iwmf.com/ The International Waldenström's Macroglobulinemia Foundation site]
*[http://www.cancer.gov/cancertopics/factsheet/Sites-Types/WM National Cancer Institute's Waldenström's Macroglobulinemia Q&A]
*[http://www.cancer.org/docroot/CRI/CRI_2_3x.asp?dt=76 American Cancer Society Detailed Guide: Waldenström's Macroglobulinemia]
*[http://www.waldenstromsresearch.org/ Research Fund for Waldenström's]
{{Hematological malignancy histology}}
[[Category:Rare cancers|Waldenström macroglobulinemia]]
[[Category:Blood disorders|Waldenström macroglobulinemia]]
[[Category:Types of cancer|Waldenström macroglobulinemia]]
[[Category:Hematology|Waldenström macroglobulinemia]]
[[de:Morbus Waldenström]]
[[fr:Maladie de Waldenström]]
[[nl:Ziekte van Waldenström]]
[[pl:Makroglobulinemia Waldenströma]]