Sindrome di POEMS-Malattie rare
Table of Contents
Definizione
Lasindrome di POEMSnoto anche come mieloma osteosclerotico,è un raro disordine paraneoplastico associato alla presenza di unadiscrasia plasmacellulare.In inglese Polyneuropathy(polineuropatia), Organomegaly,(organomegalia), Endocrinopathy(endocrinopatia), Monoclonal gammopathy(gammopatia monoclonale)Skin changes(alterazioni cutanee). La patogenesi di questo disordine è oscura, ma è caratteristico il notevole aumento dei livelli sierici dicitochine genetiche e proinfiammatorie, in particolare del VEGF, i cui livelli correlano con l’ attività della malattia e che potrebbero avere un ruolo nella patogenesi della neoplasia e delle sue manifestazioni.La sindrome di POEMS , insorge nella quinta-
Sintomatologia
Il quadro clinico è di solito dominato da unapolineuropatia cronicaprogressiva sensitivo-motoria che si manifesta con:
- debolezza muscolare di braccia e gambe
- formicolii
- intorpidimento
- dolore urente
- deficit della sensibilità
Le alterazioni cutanee sono rappresentate da:
- pigmentazione
- discromie
- angiomi
- dita a bacchetta di tamburo
Circa l’ 84% dei pazienti presenta un’ endocrinopatia, anche multipla, spesso costituita da:
- ipogonadismo
- atrofia testicolare
- ginecomastia nei maschi
- amenorrea
- ipertricosi nelle donne
Meno frequenti sono le alterazioni del metabolismo tiroideo e insufficienza surrenale. Nel 30% dei pazienti compaiono:
- edemi declivi
- ascite
- versamenti pleurici
All’esame obiettivo si riscontrano:
- epatomegalia(nel 50% dei soggetti)
- splenomegalia
- linfoadenopatia
Diagnosi
Criteri diagnostici per la sindrome di POEMS
- Disordine proliferativo plasmacellulare monoclonale
- Polineuropatia
Uno tra i seguenti:
- Lesioni osteosclerotiche
- Malattia di Clastelmanistologicamente accertata
- Organomegalia(spenomegalia, epatomegalia o linfoadenopatia)
- Endocrinopatia( surrenale, tiroidea, ipofisaria, gonadica paratiroidea,pancreatica, a esclusione e diabete mellito e anomalie tiroidee)
- Sovraccarico di volume extravascolare(edema, versamento pleurico, ascite)
- Alterazioni cutanee(iperpgmentazione, ipertricosi, emangiomi glomeruloidi, pletora, acrocianosi, flushing, alterazioni ungheali)
- Papilledema
Gli esami di laboratorio evidenziano spesso la presenza di trombocitosi, talvoltepolicitemia;anemiae piastrinopenia sono caratteristiche solo in caso di cesistente malattia di malattia di Castleman.
Decorso e prognosi
La sindrome di POEMS ha un decorso tendenzialmente cronico, con una sopravvivenza mediana superiore a 10 anni. La tempestività della diagnosi è cruciale per trattare a prevenire l’insorgenza di una grave sequela neurologica definitiva.
Terapia
La radioterapia mirata sui siti coinvolti è terapia d’elezione nei casi in cui predominano isolate lesioni osteosclerotiche. In presenza di lesioni diffuse, di coinvolgimento sistemico prominente, di mancata risposta alla radioterapia, il trattamento più efficace è costituito da regimi chemioterapiciseguite da trapianto ematopoietico di cellule staminali autologo. La sopravvivenza a cinque anni è di circa il 60%.
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