Emofilia: che cos’è e quali sono le terapie
Table of Contents
Che cos’è l’emofilia
L’emofilia è un’ affezione ereditaria familiare, a trasmissione recessiva dalla donna ai soli figli maschi, caratterizzata da un ritardo della coagulazione e da emorragie prolungate, spontanee o che compaiono al minimo trauma.
Eziologia
La trasmissione dell’emofilia è collegata al cromosoma sessuale X; le donne sono portatrici sane mentre si manifesta quasi esclusivamente negli uomini; in effetti, l’emofilia femminile si manifesta solo nei casi in cui il padre sia emofilico e la madre portatrice. In un terzo di casi di emofilia non si hanno antecedenti familiari.
Emofilia A o classica
L’emofilia A si manifesta nell’ 85% dei casi: è dovuta ad un deficit di fattore VIII, chiamato anche fattore anti-emofilico A” che è essenziale per la formazione della trombroplastina.
Emofilia B o malattia di Christmas: si manifesta nel 15% dei casi ed è causata da un deficit di fattore IX. È importante distinguerla dall’ emofilia A poiché la terapia è diversa; la stabilità del fattore IX permette il trattamento di questa forma di emofilia con sangue o plasma conservato.
Il deficit di fattore XI o PTA è a volte chiamato impropriamente emofilia C”. Questo deficit raro è infatti un’ affezione distinta dall’ emofilia; è trasmesso come carattere dominante, non legato al sesso e si manifesta nei due sessi con una sindrome emorragica relativamente benigna.
Quadri emorragici generali nelle sindromi emofiliache
- Il quadro clinico dei pazienti con sindromi emofiliche è caratterizzato dalla spiccata tendenza alle emorragie anche spontanee a livello di muscoli (ematomi) , articolazioni (emartri) , cutanee (ecchimosi), mucose (gengivorragia, epistassi, gastroenterorragia) di organi vitali (emorragie intracraniche). L’intensità e la frequenza degli episodi emorragici sono proporzionali all’entità del difetto (tanto maggiore quanto più grave la carenza) . ù
- Gli ematomi muscolari sono tra i più gravi e invalidanti i sintomi delle emofilie A e B. Le sedi più frequenti sono rappresentate da polpaccio, coscia gluteo e avambraccio; gli ematomi dell’avambraccio vanno osservati e trattati attentamente, in quanto rischiano di comprimere i nervi mediano e cubitale, con gravi disturbi sensitivi e motori.
- L’ematoma della psoas si manifesta con dolore all’inguine accompagnato da posizione antalgica della coscia in flessione e rotazione.
- Pericolosi sono gli ematomi dei muscoli del pavimento della bocca e del collo, perché possono portare a compressioni del tratto respiratorio.
- Tra tutte le manifestazioni emorragiche delle emofilia A e B, le più caratteristiche sono gli emartri; l’articolazione più colpita è quella del ginocchio, seguita da quelle del gomito, piede e anca, mentre le altre sono più rare.
- Gli emartri richiedono sempre un pronto intervento, poiché, se non trattati, portano all’artropatia emofilica, con gravi alterazioni articolari e dei capi ossei che possono condurre alla deformità.
- A carico della mucosa nasale possono presentarsi epistassi gravi.
- A carico della cavità orale, piccole morsicature o ferite della lingua, del labbro, della guancia sono spesso causa di sanguinamento grave e prolungato; la caduta dei denti e le estrazioni dentarie danno luogo a emorragie prolungate. L’ematuria è molto frequente nelle sindromi emofiliche e gravi ed è accompagnata da dolore colico, dovuto alla formazione di coaguli nella pelvi e al loro passaggio nel uretere.
- Le emorragie gastrointestinali sono più rare e sono dovute a un diffuso sanguinamento della mucosa gastrica.
- Le emorragie intracraniche sono rare e possono essere conseguenti a traumi anche lievi oppure possono insorgere senza apparenti cause locali o sistemiche.
- Le emorragie in corso di interventi chirurgici possono comparire quando un paziente con sindrome emofilica non è stato diagnosticato oppure quando non viene sottoposto a trattamento adeguato.
Diagnosi dell’emofilia
- Tendenza emorragica dopo traumi anche minimi in un paziente di sesso maschile; anamnesi familiare.
- Ematoma o emartro recidivanti.
- Prolungamento del tempo di coagulazione e \o del tempo di cefalina – caolino.
Prognosi
Le emorragie spontanee sono veramente pericolose; al contrario i traumi possono provocare emorragie anche fatali.
Emofilia: quali sono le cure
Vi sono molteplici opzioni terapeutiche per il trattamento dell’emofilia. La terapia cardine è stata la terapia sostitutiva: la somministrazione di fattore di coagulazione VIII ai pazienti con emofilia A e di fattore di coagulazione IX a pazienti con emofilia B. Lo scopo di tali terapie è quello di prevenire e curare le emorragie, che possono venire somministrati esclusivamente per via endovenosa. L’uso di emoderivati contenenti fattore VIII ha cambiato la prognosi e la qualità di vita di questi pazienti.
Terapia emostatica sistemica: negli emofilici moderati e lievi (ma non nei gravi) è possibile ottenere un moderato aumento di VII: C circolante mediante infusione ev o sc di dermopressina, analogo sintetico ell’ ADH, che stimola la secrezione di fattore VIII.
Terapia emostatica locale: può essere utile da sola o in associazione agli emoderivati e agli emostatici sistemici per favorire localmente l’emostasi e la cicatrizzazione di ferite superficiali. Si avvale di colla di fibrina o di trombina.
Terapie collaterali: importantissima è quella antalgica. A questo scopo è da prescrivere l’uso di aspirina e da limitare al minimo possibile quello di FANS e, se non sotto copertura con emoderivati, in quanto questi farmaci esplicano tutti attività antiprostaglandine e quindi antiaggregante. E’consentito l’uso di paracetamolo a (dosi 500-1000 mg/die) di pentazocina, o di buprenorfina morfina se è il caso e di altri farmaci non FANS e con effetto antalgico come il tramadolo cloridrato.
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