Lymphoplasmacytic Disorders

By Alison Cole

Lymphoplasmacytic disorder is a disease that affects the lymphoplasmacyte cells that produce monoclonal light chained immunoglobulins, which are part of the immune system.

Light chain deposit disease is a lymphoplasmacytic disorder that has uncommon monoclonal gammopathy (IgG). The symptoms should be monitored carefully in patients who also have renal disease. The diagnosis of this disease is easy when monoclonal light chains are present in the serum or urine and the renal biopsy exhibits typical morphological changes and stains for kappa or lambda light chains. It becomes difficult to diagnose when the patient does not have a known lymphoplasmacytic disorder and the monoclonal light chains are detectable only erratically.

Waldenstrom¹s macroglobulinemia (WM) is a well-known malignant disorder of lymphoplasmacytic cells that produce a monoclonal immunoglobulin M (IgM). The standardized criterion that is now established for diagnosis of this disease, includes the presence of any IgM monoclonal protein and marrow and nodal lymphoplasmacytic cells in the blood and its hyperviscosity due to increased levels of a class of heavy proteins called macroglobulins. A distinctive feature of WM is the presence of an IgM monoclonal protein that is produced by the cancer cells, and a simultaneous decrease in levels of uninvolved immunoglobulins IgG and IgA.

Many symptoms are due to the effects of the IgM paraprotein that produces autoimmune phenomenon and due to the hyperviscosity syndrome, which is attributed to the IgM monoclonal protein increasing the viscosity of the blood. Symptoms of this are manifested mainly as neurologic symptoms and can include blurring or loss of vision, headache, and at times stroke.

Monocyte function is abnormal in patients with multiple myeloma and can be further reversibly inhibited by high paraprotein levels. Further research is needed to confirm and ascertain whether monocyte function can be normalized using chemotherapy or growth factors, and whether they could be used in the treatment of this currently incurable condition.

In recent years, after intense research and study, autologous bone marrow transplantation has been included to the available treatment options.

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