multiple myeloma

    • Plasma cell disorders
      • neoplastic proliferations of well-differentiated immunoglobulin-producing cells.
      • These disorders include
        • multiple myeloma
        • Waldenström macroglobulinemia
        • benign monoclonal gammopathy
        • primary amyloidosis and heavy-chain (Franklin) disease.
      • most frequent in persons older than 40–50 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
        • 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.
      • 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)
      • There is also a marked increase in the ESR. .(MCQ)
      • Anemia
        • 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)
    • Waldenström 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
        • Serum IgM immunoglobulin of either kappa or lambda specificity occurring as an M protein .(MCQ)
        • Plasmacytoid lymphocytes infiltrating the blood, bone marrow, lymph nodes, and spleen
        • Bence Jones proteinuria in about 10% of cases
        • Absence of bone lesions.(MCQ)
      • Clinical features
        • Most frequently seen in men older than 50 years of age
        • Slowly progressive course, often marked by generalized lymphadenopathy and mild anemia
      • Complications
        • 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.
        • Abnormal bleeding,
          • due to vascular and platelet dysfunction secondary to the serum protein abnormality
    • Benign monoclonal gammopathy (monoclonal gammopathy of undetermined significance, or MGUS)
      • occurs in 5%–10% of otherwise healthy older persons.
      • A monoclonal M protein spike of less than 2 g/100 mL.(MCQ)
      • minimal or no Bence Jones proteinuria
      • less than 5% plasma cells in the bone marrow .(MCQ)
      • no decrease in concentration of normal immunoglobulins is characteristic.
      • MGUS is most often without clinical consequence.

    What is multiple myeloma?
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    Multiple myeloma is a type of cancer for which there is no cure. But treatment for this disease has improved greatly in recent years. Patients can live in remission for a long time. The man you’re about to meet was diagnosed with multiple myeloma last year, and after an intense battle, he is winning.