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Sindrome di POEMS Rita Emili Orvieto, Novembre 2009

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Presentation on theme: "Sindrome di POEMS Rita Emili Orvieto, Novembre 2009"— Presentation transcript:

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

2 Definizione Polyneuropathy Organomegaly Endocrinopaty
Monoclonal plasma cell disorder Skin changes Dispenzieri, Blood, 2003

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

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

5 IL-1β in POEMS syndrome RK Gherardi, Blood, 1994
(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 Minor criteria (plus at least one) Organomegaly
Edema Endocrinopathy Skin changes Sclerotic bone lesions Papilledema Castleman disease 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
POEMS patients – three series Mayo % n=99 French % n=25 Japanese % n =102 Peripheral neuropathy 100 Organomegaly 46 Ns Endocrinopathy 71 Monoclonal plasma cell dyscrasia 75 Skin Changes 68 Sclerotic bone lesions 97 54 Papilledema 29 40 55 Extravascular volume overload 39 Castleman disease 11 24 19 Other features Thrombocytosis 58 88 Polycythemia 18 12 Clubbing 5 32 49 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 Extravascular volume overload
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 Dispenzieri, Hematology, 2005
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 Talidomide Lenalidomide Bortezomib
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 Anti VEGF Anti TNF
(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 citochine proinfiammatorie 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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