Part III • White Cell Disorders
Plasma cell leukemia is a complication of multiple myeloma in which there is an increased number of plasma cells in the circulating blood (Fig. 13.14). This condition is usually seen late in the progression of the logists disease as plasma cells overtake the normal bone mar- Patho row elements. I
Waldenstrom's macroglobulinemia was discovered in 1944 by the Swedish physician Dr. Jan Waldenstrom. His original presentation described two patients who had abnormal mucosal bleeding, enlarged lymph nodes, anemia, and thrombocytopenia. No bone pain was evident and both patients showed an elevated ESR. He described an abnormal protein that we now know is an overproduction of IgM, which presents as a particular spike on SPE and produces the hyperviscosity syndrome. Patients tend to be older, but the condition affects both men and women equally, with more whites than blacks being affected. The overproduction of globulin is caused by abnormal B lymphocytes that manifest in the bone marrow and peripheral smear as having features of plasma cells—thus, the name plasmacytoid lymphocytes. Clinical issues related to hyperviscosity feature largely in the complications experienced by these patients. Because IgM is such a large molecule, an overproduction of this macromolecule has the ability to coat platelets, impeding their function, interfering with coagulation factors, and causing potential neurological or thrombotic complications. Although there is no unique profile of symptoms in these patients, most
experience headaches, dizziness, visual problems, and serious coagulation difficulties. The peripheral smear may show rouleaux and plasmacytoid lymphocytes. As a subset of the abnormal IgM protein, cryoglobulins may form in some patients, which leads to Raynaud's phenomenon and bleeding. Chemotherapy is available for these patients, and plasmapheresis may be used as a means to reduce the IgM concentration. In the plasma-pheresis procedure, blood is removed from the patient, which separates the plasma from the cells. The cells are returned to the patient while the offending plasma, which contains the elevated IgM protein, is discarded. Treatment for many patients consists of plasmapheresis chemotherapy, immunotherapy with monoclonal antibodies, or possibly stem cell transplantation. Interferon may also be used to relieve symptoms. Table 13.7 provides a summary of the major lymphoproliferative disorders.
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