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Sindrome di POEMS

Sindrome di POEMS. Orvieto, 20-22 Novembre 2009 Rita Emili S.C. Oncoematologia, A.O. S. Maria, Terni Università degli Studi di Perugia. Definizione. P olyneuropathy O rganomegaly E ndocrinopaty M onoclonal plasma cell disorder S kin changes. Dispenzieri, Blood, 2003. Epidemiologia.

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Sindrome di POEMS

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  1. Sindrome di POEMS Orvieto, 20-22 Novembre 2009 Rita Emili S.C. Oncoematologia, A.O. S. Maria, Terni Università degli Studi di Perugia

  2. Definizione • Polyneuropathy • Organomegaly • Endocrinopaty • Monoclonalplasma cell disorder • Skinchanges Dispenzieri, Blood, 2003

  3. Epidemiologia • Età media : 50/60 anni • Popolazione colpita : Giapponesi, Europei, Africani, Ispanici, Asiatici Dispenzieri, Orphanet Enc., 2005

  4. Patogenesi sconosciute • CAUSE : Aumento del livello delle citochine: VEGF, IL-1β, TNF-α, IL-6 RK Gherardi, Blood, 1994

  5. IL-1β in POEMS syndrome (A) Angiofollicular lymph node hyperplasia with atrophic germinal centers (arrowheads) and sinusal histicytosis (stars) (B) In situ hybridization performed with IL-p sense probe used as control showing no signal (C) In situ hybridization performed with IL-p anti-sense probe showing abundant radiolabeledI L-1p mRNA-producing cells scatteredin the interfollicular spaces (D) In the other studied patient, radiolabeledI L-1p mRNA-producing cellsw ere similarly distributed in the interfollicular spaces RK Gherardi, Blood, 1994

  6. Criteri di diagnosi • Major criteria (both) • Polyneuropathy • Monoclonal plasma cell-proliferative disorder Dispenzieri, Blood, 2003

  7. Criteri di diagnosi • Organomegaly • Edema • Endocrinopathy • Skin changes • Sclerotic bone lesions • Papilledema • Castleman disease • Minor criteria (plus at least one) Dispenzieri, Blood, 2003

  8. Criteri di diagnosi • Known associations • Weight loss • Thrombocytosis • Polycythemia • Hyperhidrosis • Clubbing Dispenzieri, Blood, 2003

  9. Criteri di diagnosi • Possible associations • Restrictive lung disease • Thrombotic diatheses • Arthralgias • Cardiomyopathy (systolic dysfunction) • Fever • Low vitamin B12 values • Diarrhea • Pulmonary hypertension Dispenzieri, Blood, 2003

  10. Caratteristiche cliniche alla diagnosi Clinical features present at diagnosis in 99 patients with POEMS seen at the Mayo Clinic from 1975 to 1998 Dispenzieri, Blood, 2003

  11. Caratteristiche cliniche alla diagnosi Ns – not stated Dispenzieri, Blood, 2003

  12. Polyneuropathy • Cerebrospinal fluid protein more than 50 mg/dlt • Peripheral neuropathy: Distal, symmetric, progressive • paresthesias • coolness • tingling • Motor involvement follows the sensory syntoms. Dispenzieri, Blood, 2003

  13. Organomegaly • Hepatomegaly • Splenomegaly • Lymphadenopaty(between 11%-30% Castelman disease presents) Dispenzieri, Blood, 2003

  14. Endocrinopathy • Diabetes mellitus • Hypothyroidism • Hyperparathyroidism • Gonadal axis abnormality • Adrenal axis abnormality Dispenzieri, Blood, 2003

  15. Monoclonal plasma proliferative disorder • Serum M-spike (gm/dL) : usually < 2 • Monoclonal heavy chain : IgG > IgA > IgM • Monoclonal light chain : Lambda > 95% • BM plasma cells % : usually < 5 Dispenzieri, Blood, 2003

  16. Skin changes • Hyperpigmentation • Acrocyanosis and plethora • Hemangioma/telangectasia • Hypetrichosis • Thickening Dispenzieri, Blood, 2003

  17. Other features 1 • Sclerotic bones lesions • mixed sclerotic and lytic • osteosclerotic only • lytic only • > 1 lesion • Extravascular volume overload • Peripheral edema • Ascites • Pleural effusion Dispenzieri, Blood, 2003

  18. Other features 2 • Papilledema • Trombocytosis • Polycythemia • Clubbing • Weight loss more 10 pounds • Fatigue Dispenzieri, Blood, 2003

  19. Overall survival A. Overall survival in 99 patients receiving conventional dose chemotherapy. B. After peripheral blood stem cell transplant. Dispenzieri, Hematology, 2005

  20. Overall survival Dispenzieri, Blood, 2003

  21. Trattamento Dispenzieri, Blood, 2003

  22. Trattamento • Radioterapia : singole o multiple lesioni osteosclerotiche in una limitata area Dispenzieri, Blood, 2003

  23. Trattamento • Terapia sistemica : diffuse lesioni osteosclerotiche • Agenti alchilanti • Corticosteroidi • Chemioterapia ad alte dosi con trapianto di cellule staminali • Talidomide • Lenalidomide • Bortezomib Dispenzieri, Blood, 2003

  24. Trattamento Nuovi Agenti • Talidomide • Lenalidomide • Bortezomib ? ? ? ? ? ? Dispenzieri, Blood, 2003

  25. Trattamento • Talidomide: 200mg/die-300mg/die • Anti VEGF • Anti TNF • Immunomodulatore Dispenzieri, Blood, 2003

  26. Trattamento • Lenalidomide: 15-25 mg/die x 21 giorni (in associazione a desametasone) • Anti VEGF • Anti TNF • Immunomodulatore Dispenzieri, Blood, 2003

  27. Trattamento • Bortezomib: 1,3-1,6 mg/mq • Inibitore del proteosoma • Inibitore di citochineproinfiammatorie e proangiogeniche Dispenzieri, Blood, 2003

  28. High -dose chemotherapy with hematopoietic stem cell transplantation 1 Dispenzieri, Blood, 2003

  29. High -dose chemotherapy with hematopoietic stem cell transplantation 2 Dispenzieri, Blood, 2003

  30. Conclusioni • Diagnosi – presenza di due criteri maggiori e almeno uno minore • Diagnosi differenziale – mieloma multiplo, malattia di Waldenström, crioglobulinemia, amiloidosi primaria • Terapia – radioterapia e chemioterapia • Sopravvivenza – migliore rispetto ai pazienti con mieloma multiplo. Dispenzieri, Blood, 2003

  31. Grazie per l’attenzione

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