Felty kaThixo Syndrome: Iimpawu, Diagnosis

Phantsi Felty engqondweni libhekisa kwingxaki ye isifo samathambo, okwenzeka% 1 kuphela zonke izigulane sifo. Fever, yoma kwezihlunu, nombala kunye susceptibility kwizigulo ezosulelayo uhlobo - zonke ezi zinto ngokuqhelekileyo ahamba Felty engqondweni. Pathology Uxilongo isekelwe ngembali isifo samathambo, obaluleke bobabo parameters elebhu. Unyango ibandakanya ukuthathwa corticosteroids, splenectomy. Kangangexesha elide ngezifo esibonakala ngemvelo ukungaqiniseki kunye nomfanekiso clinical. Ngowe-1929, AP Felty wachaza sifo. Okoko sathi esisifo umahluko ibe nosology elizimeleyo.

Felty syndrome: ntoni na?

Isifo kuchazwa bubukho ezimbi ezintathu ngaxeshanye: i isifo samathambo, udakada eyandisiweyo kunye leucopenia (iiseli igazi eliphantsi amhlophe). Esi sifo idla olufunyanwa ngabantu abadala ngaphezu kweminyaka engama-40, ngokuqhelekileyo sex enobulungisa.

Iingcali lubonisa ukuba sisiseko sifo yinkqubo sokuhlasela. Apho kunye inxaxheba ngqo izicwili lymphoid kwaye ukuyilwa koko izakhiwo omzimba kwakunye omzimba athile. Pathology wabonisa njengoko neutropenia kunye nokwehla mandatory ukumelana nezifo ezininzi, yaye xa zithe zenzeka - esiqatha. Ngoko ke namhlanje splenectomy (ukususwa udakada) ibonakaliswe kuphela indlela ephumelelayo unyango.

Felty engqondweni luphawulwa ukwahluka okukhulu, ngokufuthi Nedlac. Isifo ukuze eyandulela iminyaka eliqela (5 ukuya malunga 10) izifo-kuthiwa zihlaselana samathambo kunye nokubandakanyeka ngaxeshanye kwamalungu kwinkqubo interphalangeal lwezidumbu, ezinamazinga angafaniyo ubungnqongqo.

Kwelinye icala, uninzi lwezigulane kunye nale unesifo zimiselwa ekubonakalisweni ngumntu kwizicwili. Oku kube nangecesina, ukuyilwa maqhuqhuva ongama, myocarditis, ukwehla kobunzima, poliserozita.

Izizathu

I-umyalezo we sifo akaziwa. Ayizizo zonke izigulane unesifo "isifo samathambo 'liphuhlisa le ngezifo. Xa umongo ithambo lomntu esempilweni ziqhubela eziveliswa iiseli ezimhlophe zegazi. Izigulane nale sifo, nakuba kusasazwe iphantsi kakhulu, leukocytes nabo bakha. Ukuze yande ngezixa kakhulu kwi udakada.

Ziziphi iimpawu kunye syndrome Felty xa abadala?

• syndrome Articular (samathambo namalungu amancinane).
• Ukwandisa ubungakanani esibindini.
• lymphadenopathy ngokubanzi (olufunyanwa-50% amatyala).
• Ukwanda okuqhubekayo ubukhulu udakada (njengoko imiselwe Ngokucofa).
• Polyneuropathy.
• Nombala kakhulu eluswini, izilonda shins.
• Kuyenye yeendlela abanesigulo miphungeni.
• maqhuqhuva omzimba.

Zonke iimpawu ngaphezulu zenza kube lula ukufumanisa Felty engqondweni. Iimpawu zonke izigulane ziboniswa ngokwahlukeneyo. Ngokomzekelo, maxa wambi wawandisa udakada elifana neli kuphela amanqanaba kamva zophuhliso sifo.

uphethwe

Isiseko kokuqinisekisa lokugqibela uxilongo lwezonyango yomthonyama ethi idibanise ngaxeshanye splenomegaly kunye isifo samathambo. segazi luphawulwa kwegazi, leukopenia, neutropenia. Le immunoassay ngokuqhelekileyo ibhaqa RF titers eziphakamileyo, siz, ubukho omzimba antinuclear. Ngokutsho iziphumo myelogram myeloid lomongo hyperplasia sinesifo utshintsho ngayo izinto immature cellular. iindlela negalelo uphando (MRI, CT, ultrasound amalungu) ezizezona ukuba ukunika ingcaciso encinane. Felty kaThixo syndrome kubaluleke kakhulu ukuba umahluko kunye kwesibindi kunye sarcoidosis.

imigaqo esisiseko zonyango

Therapy, kule meko ibandakanya indlela ebanzi. isifo samathambo iphathwa njenge standard. Therapy ephindaphindekayo izifo, kwaye samathambo kuquka iziyobisi ukunceda rheumatism ( "Azathioprine", "MTX").

Izigulane izifo kakhulu kucetyiswa ukuba wenze izitofu weveki yonke into, ekhuthaza granulocyte koloni. It kwandisa inani iiseli ezimhlophe zegazi.

iziyobisi Basic kungacutha symptomatology ezihamba samathambo, Felty syndrome ngqo. zibandakanya Ezi ziquka: "Methotrexate", "Penicillamine".

Glucocorticoids zisebenza kuphela iidosi eziphezulu. Kungenjalo, zanda okuba likakade. Kwelinye icala, ukusetyenziswa elide glucocorticoids kungakhokelela kuphuhliso zizifo intercurrent.

Iikhrayitheriya ukusebenza unyango:

• Ukwandisa inani granulocytes kutsho ku-2000 / mm.
• Ukunciphisa izehlo ingxaki ezosulelayo zendalo.
• Ukuncitshiswa lokuhlaselwa umkhuhlane.
• Ukuncitshiswa izilonda kwi wabonakala esikhumbeni.

Xa uqhaqho kuyacetyiswa?

njani enye woyise Felty engqondweni? Treatment ngu utyando kuyacetyiswa ukuba unyango ngamachiza ukuba ninganqeni okanye ngenxa yaso nasiphi na isizathu, kuya contraindicated. Kulo mzekelo, nto idla ngokuba umbuzo splenectomy. Kubalulekile ukukhumbula ukuba-25% amatyala neutropenia Sibuye emva kwale nkqubo.

Phantsi splenectomy kuthetha utyando, apho ugqirha ngokupheleleyo okanye ngokuyinxenye lususa udakada. Namhlanje, kukho amathuba amabini nkqubo onjalo: splenectomy laparoscopy nevulelekileyo.

Laparoscopy lwenziwa kusetyenziswa Ukubulawa jikelele. Kulo mzekelo, umsebenzi kubandakanya ukusetyenziswa kwesixhobo obhityileyo kunye ikhamera ekupheleni, leyo ibizwa ngokuba laparoscope. Ugqirha yenza ukusikwa ezincinane ezininzi esisu apho wazisa izixhobo zazo ezilandelayo. Ithuba alulame, njengoko umthetho, akafuni kufuna ixesha elininzi.

splenectomy Vula lubandakanya liqatywe enkulu ngezantsi iimbambo, apho ugqirha lususa udakada. Emva ukususwa umzimba ukukwazi komzimba ukuze coca ulwelo ngaphandle kwiintsholongwane ukusuka igazi ngonyuka lifinyele.

iingxaki kunokwenzeka

Iingxaki izifo ezinqabe kakhulu kwaye sibonise ngokwalo ngokohlobo a udakada indlebe, uphuhliso ekuthiwa lwegazi portal, emathunjini ukopha kakhulu ziyenzeka izifo.

isiphelo

Felty kaThixo syndrome - isifo inqabile kakhulu, kodwa kukho ingxaki leqela iphela ezimbi leyo namhlanje uxilongwe enqabile. Oku akuthethi ukuba sinako lokukhathalela amayeza kwaye hayi ukuba afune uncedo abaqeqeshiweyo kwagqirha efanelekileyo. Iimpawu Primary kufuneka wazise wonke ube isizathu sokuba ufuna icebiso yincutshe. Kungenjalo, kuba kwande amathuba imandundu karhulumente jikelele kunye nophuhliso ngokwaneleyo yingozi iingxaki zempilo, ezibandakanya komsipha le udakada ngokwayo. Ukhetho kuphela unyango olusebenzayo ngoku yaziwa njenge splenectomy.

Siyathemba ukuba yonke ingcaciso enikwe kweli nqaku kwi sihloko kuya kuba luncedo ngokwenene kuwe. Hlala uphilile!