I-mastocytosis iwumlomo. Ukuzwa ukuthi wena noma othandekayo wakho unesifo sezempilo ongakaze uzwe ngaso kungesabisa. I-mastocytosis nayo ingabizwa ngokuthi isifo se-mast cell. Ama-mast cell ayingxenye yesigungu segazi elimhlophe lesistimu yakho yomzimba. Zihlobene ne-basophil futhi zihileleka ekutheni ziphiwe futhi zifakwe i- anaphylaxis (ukusabela okunamandla kakhulu).
Ngokuyinhloko, amangqamuzana omzimba adonsela amanye amasosha omzimba emasukeni lapho kudingeka khona ngokukhulula amakhemikhali. Uma inani eliningi kakhulu lamasosha omzimba liqoqa ndawonye ngezicubu, libizwa ngokuthi i-mastocytosis. Lapho i-mast cells iqoqa esikhumbeni kuphela ibizwa ngokuthi i-mastocytosis e-cutaneous. Uma kwenzeka ezinhlanganweni eziningi kuthiwa i-mastocytosis yesistimu. I-mastocytosis yesistimu ibhekwa njenge- neoplasm ye-myeloproliferative .
Izingozi, Izimpawu, nezimpawu ZeMastocytosis
Ngoba i-mastocytosis yizifo ezinjalo ezingajwayelekile ezingaziwa ukuthi bangaki abantu abanalo. Abesilisa nabesifazane kubonakala sengathi bayathinteka ngokufanayo. Ezinganeni, amacala amaningi ahlukaniswa ngenkathi abantu abadala bejwayelekile kakhulu.
Ngenxa yokuthi i-mastocytosis ingavela ezingxenyeni eziningi, izimpawu zokubonisa zingahluka kakhulu. Iningi lezimpawu zenzeka lapho amangqamuzana amancane ekhulula i-histamine namanye amakhemikhali.
Ukuthola izikhumba kuvame kakhulu futhi kufaka phakathi:
- I-Rash: Kunezinhlobo eziningana zokushayeka kwe-mastocytosis. Ngezinye izikhathi kungaba izindawo ezinomsoco / ezimnyama ezibukeka njengezinsalela. Ngezinye izikhathi kubonakala njengamabala abomvu esikhumbeni.
- Ukunambitheka (pruritus): Ukuqhuma kungase kubonakale ikakhulukazi uma kucasuliwe (ukuhlutshwa / ukuhlungululwa) noma uma kuvezwa ushintsho oluzezelayo ekushiseni (njengamanzi ashisayo eshaweni). Lokhu kubizwa ngokuthi isibonakaliso sikaDarier.
- Ukuqhuma (isikhumba esibomvu, esifudumele)
- Blisters
Ezinye izimpawu zibonisa isitho kanye nezindawo ze-mast cell infiltration. Izimpawu zamathumbu zivamile futhi zihlanganisa ubuhlungu besisu, isicanucanu, ukuhlanza nokuhuda. Ukubandakanywa kwemisipha namathambo kungabonisa ubuhlungu noma i-osteopenia / osteoporosis (amandla esithambo esinciphile). Ukusabela kwe-allergen kanye nokuphendula kwe-anaphylactic kungase kwenzeke nomfutho wegazi ophansi (hypotension), i-syncope (ukuphelelwa amandla), ukukhathala (ukukhathala), ukuphefumula, ukugubha noma ukuvuvukala kwamehlo, izindebe, ulimi noma umphimbo. Akuzona zonke iziguli ezinalezi zimpawu.
Yini Engabangela Izimpawu Mastocytosis?
Abantu abane-mastocytosis abanalo izimpawu sonke isikhathi. Ngezinye izikhathi izimpawu zibangelwa ezinye izinto.
- Imithi: imithi yobuhlungu (i-morphine, i-codeine), imishanguzo engeyona i-steroidal anti-inflammatory (i-NSAID, njenge-ibuprofen), i-vancomycin (i-antibiotic) noma i-relaxant muscle
- Ukuzivocavoca
- Ukukhwehlela isikhumba, ikakhulukazi ukuqhuma okuhambisana ne-mastocytosis
- Ukushisa okwedlulele kuyashintsha
- Ukudla okumnandi
- Utshwala
- Izifo
- Ukuhlinzwa
- Ukubamba nokugquma kusuka ezinyosi, ama-wasps, amajackethi aphuzi, amaphondo, izintuthwane, i-jellyfish noma izinyoka
- Ukucindezeleka ngokomzwelo
Ukuxilongwa kweMastocytosis
Ukuxilongwa kwe-mastocytosis kugxile kakhulu kwizinhlobonhlobo zendawo ethintekile (isikhumba, umnumongo, umuthi wamathunjini, njll).
Ngoba izimpawu zingafani kakhulu ukuthola ukuxilongwa kungaba inselele.
I-World Health Organisation (WHO) ishicilele izindlela zokuxilongwa kwe-mastocytosis ebuyekezwa ngezansi.
I-Mastocytosis ehlukanisiwe: Izimpawu kanye ne-biopsy yesikhumba kuyavumelana ne-mastocytosis encane futhi azikho izici ezihambisana ne-mastocytosis yesistimu.
I-mastocytosis enobuciko: Kumele ibe nomgogodla omkhulu kanye nomgomo owodwa omncane noma okungenani umgomo omncane omncane.
- Umgogodla omkhulu: Nge-biopsy (okuvela emnothweni we-bone noma isakhi esithintekile) kumele kube nezindawo eziningi ze-mast cell infiltration (echazwe ngokuthi yi-≥15 amamitha amancane).
- Isimiso esincane:
- Nge-biopsy (mhlawumbe umnkantsha wesithambo noma isitho esithintekayo): Amaphesenti angaphezu kwama-25 wamaseli esisindo esingaphakathi angenawo ama-atypical (angabonakali njengama-mast cells avamile)
- Ukuthola ukuguquguquka kwezakhi zofuzo ezihlobene ne-mastocytosis egazini, emnkantsheni wesinye noma kwezinye izicubu (i- KIT gene).
- Amangqamuzana amancane ku-biopsy aveza amanye amamaki (CD2 kanye / noma i-CD25) ukuthi amasosha omzimba ajwayelekile awenzi.
- I-Tryptase (i-enzyme ephula iphrotheni etholakala kumaseli amancane) okungaphezu kuka-20 ng / mL. La mazinga ngokuvamile aphezulu ngokweqile ku-mast cell leukemia, uhlobo lomdlavuza wesifo se-mast cell.
Ukwelapha
Ngokufanayo nezinye izimo ezifanayo, ukuqina kwesifo kunquma ukuthi ukwelashwa okudingekayo. Izinyathelo zokwelashwa ezijwayelekile zifana ne-mastocytosis engahlanganisi futhi ehlelekile.
- Gwema izinkinga ezibuyekezwe ngenhla.
- Ukulungele i-anaphylaxis (ukusabela okunzima kwe-allergen). Lokhu kubandakanya ukujola kwe-epinephrine etholakalayo ekhaya ukuze uthole ukwelashwa okuphuthumayo uma kudingeka.
- Ukwelashwa okuvimbelayo lapho izidakamizwa ezaziwayo zingenakugwema. Lokhu kufaka phakathi i-prednisone ne-anti-histamines (ranitidine, diphenhydramine) ngaphambi kokubonakala kwe-trigger njengemigomo nokuhlinzwa.
- I-Immunotherapy : I-Immunotherapy, eyaziwa nangokuthi izifo ezithathelwanayo zohlobo lwezinambuzane ezinjengezinyosi, izinambuzane, nezintuthwane zingasetshenziswa.
- Ama-Antihistamines: Ngoba izimpawu zihlobene nokukhululwa kwe-histamine kusuka kumaseli omzimba, akumangazi ukuthi udokotela wakho angase anikeze imithi ebizwa ngokuthi i-antihistamines. Lokhu kuzobandakanya imithi esetshenziselwa ukwelapha isifo sofuba noma ukucheka njenge-cetirizine ne-hydroxyzine. Eminye imithi ihlanganisa i-ranitidine ne-cimetidine, ejwayele ukusetshenziselwa ukwelapha i-reflux ye-gastroesophageal.
- I-Cromolyn: I-Cromolyn imithi yomlomo esetshenziselwa ukuphatha izimpawu zomzimba ezinjengamahlungu esiswini kanye nesifo sohudo esabangelwa yisisindo sokukhululwa kwe-histamine. Ngezinye izikhathi imithi ingase ihlanganiswe ngamafutha okugaya izikhumba.
- Imithi ye-Antileukotriene: Imithi ye-Antileukotriene njenge-montelukast ne-zileuton ingasetshenziswa uma izimpawu zingalawulwa ngama-antihistamine.
- I-aspirini: I-Aspirin ingasetshenziswa ukuphatha ukuqhuma kwesikhumba.
- I-Tyrosine Kinase Inhibitors (TKI): Amakhodi aphethwe yi- KIT ama-protein okuthiwa i-tyrosine kinase. Ukuguqulwa okutholakala ku-mastocytosis kwandisa isisindo se-mast cell in tishu. Imithi ebizwa ngokuthi i-tyrosine kinase inhibitors ingase ibe usizo. I-TKI efundwa kakhulu kakhulu, i-imatinib, ayiphumeli emastocytosis. Enye i-TKI, i-midostaurin, okwamanje ifundiswa mastocytosis.
- I-Chemotherapy: Izinhlobo eziningi ezinonya zamastocytosis zingadinga ukwelashwa nge-chemotherapy efana ne-hydroxyurea ne-cladribine.
I-mastocytosis ingaba yinkinga enzima ukuyiqonda. Ungadinga odokotela abaningi okhethekile ukuze uthole ukuxilongwa futhi ukwandise ukwelashwa kwakho.
> Imithombo:
> I-Castells MC ne-Akin C. Mastocytosis (i-cutaneous and systemic): i-Epidemiology, i-pathogenesis, nokubonakaliswa kwemitholampilo, i-Mastocytosis (i-cutaneous and systemic): Ukuhlolwa nokuxilongwa kubantu abadala, i-Mastocytosis ehlukeneyo: Ukwelashwa nokubikezela, kanye neMastocytosis ejwayelekile. Ku: Phezulu, I-post TW (Ed), UpToDate, Waltham, MA.