Waldenström macroglobulinemia (WM) is a hematological malignancy involving lymphocytes. It is a type of nonaggressive non-Hodgkin lymphoma. It is also classified as a lymphoplasmacytic lymphoma. A distinguishing feature of WM is the presence of an IgM monoclonal protein (or paraprotein) that is produced by the cancer cells. It is named after the Swedish physician Jan G. Waldenström (1906-1996), who identified the condition. The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... Although hematological malignancies are a form of cancer, they are generally treated by specialists in hematology, although in many hospitals oncology specialists also manage these diseases. ... A lymphocyte is a type of white blood cell involved in the human bodys immune system. ... Non-Hodgkins lymphoma is a type of cancer. ... Schematic of antibody binding to an antigen An antibody is a protein complex used by the immune system to identify and neutralize foreign objects like bacteria and viruses. ... A paraprotein is an abnormal protein in the urine or blood, most often associated with multiple myeloma. ...

Clinical charateristics

WM is a rare disorder, with 1,400 cases occurring in the United States annually. Symptoms of WM include weakness, fatigue, weight loss and chronic oozing of blood from the nose and gums. Peripheral neuropathy can occur in 10% of patients. Some of these symptoms are due to the effects of the IgM paraprotein, which may cause autoimmune phenomenon or cryoglobulinemia. Peripheral neuropathy is the medical term for damage to nerves of the peripheral nervous system, which may be caused either by diseases of the nerve or from the side-effects of systemic illness. ... Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... Cryoglobulinemia is the presence of abnormal proteins in the bloodstream which thicken or gel on exposure to cold. ...

Unique to WM is the occurrence of the hyperviscosity syndrome. 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. Rarely this can lead to stroke or coma. Hyperviscosity syndrome is an increase in the viscosity of the blood. ... A stroke or cerebrovascular accident (CVA) occurs when the blood supply to a part of the brain is suddenly interrupted. ...

Median survival is approximately 5 years from time of diagnosis.

Treatment

Treatment includes the monoclonal antibody rituximab, sometimes in combination with chemotherapy like chlorambucil or cyclophosphamide. Corticosteroids are also used in combination. Rituximab, sold under the trade names Rituxan and Mabthera, is a monoclonal antibody used in the treatment of B cell Non-Hodgkins lymphoma, a kind of cancer. ... Chlorambucil (marketed as Leukeran) is a chemotherapy drug that has been mainly used in the treatment of chronic lymphocytic leukemia. ... Cyclophosphamide is a nitrogen mustard alkylating agent, used to treat various types of cancer and some autoimmune disorders. ...

Plasmapheresis can be used to treat the hyperviscosity syndrome. Plasmapheresis is the removal of (components of) blood plasma from the circulation. ...

See also

• Multiple myeloma