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Myeloma
 

Myeloma

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    Myeloma Myeloma Presentation Transcript

    • Immunosecretory Disorders www.freelivedoctor.com
    • Immunosecretory Disorders
      • tend to occur in middle aged to elderly people
      • monoclonal proliferation of B-cells
      www.freelivedoctor.com
    • 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)
      www.freelivedoctor.com
    • The amount of paraprotein produced is roughly proportional to the size of the proliferating cells www.freelivedoctor.com
    • 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
      www.freelivedoctor.com
    • 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
      www.freelivedoctor.com
    • 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
      www.freelivedoctor.com
    • 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 www.freelivedoctor.com
    • 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
      www.freelivedoctor.com
    • Multiple Myeloma (Plasma Cell Myeloma) genetic abnormality
        • deletion 13q
        • rearrangement 14q
        • balanced translocation t(4;14)(p6.3;q32)
      www.freelivedoctor.com
    • Multiple Myeloma (Plasma Cell Myeloma) clinical course
      • infection, renal failure, hemorrhage
      • progressive course
      • death within 2-5 yrs
      www.freelivedoctor.com
    • Multiple Myeloma (Plasma Cell Myeloma) Ig secretion
      • 52% Ig G
      • 18% Ig A
      • 11% Ig M
      • 1% Ig D
      • rare Ig E
      www.freelivedoctor.com
    • 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
      www.freelivedoctor.com
    • 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
      www.freelivedoctor.com
    • 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
      www.freelivedoctor.com
    • 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
      www.freelivedoctor.com
    • 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)
      www.freelivedoctor.com
    • 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
      www.freelivedoctor.com
    • 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
      www.freelivedoctor.com
      • 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) www.freelivedoctor.com
      • 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) www.freelivedoctor.com