Waldenström macroglobulinemia
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| ICD-10 | C88.0 |
|---|---|
| ICD-9 | 273.3 |
| ICD-O: | M9761/3 |
| OMIM | 153600 |
| DiseasesDB | 14030 |
| MedlinePlus | 000588 |
| eMedicine | med/2395 |
Waldenström macroglobulinemia (WM) is cancer involving a subtype of white blood cells called lymphocytes. It is a type of lymphoproliferative disease, and shares clinical characteristics with the indolent non-Hodgkin lymphomas (Cheson, 2006).
It is named after the Swedish physician Jan G. Waldenström (1906-1996), who first identified the condition.
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[edit] 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), 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 (Waldenstrom, 1944).
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. (Harris et al, 2000).
[edit] Epidemiology
WM is a rare disorder, with fewer than 1,500 cases occurring in the United States annually (Cheson et al, 2006). The median age of onset of WM is between 60 and 65 years (Raje et al, 2003;Cheson et al, 2006).
[edit] Symptoms
Symptoms of WM include weakness, fatigue, weight loss and chronic oozing of blood from the nose and gums (Kyle, 1998). Peripheral neuropathy can occur in 10% of patients. Lymphadenopathy, splenomegaly, and/or hepatomegaly are present in 30-40% of cases (Raje et al, 2003). 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 (Owen et al, 2001; San Miguel et al, 2003; Ghobrial et al, 2004; Dimopoulos et al, 2005). 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.
[edit] 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).
[edit] Prognosis
Median survival is approximately 5 years from time of diagnosis (Cheson et al, 2006). New treatments have made longer term survival a reality for many with this condition. In rare instances, WM progresses to multiple myeloma (Johansson et al, 1995).
[edit] Treatment
There is no single accepted treatment for WM. Indeed, in 1991, Waldenström himself raised the question of the need for effective therapy. 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 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 (Kyle et al, 2003).
Treatment includes the monoclonal antibody rituximab, sometimes in combination with chemotherapeutic drugs such as chlorambucil, cyclophosphamide, or vincristine. Corticosteroids 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 (Gertz, 2005). Recently, autologous bone marrow transplantation has been added to the available treatment options (Yang et al, 1999; Martino et al, 1999; Anagnostopoulos et al, 2001; Tournilhac et al, 2003).
[edit] References
- Anagnostopoulos A, Dimopoulos MA, Aleman A, Weber D, Alexanian R, Champlin R, Giralt S (2001). "High-dose chemotherapy followed by stem cell transplantation in patients with resistant Waldenstrom's macroglobulinemia". Bone Marrow Transplant 27 (10): 1027-9. PMID 11438816.
- Cheson BD (2006). "Chronic Lymphoid Leukemias and Plasma Cell Disorders", in Dale DD, Federman DD: ACP Medicine. New York, NY: WebMD Professional Publishing. ISBN 0974832715.
- Dimopoulos MA, Kyle RA, Anagnostopoulos A, Treon SP (2005). "Diagnosis and management of Waldenstrom's macroglobulinemia". J Clin Oncol 23 (7): 1564-77. PMID 15735132.
- Gertz MA (2005). "Waldenstrom macroglobulinemia: a review of therapy". Am J Hematol 79 (2): 147-57. PMID 15929102.
- Ghobrial IM, Witzig TE (2004). "Waldenstrom macroglobulinemia". Curr Treat Options Oncol 5 (3): 239-47. PMID 15115652.
- Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, Lister TA, Bloomfield CD (2000). "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". Histopathology 36 (1): 69-86. PMID 10632755.
- Johansson B, Waldenstrom J, Hasselblom S, Mitelman F (1995). "Waldenstrom's macroglobulinemia with the AML/MDS-associated t(1;3)(p36;q21)". Leukemia 9 (7): 1136-8. PMID 7630185.
- Kyle RA (1998). "Chapter 94: Multiple Myeloma and the Dysproteinemias", in Stein JH: Internal Medicine, 5th ed., New York: C.V.Mosby. ISBN 0815186983.
- Kyle RA, Treon SP, Alexanian R, Barlogie B, Bjorkholm M, Dhodapkar M, Lister TA, Merlini G, Morel P, Stone M, Branagan AR, Leblond V (2003). "Prognostic markers and criteria to initiate therapy in Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia". Semin Oncol 30 (2): 116-20. PMID 12720119.
- Martino R, Shah A, Romero P, Brunet S, Sierra J, Domingo-Albos A, Fruchtman S, Isola L (1999). "Allogeneic bone marrow transplantation for advanced Waldenstrom's macroglobulinemia". Bone Marrow Transplant 23 (7): 747-9. PMID 10218857.
- Owen RG, Barrans SL, Richards SJ, O'Connor SJ, Child JA, Parapia LA, Morgan GJ, Jack AS (2001). "Waldenstrom macroglobulinemia. Development of diagnostic criteria and identification of prognostic factors". Am J Clin Pathol 116 (3): 420-8. PMID 11554171.
- Raje N, Hideshima T, Anderson KC (2003). "Plasma Cell Tumors", in Kufe DW, Pollock RE, Weichselbaum RR, Bast RC, Gansler TS: Holland-Frei Cancer Medicine, 6th edition, New York, NY: B.C. Decker. ISBN 1550092138.
- 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 (2003). "Immunophenotypic analysis of Waldenstrom's macroglobulinemia". Semin Oncol 30 (2): 187-95. PMID 12720134.
- 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 (2003). "Transplantation in Waldenstrom's macroglobulinemia--the French experience". Semin Oncol 30 (2): 291-6. PMID 12720155.
- Waldenstrom J (1944). "Incipient myelomatosis or "essential" hyperglobulinemia with fibrinognenopenia-a new syndrome?". Acta Med Scand 117: 216-247.
- Waldenstrom J (1991). "To treat or not to treat, this is the real question". Leuk Res 15 (6): 407-8. PMID 1907339.
- Yang L, Wen B, Li H, Yang M, Jin Y, Yang S, Tao J (1999). "Autologous peripheral blood stem cell transplantation for Waldenstrom's macroglobulinemia". Bone Marrow Transplant 24 (8): 929-30. PMID 10516708.
