La linfangioleiomiomatosi (LAM) e una malattia rara ad eziologia sconosciuta che affligge solamente soggetti di sesso femminile. Essa coinvolge soprattutto i polmoni ove, come si evince dal nome, i dotti linfatici (linf-), i vasi sanguigni (-angio-) e le vie aeree, sono circondati da proliferazione di muscolo liscio (-leiomio) (Johnson S. Rare diseases. 1. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms. Thorax. 1999;54(3):254-64).La LAM e una malattia caratterizzata dalla anormale proliferazione di cellule muscolari lisce (cellule muscolari con aspetto immaturo - cellule di LAM) (Lymphatic obstruction leads to chylothorax and chylous ascites Chu SC, Horiba K, Usuki J, Avila NA, Chen CC, Travis WD, Ferrans VJ, Moss J. Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis. Chest. 1999;115(4):1041-52) in sede peribronchiale, perivascolare e perilinfatica, su tutto l'ambito polmonare (Ryu JH, Doerr CH, Fisher SD, Olson EJ, Sahn SA. Chylothorax in lymphangioleiomyomatosis. Chest. 2003;123(2):623-7).Altresi queste cellule muscolari formano degli affastellamenti e crescono nello spessore delle pareti delle vie aeree portando ad ostruzione delle vie stesse. Sebbene queste cellule non siano considerate cancerose esse crescono all'interno dei polmoni senza l'usuale controllo proliferativi (Scott-Emuakpor AB, Warren ST, Kapur S, Quiachon EB, Higgins JV. Familial occurrence of congenital pulmonary lymphangiectasis. Genetic implications. Am J Dis Child. 1981;135(6):532-4).

La malattia generalmente nasce spontaneamente sebbene in alcuni casi si sviluppa in soggetti affetti da sclerosi tuberosa (Johnson S. Rare diseases. 1. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms. Thorax. 1999;54(3):254-64). I pazienti si presentano piu comunemente con dispnea (59%), pneumotorace (49%) e tosse (39%) a seconda delle varie casistiche.Dolore toracico (22%), versamento pleurico chiloso (13%), emottisi (18%), e respiro affannoso sono meno comuni. La Chiloptisi si puo presentare con sputo bianco e schiumoso a seguito di pleurodesi, e i pazienti potrebbero lamentarsi di occasionale gorgoglio intratoracico (probabilmente dovuto alla rottura di cisti). Manifestazioni extrapolmonari, per un terzo dei pazienti, sono caratterizzate dalla presenza di masse linfonodali, masse cistiche di tessuto lasso e asciti chilose.Fibromi uterini (leomiomi) e angiomiolipomi renali (linfonodi addominali e pelvici potrebbero essere coinvolti e circa la meta dei pazienti avere angiomiolipomi renali) si sono altresi visti; gli angiomiolipomi sono spesso asintomatici sebbene si possano verificare sanguinamenti (Johnson S. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms. Thorax. 1999;54:254-264).

La storia naturale della linfangioleiomiomatosi vede una progressiva ostruzione delle vie aeree che conduce ad insufficienza respiratoria e cuore polmonare. La percentuale di progressione e altamente variabile fra i pazienti, variando da un rapido declino in pochi anni, a malattia asintomatica della durata di due o tre decadi (Johnson S. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms. Thorax. 1999;54:254-264). L'effetto distruttivo delle metalloproteinasi espresse dalle cellule di LAM si pensa contribuisca allo sviluppo delle cisti. L'ostruzione delle venule polmonari causa congestione venosa e distruzione portando ad emorragia polmonare ed emosiderosi. L'ostruzione linfatica conduce a chilotorace e ad asciti chiose (Ryu JH, Doerr CH, Fisher SD, Olson EJ, Sahn SA. Chylothorax in Lymphangioleiomyomatosis. Chest. 2003;123:623-627).La gravidanza in taluni casi puo essere correlata all'aumentato rischio di pneumotorace ed eventuale accelerazione del corso naturale della malattia, anche se altre non incorrono in complicazioni legate alla gravidanza. Le pazienti dovrebbero essere informate della evenienza, ovviamente prima del concepimento (Johnson S. Rare diseases. 1. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms. Thorax. 1999;54(3):254-64).

Gli organi affetti dalla malattia sono tutti infiltrati da cellule di LAM. Tali cellule originano dal muscolo liscio ed esprimono recettori per estrogeni e progesterone e contengono inclusioni tipiche pre-melanosomiali che possono essere marcate con anticorpo HMB-45. Sebbene l'origine della cellule sia controverso questo inusuale fenotipo e altresi distribuito in elementi del muscolo liscio dell'angiomiolipoma e nel raro tumore a cellule chiare del polmone. Le cellule di LAM sono anche presenti nella sclerosi tuberosa associata alla LAM e mutazioni nel gene TSC-2 sono state dimostrate nei polmoni, linfonodi e angiomiolipomi di donne con malattia sporadica e con malattia associata alla sclerosi tuberosa. Diversamente dalle donne con sclerosi tuberosa associata, pazienti con malattia sporadica difficilmente hanno altre manifestazioni della sclerosi ne mutazioni nella linea germinale nel gene TSC-2. Ad ogni modo non si sa ancora se le mutazioni del gene TSC-2 siano presenti in tutte le donne con LAM o se altri meccanismi possano intervenire. Il gene TSC-2 codifica per una tuberina che inibisce mTOR (target mammifero della Rapamicina). L'mTOR e una molecola chiave del segnale di attivazione alla risposta allo stimolo di fattori di crescita via fosforochinasi o altri segnali. Cellule senza la tuberina hanno un'attivazione costitutiva di mTOR e proliferano molto piu rapidamente delle normali cellule. Da ricordare che la Rapamicina, un farmaco normalmente usato per l'immunosoppressione, puo inibire l'mTOR e aumentare la crescita nelle cellule deficienti di tuberina e i processi verso la LAM e l'angiomiolipoma vengono avviati. Recentemente l'osservazione che mutazioni identiche sono distribuite tra lesioni renali e polmonari ha portato alla formulazione dell'ipotesi che le cellule LAM polmonari possano avere un'origine da siti a distanza e di conseguenza definire la LAM polmonare una malattia metastatica (Karbowniczek M, Astrinidis A, Balsara BR, Testa JR, Lium JH, Colby TV, McCormack FX, Henske EP. Recurrent lymphangiomyomatosis after transplantation: genetic analyses reveal a metastatic mechanism. Am J Respir Crit Care Med. 2003;167: 976-982).

La diagnosi dovrebbe essere sospettata in donne con dispnea o asma associate a pneumotorace, emottisi, alterati radiogrammi del torace, enfisema senza una storia pregressa di tabagismo, deficienza di Alfa 1-Antitripsina, o quando accade una malattia interstiziale del polmone con ostruzione delle vie aeree. Biopsie a polmone aperto hanno rappresentato il gold standard nella diagnosi della linfangioleiomiomatosi. La sensibilita e specificita dell'analisi istologica e considerevolmente migliorata con l'uso di anticorpi monoclinali HMB-45 i quali, nel polmone, marcano solamente le cellule di LAM, cosi facendo escludono quelle condizioni che possono mimare la linfangioleiomiomatosi come ad esempio il sarcoma metastatico dell'endometrio. Tali biopsie possono determinare lo stato recettoriale ormonale o se predomina la proliferazione muscolare liscia o la degenerazione cistica, ma se l'evidenza clinica di questi campi d'indagine e dubbia essi da soli non possono giustificare l'utilizzo di un prelevamento biotico a polmone aperto. Campionamenti bioptici transbronchiali in combinazione con il marcatore monoclinale HMB-45 puo essere sufficiente a far diagnosi di linfangioleiomiomatosi. Lo studio radiografico inizialmente potrebbe non rilevare anormalita ma in caso di progressione della malattia si evidenziano immagini reticolari e aumentano le degenerazioni cistiche, mentre i volumi polmonari generalmente si preservano o aumentano. L'associazione di ampi volumi polmonari e diffuse ombre nell'indagine radiologica, puo altresi essere osservata nella istiocitosi a cellule di Langerhans, nella sarcoidosi, nella alveolite allergica estrinseca. Gli aspetti alla tomografia computerizzata ad alta risoluzione, sono caratteristici e sorprendenti, evidenziando cisti con sottili pareti di 2-20 mm o piu di diametro, distribuite su tutto l'ambito polmonare. Altre caratteristiche riguardano occasionali linfonodi ilari e mediastinici con ombre alveolari, possibile segno di emorragia, nel 60% delle scansioni. La principale diagnosi differenziale radiologica e l'istiocitosi a cellule di Langerhans, che come la linfangioleiomiomatosi, solitamente ha un aspetto nodulare negli stadi precoci e risparmia i seni costofrenici. Dal momento che le scansioni TAC nella linfangioleiomiomatosi sono cosi distintive, molti medici non necessariamente ricorrono alla biopsia a polmone aperto nel far diagnosi. Questa strategia e probabilmente ragionevole se l'immagine TAC e patognomonica e la storia clinica e dirimente per linfangioleiomiomatosi; il ritrovamento di angiomiolipomi renali o versamenti chilosi senza dubbio rafforza la diagnosi. In pratica, il tessuto polmonare diventa esaminabile quando i pazienti ricorrono all'intervento chirurgico per pneumotorace o versamento chiloso.

Misure generali: come in altre malattie croniche respiratorie, pazienti con LAM dovrebbero astenersi dal fumo e sottoporsi a vaccinazione influenzale e pneumococcica. Pazienti con ostruzione delle vie respiratorie possono beneficiare dell'utilizzo di broncodilatatori. Terapie di supporto: pazienti con linfangioleiomiomatosi ed ostruzione delle vie aeree traggono beneficio dalla inalazione di beta-agonisti e si stanno ricercando nuove strategie terapeutiche. Il pneumotorace dovrebbe essere trattato convenzionalmente, ma dal momento che piu comunemente e ricorrente, bilaterale, e poco responsivo ai trattamenti conservativi, la chirurgia dovrebbe attuarsi negli stadi precoci. Pneumotoraci ricorrenti richiedono abrasione pleurica, talcaggio o pleurodesi chimica o pleurectomia. La possibilita che pazienti vengano candidati a trapianto di polmone necessita che sia ben chiaro che tutte e tre le procedure aumentano il rischio di sanguinamento nell'atto del trapianto. Molti pazienti ora ricevono il trapianto di un singolo polmone e sebbene la pleurectomia totale dovrebbe essere il piu possibile evitata ne la pleurodesi ne la pleurectomia precludono il singolo o il doppio trapianto di polmone. E consigliabilie consultare una equipe di trapiantisti prima di procedere chirurgicamente sulla pleura, sebbene ogni decisione da prendersi sul trattamento del pneumotorace, debba basarsi primariamente sulle necessita cliniche del paziente in quel determinato momento. Il versamento pleurico chiloso, che risulta dalla ostruzione del dotto toracico, richiede un drenaggio sebbene sia dovuto ad una perdita di proteine e linfociti. L'accumulo linfatico puo essere ridotto da una modificazione della dieta oppure sostituendo una dieta ricca di grassi, con una dieta a trigliceridi a media catena (che sono trasportati nel sangue venoso piuttosto che per via linfatica) o una dieta con un basso contenuto di grassi.Concretamente una dieta con trigliceridi a media catena e spiacevole mentre una dieta a basso contenuto di grassi e piu accettabile (Johnson S. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms. Thorax. 1999;54:254-264).Terapia ormonale: nessun trattamento si e dimostrato essere efficace nella LAM. Dal momento che la malattia fino ad un certo punto e chiaramente ormone-dipendente il trattamento ha puntato sulla deplezione estrogenica e sulla supplementazione progestinica. Tale strategia terapeutica varia da paese a paese, ma il progesterone e il farmaco piu comunemente usato in Europa e negli Stati Uniti. L'Ooforectomia e stata praticata in un piccolo numero di pazienti ma spesso in combinazione con altri trattamenti come il progesterone; la sua efficacia e di difficile valutazione ed ora e stata soppiantata. Gli agonisti del GRH sono in grado di ridurre i livelli di estrogeni similarmente all'ooforectomia ma il loro uso non e supportato dall'evidenza. Vari altri trattamenti che impiegano Interferone Alfa e Somatostatina sono stati applicati, ma la loro efficacia e di difficile valutazione. L'assenza di trattamenti di comprovata efficacia suggerisce che il trattamento ormonale con progesterone (400-800 mg/mese IM o 10-20 mg/die OS) potrebbe essere impiegato come prima linea in pazienti con malattia severa o in rapida progressione. TAMOXIFEN: il razionale dell'utilizzo del tamoxifen come singolo agente nella cura della linfangioleiomiomatosi e debole; esso e un agonista parziale del recettore per l'estrogeno e nuovi antagonisti del recettore per estrogeno o inibitori dell'aromatasi, che bloccano la sintesi di estrogeno, potrebbero risultare piu efficaci (Johnson S. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms. Thorax. 1999;54:254-264).Pneumotorace: il pneumotorace nella LAM e frequente e spesso ricorre dopo un trattamento conservativo (aspirazione o drenaggio toracico). In questi pazienti il trattamento chirurgico dovrebbe esser preso in considerazione precocemente (Johnson S. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms. Thorax. 1999;54:254-264).Versamento Chiloso: il versamento chiloso si verifica in circa il 20% dei pazienti. Alcuni sono modesti e non richiedono alcun trattamento. Altri potrebbero beneficiare della aspirazione ma molti continuano a riformarsi e richiedono un trattamento chirurgico di pleurectomia e occasionalmente di legaggio del dotto toracico. Lavori aneddotici suggeriscono che il progesterone potrebbe essere piu efficace nelle donne con raccolta chilosa. Una riduzione dell'accumulo linfatico mediante una dieta a basso contenuto di grassi o di trigliceridi a media catena potrebbe altresi essere d'aiuto (Kitaichi M, Izumi T. Lymphangioleiomyomatosis. Curr Opin Pulm Med. 1995;10:417-424).Trapianto polmonare: il trapianto polmonare, soprattutto di singoli polmoni, e stato praticato con successo in pazienti negli stadi finali di linfangioleiomiomatosi e in una review di 59 casi di tutto il mondo, la sopravvivenza attuariale a due anni (58%) era simile a quella ottenuta nel trapianto resosi necessario per altri tipi di malattie polmonari.I principali problemi legati alla linfangioleiomiomatosi o alla pregressa pleurectomia furono l'emorragia intraoperatoria, responsabile di un decesso e due reinterventi toracotomici, chilotorace postoperatorio, un pneumotorace spontaneo nel polmone natio.Una linfangioleiomiomatosi ricorrente si e verificata in tre casi nel polmone trapiantato (tutti maschi) e in un caso fu confermata mediante l'utilizzo dell'HMB 45 (tecnica utilizzata per differenziare ripresa di malattia dalla bronchiolite obliterante) (Boehler A, Speich R, Russi EW, Weder W. Lung transplantation for lymphangioleiomyomatosis. 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