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GAMMOPATIE MONOCLONALI. ACETATO DI CELLULOSA ROSSO PONCEAU.

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Presentation on theme: "GAMMOPATIE MONOCLONALI. ACETATO DI CELLULOSA ROSSO PONCEAU."— Presentation transcript:

1 GAMMOPATIE MONOCLONALI

2 ACETATO DI CELLULOSA ROSSO PONCEAU

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5 ACETATO CELLULOSA AGAROSIO

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9 STABLE PROGRESSIVE

10 BENIGN MONOCLONAL GAMMOPATHY DOES EXIST ? frequency Percentage of neoplastic transformation M. Boccadoro DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

11 ASYMPTOMATIC NO OSTEOLYTIC LESIONS M-COMPONENT BENIGN MONOCLONAL GAMMOPATHY DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

12 STABLE PROGRESSIVE MONOCLONAL GAMMOPATHIES MDD NN DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

13 Multistep cancerogenesis of myeloma Normal plasma cell MonoclonalgammopathyMyelomaExtra-medullarymyelomaChromosometranslocation IgH switch region Kariotipic instability K, N-ras, P53 mutations DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

14 Incidence of Multiple Myeloma, macroglobulinemia, amyloidosis after recognition of M-component Kyle R.A., Baillieres Clin Hematol, 1995 DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

15 BENIGN MONOCLONAL GAMMOPATHY MONOCLONAL GAMMOPATHY OF UNDETERMINED SIGNIFICANCE MGUS MONOCLONAL GAMMOPATHY OF UNDETERMINED SIGNIFICANCE MGUS DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

16 FREQUENCY OF MONOCLONAL GAMMOPATHIES GAMMOPATHIES Related to the sensitivity of the method Related to the sensitivity of the method DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

17 1-2 % normal population > 10 g/L Detected by standard methods Detected by sensitive methods ~ 10 % normal population < 5 g/L MONOCLONAL GAMMOPATHIES DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

18 - Out-patients referred for routine laboratory tests - Ospedale Evangelico Valdese, Torino - Laboratorio analisi (Director: M. Saitta) - routine agarose electrophoresis (Hydrasis Ciampolini) (Hydrasis Ciampolini) - period: Genuary- May serum samples analysed monoclonal gammopathies detected (4.2%) OCCURRENCE OF MONOCLONAL GAMMOPATHIES IN A GENERAL HOSPITAL DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

19 FREQUENCY OF MONOCLONAL GAMMOPATHY ACCORDING TO AGE % DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

20 DISTRIBUTION OF M-COMPONENT CONCENTRATION (g/L) Aguzzi et al, Eur J Haematol, 1992 DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

21 DISTRIBUTION OF M-COMPONENT CONCENTRATION (g/L) DISTRIBUTION OF M-COMPONENT CONCENTRATION (g/L) DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY Ospedale Evangelico Valdese, Torino

22 MONOCLONAL GAMMOPATHY PROBABILITY OF TRANSFORMATION TO A MALIGNANT DISEASE Evaluated in patient series: Diagnosis 1960s - 70s Standard electrophoresis M-component at presentation >15 g/L DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

23 MONOCLONAL GAMMOPATHY PROBABILITY OF TRANSFORMATION FOR PATIENTS WITH M-component > <30 g/L DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

24 Neoplastic transformation is related to the M-component concentration MONOCLONAL GAMMOPATHY IgG<15 g/L 1.3% transformation after 6 years (Baldini, 1996) IgG > 50 g/L require chemotherapy IgG > 30 g/L transformation within 1 year (Dimopoulos, 1993) IgG > < 30 g/L 26% transformation after 10 years (Kyle, 1995) DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

25 BENIGN MONOCLONAL GAMMOPATHY A pre-neoplastic disorder? - 5% frequency M-component - incidence of myeloma 2-4/ /year 1 out of M-component is transformed to myeloma every year DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

26 MONOCLONAL GAMMOPATHIES Related to M-component level Related to M-component level DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY TRANSFORMATION TO MYELOMA

27 BENIGN MONOCLONAL GAMMOPATHY MONOCLONAL GAMMOPATHY OF UNDETERMINED SIGNIFICANCE MULTIPLE MYELOMA ~ 70% < 10 g/L ~ 4-6% g/L 30 g/L DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

28 BENIGN MONOCLONAL GAMMOPATHY DOES EXIST? PROSPECTIVE LARGE STUDIES ON PATIENTS WITH SMALL M-COMPONENTS ARE REQUIRED PROBABLY YES DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

29 The New England Journal of Medicine DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

30 Multistep cancerogenesis of myeloma Normal plasma cell MonoclonalgammopathyMyelomaExtra-medullarymyelomaChromosometranslocation IgH switch region Kariotipic instability K, N-ras, P53 mutations DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY

31 TRANSFORMATION BENIGN PRE-NEOPLASTIC FROMTO

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34 ITALIAN MULTIPLE MYELOMA STUDY GROUP A. Pileri Principal investigators A. Palumbo P. Omedè M.Ladetto M. Massaia B. Bruno S. Battaglio Investigators: S. Bringhen, A. Bertola, G. Aitoro, F. Cavallo, P. Falco, L. Giaccone. R. Ghignone, F. Giaretta, F. Morrone, M. Ruggeri DIVISIONE UNIVERSITARIA DI EMATOLOGIA AZIENDA OSPEDALIERA SAN GIOVANNI TORINO, ITALY


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