Plasma Cell Myeloma with concomitant chronic Lymphocytic Leukemia/monoclonal B-Cell Lymphocytosis
Multiple myeloma occurring concurrently with chronic Lymphocytic Leukemia or its pre-malignant precursor, monoclonal B-cell lymphocytosis, is an extremely rare condition in which patients evidence findings of the  plasma cell dyscrasia plus either one of the cited clonal lymphocytic diseases. Patients are typically elderly (median age of 74, range 42–91 years old) males (51 of 66 case reports) and commonly present with a combination of symptoms related to chronic lymphocytic leukemia symptoms (fatigue, autoimmune hemolytic anemia, enlargements of liver and/or spleen and lymphadenopathy) plus symptoms of multiple myelomas. Patients exhibit two distinct populations of clonal cells in their bone marrow, blood, and/or other tissues: plasma cells, which may have an immature plasmablastic morphology and small lymphocytes, which have a morphology typical of chronic lymphocytic leukemia cells. Patients blood and/or urine evidences a plasma cell-derived myeloma proteins, either IgG, IgA, or free light chain in ~50%, 20%, and 20% of cases, respectively, but may also have a second myeloma protein made by the lymphocytic cells, either an IgM or IgG. Signs and symptoms of chronic lymphocytic leukemia commonly precede those of multiple myeloma, sometimes by years. The relationship between the two clones of cells in this combined disease has not been established although one study suggests that the clonal plasma cells and clonal lymphocytes arise from a common hematological stem cell.
The syndrome is defined by the presence of; both of two major criteria, peripheral neuropathy and a clonal plasma cell dyscrasia (increased bone marrow plasma cells in ~67% of cases; ≥1 plasmacytoma in ~33% of cases); at least one other major criteria (Castlemans disease, sclerotic bone lesions, elevated serum levels of the cytokine VEGF); and at least one minor criterion (organomegaly, extravascular volume overload [e.g. ascites, edema, pleural effusion, and/or pericardial effusion], endocrinopathy [i.e. hypogonadism, defects in the hypothalamic–pituitary–adrenal axis], skin changes, papilledema, and/or hematological manifestations [i.e. thrombocytosis or polycythemia]). The monoclonal protein in POEMS patients is typically identified as IgA or IgG which in >95% of cases contains a λ chain that is restricted to either of two members of the V lambda 1 subfamily viz., IGLV1-40*01 and IGLV1-44*01 (there are 29 other members in the V lambda family). That is, the myeloma protein in POEMS is almost invariably a clonal λ light chain variant. Deletion of chromosome 13 and chromosomal translocations but not increases in chromosome number have also been reported to occur in POEMS patients.Patients with 1 or 2 isolated plasmacytomas have been successfully treated with targeted radiotherapy to obtain relief of symptoms and sometimes complete remission of disease. (Isolated plasmacytomas may regress spontaneously.) Patients with >2 plasmacytomas or symptomatic disseminated disease have been treated with chemotherapy often followed by autologous stem-cell transplantation; these treatments have been found to reduce symptoms of the disease and lead to long-term partial remissions of disease.