Waldenström macroglobulinemia

Waldenström macroglobulinemia (WM) is cancer involving a subtype of white blood cells called lymphocytes. It is a type of lymphoplasmacytic lymphoma (LPL), and shares clinical characteristics with nonaggressive non-Hodgkin lymphoma (Cheson, 2006).

It is named after the Swedish physician Jan G. Waldenström (1906-1996), who 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), swollen lymph nodes (lymphadenopathy), increased numbers of lymphocytes in the bone marrow (lymphocytosis), and hyperviscosity 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 WHO classification, however, places WM under the category of lymphoplasmacytic lymphomas (Harris et al, 2000).

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).

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.

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

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).

Treatment

There is no single accepted treatment for WM. Indeed, in 1991, Waldenström himself raised the question of the need for 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 chemotherapy like chlorambucil or cyclophosphamide. Corticosteroids may also be used in combination. Plasmapheresis can be used to treat the hyperviscosity syndrome (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).

References

External links

Rare diseases | Blood disorders | Types of cancer | Hematology

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