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Multiple myeloma
Most common plasma cell dyscrasia
Peak at 50-60 YOA, both sexes equally affected
Monoclonal IgG most frequently seen (55%), followed by IgA (25%) or light chain only (25%); serum concentrations of these are generally > 3 g/dL
Poor prognosis if Bence-Jones protein levels > 6 mg/dL from renal damage and generalized amyloidosis
May have “punched out bone lesions” or generalized osteoporosis; both lead to pathological fractures
Hypercalcemia from bone resorption may lead to confusion, weakness, lethargy, C, and PU
Believed to be a disease of hematopoietic stem cells
Suppression of normal Ig production can lead to recurrent infection with encapsulated bacteria
7% of patients have hyperviscosity (especially with monoclonal IgA since it forms polymers); see retinal hemorrhages, prolonged bleeding, and neurologic changes like H/A, dizziness, deafness, and stupor
Peripheral blood findings: normochromic and normocytic anemia, rouleaux formation, and rarely leukemic involvement (plasma cell leukemia)
Increased plasma cells in marrow in a diffuse or nodular pattern (must be discriminated from causes of reactive plasmacytosis)
Solitary plasmacytoma
Infrequent variant with an isolated mass of neoplastic cells in bone or soft tissue
Soft tissue plasmacytoma often cured with just surgery; osseous plasmacytoma progresses to multiple myeloma no matter what the therapy (may take up to 20 years to do so, though)
Called light-chain or heavy-chain disease in the absence of demonstrable associated heavy chains or light chains, respectively
Chromosome 14 problems have been seen (worse prognosis)
Prognosis depends on stage at time of diagnosis
Those with multiple bone lesions rarely live more than a year if untreated
May increase survival to 2-3 years with advanced cases using chemo with alkylating agents
Elevated IL-6 levels associated with poor prognosis (IL-6 causes proliferation and differentiation of myeloma cells)
Waldenstrom’s macroglobulinemia
Disease of old age (60 and 70 YOA)
Monoclonal IgM with diffuse infiltrate of “plasmacytoid lymphocytes” in the marrow, nodes, liver, and spleen
Lytic bony lesions are absent
Often seen with hyperviscosity because of larger size of IgM
May get things like Raynaud’s phenomenon or cold urticaria if abnormal Igs precipitate with cold temperatures
Average survival with appropriate chemo = 2-5 years
Heavy chain disease
κ chain disease
Most common type
Mostly in young adults, especially in the Mediterranean area
Massive infiltration of lamina propria of the intestine and abdominal nodes resulting in villous atrophy and severe malabsorption with D, steatorrhea, and hypocalcemia
Progressive and uniformly fatal
May occur as a complication of sprue
κλchain disease
In elderly where it resembles a malignant lymphoma with lymphadeopathy, anemia, and F, often also with malaise, weakness, and hepatomegaly or splenomegaly
Course is variable
λ chain disease
Rarest type
Often in patients with CLL
Hepatosplenomegaly is usually present, but peripheral lymphadeopathy is inconspicuous
Monoclonal gammopathy of uncertain significance
See monoclonal serum Ig without demonstrable evidence of defined plasma cell dyscrasia or lymphoproliferative syndrome
Seen in 1% of asymptomatic healthy folk over 50 YOA and in 3% of people > 65 YOA
Monoclonal serum protein usually of IgG with serum concentration <>
Bence Jones proteinuria is usually absent
About 20% of these eventually develop a plasma cell dyscrasia, malignant lymphoma, or amyloidosis
Normal Igs don’t decrease in number
Category: Medical Subject Notes , Pathology Notes
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