I-Mastocytosis inomlomo. Ukuva ukuba wena okanye wakho umthandayo unempilo yonyango ongakaze uyive ngayo inokuyikrakra. I-Mastocytosis ingabizwa nangokuthi isifo se-mast cell. Iiseli ezincinci zihlobo lentsholongwane yamhlophe yegazi le-immune system. Zidibene ne-basophil kwaye zibandakanyeka kwi-allergies kunye ne- anaphylaxis (i-reaction effect).
Okubalulekileyo, iisaliti zesitrasi zikhangele ezinye iiseli ze-immune kwiindawo zeethambo xa zifunwa ngokukhulula iikhemikhali. Xa inani eligqithisileyo leeseli zesisitye liqokelela ndawonye kwiishubhu, libizwa ngokuba yi-mastocytosis. Xa iifestile ezigciniweyo ziqokelela kwesikhumba kuphela zibizwa ngokuba yi-mastocytosis engxube. Xa kwenzeka kumalungu amaninzi kuthiwa yi-systemic mastocytosis. I-mastocytosis ebonakalayo ithathwa njenge- neoplasm ye-myeloproliferative .
Izingozi, iimpawu kunye nezibonakaliso zeMastocytosis
Ngenxa yokuba i-mastocytosis yinto enqabileyo yintlupheko ayiyazi ukuba bangaphi abantu. Amadoda kunye namabhinqa kubonakala ngathi achaphazelekayo ngokufanayo. Kubantwana, amaninzi amaninzi ahlukeneyo xa abantu abadala bexakeka kwi-mastocytosis.
Ngenxa yokuba i-mastocytosis inokuvela kwizitho ezininzi, iimpawu zokubonisa ziyahluka kakhulu. Uninzi lweempawu zivela xa iisitrasi ezigciniweyo zikhulula i-histamine nezinye iikhemikhali.
Iziphumo zesikhumba zizona ziqhelekileyo kwaye ziquka:
- I-Rash: Kukho iintlobo ezininzi zokugqithisa kwi-mastocytosis. Ngamanye amaxesha inokuba yimimandla emanci / emdaka ebonakala ngathi i-freckles. Ezinye izihlandlo zibukeka njengamabala obomvu kwesikhumba.
- Ukutshiza (pruritus): Ukugqithisa kungabetha ngokukodwa xa ecatshulwa (ukuhluthwa / ukuhluthwa) okanye xa ubonakaliswe utshintsho olusenyongweni (njengamanzi ashushu kwisitya). Oku kubizwa ngokuba nguDarier.
- Ukuhluthwa (obomvu, isikhumba esifudumele)
- Blisters
Ezinye iimpawu zibonakalisa ilungu kunye nemimandla yesigxina sokungena kweseli. Izimpawu zesisu ziqhelekileyo kwaye zibandakanya iintlungu zesisu, isicasuliso, ukuhlanza kunye nehudo. Ukubandakanywa kweemisipha namathambo kungabonisa intlungu okanye i-osteopenia / i-osteoporosis (iyancipha amandla amathambo). Ukuphendulwa kweengxaki kunye nokuphendula kwe-anaphylactic kunokufumaneka nangomxhesho ophantsi wegazi (hypotension), syncope (ukuphelelwa amandla), ukukhathala (ukukhathala), ukuphefumula okufutshane, ukuvutha okanye ukuvuvukala kwamehlo, imilomo, ulwimi okanye umqala. Ababona zonke izigulane zinaloo mpawu.
Yintoni Enokubangela Iimpawu kuMastocytosis?
Abantu abane-mastocytosis abanalo iimpawu zonke ixesha. Ngamanye amaxesha iimpawu zibangelwa ezinye izinto.
- Amachiza: amayeza enhlungu (i-morphine, i-codeine), imithi engeyiyo-steroidal anti-inflammatory (i-NSAID, njenge-ibuprofen), i-vancomycin (i-antibiotic) okanye i-muscle relaxants
- Ukuzivocavoca
- Ukukhupha isikhumba, ngokukodwa ukukhawuleza okubandakanya i-mastocytosis
- Ukushisa okugqithiseleyo
- Ukutya okunesonka
- Utywala
- Izifo
- Ukuhlinzwa
- Ukubetha kunye nezibonda ezivela kwizinyosi, amaqabunga, iibhattshi eziphuzi, iipompet, iintuthwane, i-jellyfish okanye inyoka
- Ukuxinezeleka ngokomzwelo
Ukuxilongwa kweMastocytosis
Ukuxilongwa kwe-mastocytosis ixhomekeke kakhulu kwii-biopsies zommandla ochaphazelekayo (isikhumba, umongo, isondlo sesisu, njl).
Ngenxa yokuba iimpawu ziyahluka kakhulu ukufumana ukuxilongwa kunokuba nzima.
I-World Health Organization (WHO) iye yapapasha iikhrayitheriya zokuxilongwa kwe-mastocytosis ehlaziywa ngezantsi.
I-Mastocytosis encinane: Iimpawu kunye ne-biopsy yesikhumba zihambelane ne-mastocytosis ene-cutaneous kwaye akukho zixhobo ezihambelanayo ne-systemic mastocytosis.
I-Mastocytosis yoMgaqo-nkqubo: Kumelwe ube nekhrayithi enkulu kunye nomlinganiselo omnye omncinci okanye ubuncinane ubuncinane emithathu.
- Umlinganiselo omkhulu: Kwi-biopsy (mhlawumbi ukusuka kumnxeba wethambo okanye ilungu elithintekayo) kufuneka kube neendawo ezininzi zokungena kwesitrasi (esichazwe njenge -15).
- Icandelo elincinci:
- Kwi-biopsy (nokuba ngumnkantso wethambo okanye iqumrhu elichaphazelekayo): Ngaphezu kweepesenti ezingama-25 zamaseli ezitshixo ekungeneni kwe-intl
- Ukufumanisa ukuguqulwa kwemfuzo ehambelana ne-mastocytosis egazini, umongo weethambo okanye ezinye izicubu (i- KIT gene).
- Iiseli ezincinci kwi-biopsy zibonisa ezinye iimpawu zamanqaku angaphezulu (iCD2 kunye / okanye iCD25) eziqhelekileyo zeeseli zamathambo ezingenzi.
- I-Tryptase (i-enzyme ephula iprotheni efunyenwe kwiiseli zesigxina) engaphezulu kwama-20 ng / mL. Ezi nqanaba zihlala ziphakamileyo kakhulu kwi-cell stroke leukemia, uhlobo lwesifo somhlaza wesisitragi.
Unyango
Kuyafana nezinye iimeko ezifanayo, ubunzima bezifo zibeka unyango olufunekayo. Amanyathelo athatywayo ngokuqhelekileyo afana ne-mastocytosis ehlukeneyo kunye neenkqubo.
- Gwema iziphumo ezihlaziywe ngasentla.
- Ukulungiselela i-anaphylaxis (i-reaction effect). Oku kubandakanya ukufaka iinjengesifo ze-epinephrine ekhaya ukuze unyango oluphuthumayo xa lufuneka.
- Unyango olukhuselayo xa iziphumo eziyaziwayo zingenakunqandwa. Oku kuya kubandakanya i-prednisone kunye ne-anti-histamines (ranitidine, diphenhydramine) phambi kokutsalwa kwezinto ezinjengezitofu kunye nokuhlinzwa.
- I-Immunotherapy : Immunotherapy, eyaziwa ngokuba yizilwanyana ezinqabileyo ezinjengamazinyo, ama-aps, kunye ne-ants zingasetyenziswa.
- I-Antihistamines: Ngenxa yokuba iimpawu zihambelana nokukhululwa kwe-histamine kwii-cell cells, akumangalisi ukuba ugqirha wakho unokunika imithi ebizwa ngokuba yi-antihistamines. Ezi ziza kubandakanya imishanguzo esetyenziselwa ukunyanga i-allergies okanye ukubetha njenge-cetirizine kunye ne-hydroxyzine. Amanye amayeza afaka i-ranitidine kunye ne-cimetidine, esetyenziswa ngokuqhelekileyo ukuphathwa kwe-reflux ye-gastroesophageal.
- I-Cromolyn: I-Cromolyn yonyango lomlomo elisetyenziselwa ukunyanga iimpawu zesisu ezinjengeentlungu zesisu kunye nesifo sohudo esibangelwa yi-mast cell release of histamine. Maxa wambi amayeza angenakunqandwa kwioli yezilonda zesikhumba.
- Iyeza ze-Antileukotriene: Iyeza ze-Antileukotriene ezifana ne-montelukast kunye ne-zileuton zingasetyenziswa ukuba iimpawu azilawulwa ngama-antihistamines.
- I-Aspirin: I-Aspirin ingasetyenziselwa ukuphatha ukucoca ulusu.
- I-Tyrosine Kinase Inhibitors (TKI): Iikhowudi ze- KIT zithintela iiprotheyini ezibizwa ngokuba yi-tyrosine kinase. Inguqulelo efumaneka kwi-mastocytosis yenza ukwanda kweeseli zesistim kwiishukela. Imithi ebizwa ngokuba yi-tyrosine kinase inhibitors inokuba luncedo. I-TKI efundwa kakuhle kakhulu, i-imatinib, ayisebenzisekanga kwi-mastocytosis. Enye i-TKI, midostaurin, ngoku iqhutyelwa i-mastocytosis.
- I-Chemotherapy: Iindlela ezininzi ezinobundlobongela be-mastocytosis zingadinga unyango ngekhemotherapy njenge-hydroxyurea kunye ne-cladribine.
I-Mastocytosis ingaba ngumngeni onzima ukuyiqonda. Unokudinga amayeza athile okhethekileyo ukuze ufikelele ukuxilongwa kwaye uphucule unyango lwakho.
> Imithombo:
> Castell MC noAkin C. Mastocytosis (cutaneous and systemic): I-epidemiology, i-pathogenesis, kunye nokubonakaliswa kweklinikhi, iMastocytosis (i-cutaneous and systemic): Ukuvavanya kunye nokuxilongwa kubantu abadala, iMastocytosis ye-Cutaneous: Ukonyango kunye nokugxekwa, kunye ne-Mastocytosis ye-Systemic. Ku: U-UpToDate, i-Post TW (Ed), UpToDate, i-Waltham, MA.