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myeloma_06-07.ppt Presentation Transcript

  • 1. Immunosecretory Disorders Masatoshi Kida, M.D. Dept. of Pathology University of Vermont
  • 2. Immunosecretory Disorders
    • tend to occur in middle aged to elderly people
    • monoclonal proliferation of B-cells
  • 3. Immunosecretory Disorders
    • tend to occur in middle aged to elderly people
    • monoclonal proliferation of B-cells
    • secretion of Ig or portions of Ig (paraproteins, M component)
  • 4. The amount of paraprotein produced is roughly proportional to the size of the proliferating cells
  • 5. Immunosecretory Disorders
    • tend to occur in middle aged to elderly people
    • monoclonal proliferation of B-cells
    • secretion of Ig or portions of Ig (paraproteins, M component)
    • generally behave in a malignant fashion
  • 6. Immunosecretory Disorders
    • I. Monoclonal Gammopathies of Unknown Significance
    • (“Benign” Monoclonal Gammopathies)
    • II. Multiple Myeloma (Plasma Cell Myeloma)
    • III. Variant Forms of “Myeloma”
    • A. smoldering multiple myeloma
    • B. plasma cell leukemia
    • C. non-secretory myeloma
    • D. osteosclerotic myeloma (POEMS syndrome)
    • E. solitary plasmacytoma of bone
    • F. extramedullary plasmacytoma
    • G. primary macroglobulinemia (lymphoplasmacytic lymphoma)
    • (Waldenstrom’s macroglobulinemia)
    • H. heavy-chain disease
  • 7. Monoclonal Gammopathies of Unknown Significance (“Benign” Monoclonal Gammopathies)
    • presence of M protein in asymptomatic individual
    • 1-2% of adults over 30 y/o (usually over 50 y/o)
    • 90% are of IgG
    • stable for >3 yr
    • BM: plasma cells are <15% of all cells
    • ~20% evolves into overt myeloma in 10-15 yr
    • occasionally associated with a. carcinomas, lymphomas & leukemias b. other immune disorders
  • 8. Multiple Myeloma (Plasma Cell Myeloma)
    • most common form of plasma cell malignancy
    • extensively involves BM
    • middle aged to elderly male
    • Sx: bone pain osteolytic lesions fractures severe osteoporosis (osteoclast activating factor)
    most common primary bone tumor in adult
  • 9. Multiple Myeloma (Plasma Cell Myeloma)
    • most common form of plasma cell malignancy
    • extensively involves BM
    • middle aged to elderly male
    • Sx: bone pain osteolytic lesions fractures severe osteoporosis (osteoclast activating factor)
    • monoclonal protein spikes
    • Bence-Jones proteins (Ig light chains) in the urine
    • plasmacytosis/multiple plasma cell aggregates
  • 10. Multiple Myeloma (Plasma Cell Myeloma) genetic abnormality
      • deletion 13q
      • rearrangement 14q
      • balanced translocation t(4;14)(p6.3;q32)
  • 11. Multiple Myeloma (Plasma Cell Myeloma) clinical course
    • infection, renal failure, hemorrhage
    • progressive course
    • death within 2-5 yrs
  • 12. Multiple Myeloma (Plasma Cell Myeloma) Ig secretion
    • 52% Ig G
    • 18% Ig A
    • 11% Ig M
    • 1% Ig D
    • rare Ig E
  • 13. Variant Forms of “Myeloma”
    • smoldering multiple myeloma
    • plasma cell leukemia
    • non-secretory myeloma
    • osteosclerotic myeloma
    • solitary plasmacytoma of bone
    • extramedullary plasmacytoma
    • primary macroglobulinemia (lymphoplasmacytic lymphoma)
    • heavy-chain disease
  • 14. smoldering multiple myeloma
    • serum M-protein >3g/dL
    • >10% plasma cells in BM
    • no anemia, renal insufficiency or skeletal lesion
    • Clinical feature:
    • starting as “benign” monoclonal gammopathy
    • transforms into multiple myeloma need to be closely observed over time
    • Treatment:
    • must not be treated unless progression occurs
  • 15. plasma cell leukemia
    • >20% plasma cells in peripheral blood
    • absolute plasma cell count at least 2000/  L
    • Clinical feature:
    • primary (60%) : diagnosed in leukemic phase
        • younger age
        • hepatosplenomegaly, lymphadenopathy
        • longer survival (median 6.8 mo)(vs 1.3 mo)
    • secondary (40%) : following previous MM
    • Treatment:
    • generally unsatisfactory, but some response to melphalan and prednisone
    • secondary plasma cell leukemia rarely responds to chemotherapy
  • 16. non-secretory myeloma
    • no M-protein in serum or urine
    • account for 1 to 2% of myeloma
    • less renal involvement
    • Treatment:
    • similar response to chemotherapy as “secretory” myeloma
  • 17. osteosclerotic myeloma (POEMS syndrome)
    • P olyneuropathy, O rganomegaly, E ndocrionopathy, M -protein, S kin change
    • Clinical feature:
    • chronic inflammatory-demyelinating polyneuropathy with motor disability
    • sclerotic skeletal lesions
    • hepatomegaly (~50%)
    • Treatment:
    • radiation for localized lesion
    • chemotherapy (melphalan and prednisone)
  • 18. solitary plasmacytoma of bone
    • solitary bone lesion (histologically similar to MM) (vertebrae, pelvis, femur, humerus)
    • no M-protein in serum or urine
    • Clinical feature:
    • 50% alive at 10 yrs
    • 15 to 25% disease-free at 10 yrs
    • progression into MM within 3 to 4 yrs
    • Treatment:
    • radiation therapy
    • no significant effect with chemotherapy
  • 19. extramedullary plasmacytoma
    • lesions outside BM
    • Clinical feature:
    • upper respiratory tract involvement (~80%) (nasal cavity, sinuses, nasopharynx, larynx)
    • may also seen in GI tract, CNS, U bladder, thyroid, breast, testes, parotid gland, lymph nodes
    • may be solitary, may metastasize
    • rarely transforms into MM
    • Treatment:
    • radiation therapy
  • 20.
    • caused by uncontrolled proliferation of lymphocytes and plasma cells (hybrid of lymphoplasmacytic lymphoma/leukemia and multiple myeloma) (lymphoplasmacytic lymphoma)
    • Clinical feature:
    • age : ~65 yrs, 60% male
    • normocytic, normochromic anemia (moderate to severe)
    • weakness, fatigue, bleeding (oozing from oronasal area), pallor, hepatosplenomegaly, lymphadenopathy, sensorimotor peripheral neuropathy
    • no destructive skeletal lesions
    • hyperviscosity syndrome (retinal hemorrhage, transient paresis, mental confusion, CHF, bleeding)
    primary macroglobulinemia (Waldenstrom’s macroglobulinemia)
  • 21.
    • Treatment:
    • should not be treated unless symptomatic
    • chemotherapy (chlorambucil)
    • RBC transfusion (for anemia)
    • plasmapheresis (for hyperviscosity)
    • Prognosis:
    • median survival : 5 yrs
    primary macroglobulinemia (Waldenstrom’s macroglobulinemia)
  • 22.  
  • 23.