Myeloma

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Myeloma

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

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