Plasma cell disorders neoplastic proliferations of well-differentiated immunoglobulin-producing cells.

. These disorders include o multiple myeloma o Waldenstrm macroglobulinemia o benign monoclonal gammopathy o primary amyloidosis and heavy-chain (Franklin) disease. most frequent in persons older than 4050 years of age.

Multiple myeloma (plasma cell myeloma/plasmacytoma)

is a malignant plasma cell tumor usually affect older persons typically involves bone associated with prominent serum and urinary protein abnormalities. Clinical characteristics of multiple myeloma o Bone lesions and protein abnormalities The neoplastic cell is an end-stage derivative of B lymphocytes that is clearly identifiable as a plasma cell. The neoplastic cells can easily be identified by bone marrow biopsy or aspiration smears. The tumor cells produce lytic lesions in bone, especially in the skull and axial skeleton. Appearance of bone lesions lucent on radiographic examination, with characteristic sharp borders, and are referred to as punched-out lesions manifest radiographically as diffuse demineralization of bone (osteopenia).(MCQ) caused by an osteoclast-activating factor secreted by the neoplastic plasma cells. .(MCQ) associated with severe bone pain and spontaneous fractures. o Paraproteinemia Myeloma arises from proliferation of a single clone of malignant antibody-producing cells. tumor cells produce massive quantities of identical immunoglobulin molecules demonstrable electrophoretically as a narrow serum band or, after densitometric scanning, as a sharp spike referred to as an M protein. .(MCQ) M protein in multiple myeloma is most often an IgG or IgA immunoglobulin of either kappa or lambda light-chain specificity. (MCQ) Synthesis of normal immunoglobulins is often impaired. The marked serum immunoglobulin increase is often initially detected by laboratory screening as increased total protein with an increase in serum globulin .(MCQ) The urine often contains significant quantities of free immunoglobulin light chains, either kappa or lambda, which are referred to as Bence Jones protein. .(MCQ) As a consequence of hyperglobulinemia, the red cells tend to congregate together in a manner reminiscent of a stack of poker chips (rouleaux formation). .(MCQ) o There is also a marked increase in the ESR. .(MCQ) o Anemia

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due to neoplastic encroachment on myeloid precursor cells; Possible leukopenia and thrombocytopenia Increased susceptibility to infection Due to impaired production of normal immunoglobulins Hypercalcemia secondary to bone destruction in contrast to the increased serum alkaline phosphatase that accompanies most other instances of hypercalcemia, the serum alkaline phosphatase in multiple myeloma is not increased. .(MCQ) Renal insufficiency with azotemia due to myeloma kidney (myeloma nephrosis). The renal lesion is characterized by prominent tubular casts of Bence Jones protein numerous multinucleated macrophage-derived giant cells metastatic calcification interstitial infiltration of malignant plasma cells. Amyloidosis of the primary amyloidosis type .(MCQ)

m macroglobulinemia
a manifestation of lymphoplasmacytic lymphoma a B-cell neoplasm of lymphoid cells of an intermediate stage between B lymphocytes plasma cells referred to as plasmacytoid lymphocytes neoplastic cells produce a monoclonal IgM protein (lymphoplasmacytic lymphomas can also occur without protein production). .(MCQ) Defining characteristics o Serum IgM immunoglobulin of either kappa or lambda specificity occurring as an M protein .(MCQ) o Plasmacytoid lymphocytes infiltrating the blood, bone marrow, lymph nodes, and spleen o Bence Jones proteinuria in about 10% of cases o Absence of bone lesions.(MCQ) Clinical features o Most frequently seen in men older than 50 years of age o Slowly progressive course, often marked by generalized lymphadenopathy and mild anemia Complications o Hyperviscosity syndrome results from marked increase in serum IgM .(MCQ) retinal vascular dilation, sometimes with hemorrhage, confusion, central nervous system changes are seen emergency plasmapheresis is required to prevent blindness. o Abnormal bleeding, due to vascular and platelet dysfunction secondary to the serum protein abnormality o occurs in 5%10% of otherwise healthy older persons. o A monoclonal M protein spike of less than 2 g/100 mL.(MCQ) o minimal or no Bence Jones proteinuria

Benign monoclonal gammopathy (monoclonal gammopathy of undetermined significance, or MGUS)

o less than 5% plasma cells in the bone marrow .(MCQ) o no decrease in concentration of normal immunoglobulins is characteristic. o MGUS is most often without clinical consequence.