Mastocytosis ndi wolankhula. Kumva kuti inu kapena wokondedwa wanu muli ndi matenda omwe simunamvepo akhoza kuwopsya. Mastocytosis angathenso kutchedwa mast cell disease. Masi maselo ndi mtundu wa maselo oyera a chitetezo cha m'thupi. Zimagwirizana ndi basophil ndipo zimakhudzidwa ndi chifuwa chachikulu komanso anaphylaxis (zomwe zimawopsa kwambiri).
Makamaka maselo amatenga maselo ena ammadzimadzi kumadera a minofu pamene pakufunika kumasula mankhwala. Pamene minofu yambiri ya masi imasonkhana pamodzi mu matenda, imatchedwa mastocytosis. Pamene mbola imasonkhanitsa khungu kokha amatchedwa cutaneous mastocytosis. Mukapezeka mu ziwalo zambiri zimatchedwa systemic mastocytosis. Mastocytosis yowonongeka imatengedwa ngati myeloproliferative neoplasm .
Zoopsa, Zizindikiro, ndi Zizindikiro za Mastocytosis
Chifukwa mastocytosis ndizovuta zosayembekezereka sizidziwika kuti ndi anthu angati omwe ali nazo. Amuna ndi akazi amawoneka ngati akukhudzidwa chimodzimodzi. Kwa ana, nthawi zambiri zimadulidwa pamene akuluakulu a mastocytosis ali ofala kwambiri.
Chifukwa mastocytosis ikhoza kuchitika mu ziwalo zambiri, zizindikiro zowonetsa zimasiyana kwambiri. Zizindikiro zambiri zimachitika pamene maselo akuluakulu amatulutsa histamine ndi mankhwala ena.
Zotsatira za khungu ndizofala kwambiri ndipo zimaphatikizapo:
- Mtundu: Pali mitundu yambiri ya chiphuphu mu mastocytosis. Nthawi zina zikhoza kukhala malo amdima / bulauni omwe amawoneka ngati mabala. Nthawi zina amawoneka ngati malo ofiira pa khungu.
- Kuwongolera (pruritus): Kuthamanga kungakhale kovuta makamaka pamene kunakwiyidwa (kutsekedwa / kunyowa) kapena pakusintha kwadzidzidzi kutentha (monga madzi otentha mumsamba). Izi zimatchedwa chizindikiro cha Darier.
- Flushing (khungu lofiira, lotentha)
- Ziphuphu
Zizindikiro zina zimagwiritsa ntchito limba ndi malo olowa m'madzi. Zizindikiro za m'mimba ndizofala ndipo zimapweteka m'mimba, kunyoza, kusanza, ndi kutsekula m'mimba. Kuphatikizidwa kwa minofu ndi mafupa kungabweretse ndi ululu kapena osteopenia / osteoporosis (mphamvu ya fupa ya fupa). Zomwe zimayambitsa matendawa ndi zotsatira za anaphylactic zikhoza kuchitika ndi kutsika kwa magazi (hypotension), syncope (kutaya mtima), kutopa (kutopa), kupuma pang'ono, kupuma kapena maso, milomo, lilime kapena mmero. Osati odwala onse ali ndi zizindikiro izi.
Kodi N'chiyani Chingachititse Zizindikiro M'mastocytosis?
Anthu omwe ali ndi mastocytosis alibe zizindikiro nthawi zonse. Nthawi zina zizindikiro zimayambitsidwa ndi zinthu zina.
- Mankhwala: Mankhwala opweteka (morphine, codeine), mankhwala osagwiritsidwa ntchito ndi anti-inflammatory (NSAIDs, monga ibuprofen), vancomycin (antibiotic) kapena minofu yopumula
- Kuchita masewera olimbitsa thupi
- Khungu lopukuta, makamaka kutupa komwe kumagwirizanitsidwa ndi mastocytosis
- Kutentha kwakukulu kumasintha
- Zakudya zokometsera
- Mowa
- Matenda
- Opaleshoni
- Kukwapula ndi kupweteka kwa njuchi, ululu, majezi achikasu, nyanga, nyongolotsi, nsomba zam'madzi kapena njoka
- Kusokonezeka maganizo
Kusanthula Mastocytosis
Kuzindikira kwa mastocytosis kumayang'ana makamaka pa malo omwe amachitika (khungu, fupa, mawere m'mimba, ndi zina).
Chifukwa zizindikirozo zimasiyana mosiyanasiyana kupeza matendawa zingakhale zovuta.
Bungwe la World Health Organization (WHO) lafalitsa zoyenera kuti adziwe matenda a mastocytosis omwe akuwunika pansipa.
Mastocytosis yochepa: Zizindikiro ndi chikopa cha khungu zimagwirizana ndi mastocytosis osakaniza ndipo palibe zofanana ndi dongosolo la mastocytosis.
Mastocytosis oyenera: Ayenera kukhala ndi chiyeso chachikulu ndi chilinganizo chimodzi chaching'ono kapena osachepera atatu.
- Chinthu chachikulu: Pa chinyama (kaya ndi mafupa kapena chamoyo chokhudzidwa) chiyenera kukhala ndi mbali zambiri za maselo ochepa kwambiri (omwe amadziwika kuti ≥15 mamita maselo).
- Chinthu chochepa:
- Pamalo opangira mafupa (kaya mafupa a mafupa kapena gulu lokhudzidwa nalo): Zoposa 25 peresenti ya maselo omwe amatha kulowa mkati amakhala otsika (samawoneka ngati maselo achibadwa)
- Kuzindikira kusintha kwa chibadwa chokhudzana ndi mastocytosis mu magazi, fupa la mafupa kapena minofu ina ( KIT gene).
- Maselo akuluakulu mu biopsy amasonyeza zizindikiro zina (CD2 ndi / kapena CD25) zomwe maselo osadziwika amatha.
- Tryptase (mankhwala a enzyme omwe amathyola mapuloteni omwe amapezeka m'maselo akuluakulu) omwe ndi oposa 20 ng / mL. Maseŵerawa nthawi zambiri amakhala okwera kwambiri m'kati mwa maselo a khansa ya m'magazi, omwe amakhala ndi khansa yambiri ya khansa.
Kuchiza
Mofanana ndi zikhalidwe zina zofanana, matenda aakulu amachititsa chithandizo chofunikira. Njira zochiritsira zowonongeka ndi zofanana ndi zowonongeka ndi zowononga mastocytosis.
- Pewani zokopa zomwe zafotokozedwa pamwambapa.
- Kukhala wokonzekera anaphylaxis (zoopsa zowonongeka). Izi zikuphatikizapo kukhala ndi jekeseni wa epinephrine yomwe ilipo pakhomo ngati mukufunikira thandizo.
- Katetezedwe chithandizo pamene zidziwitso zodziwika zimapeweka. Izi ziphatikizapo prednisone ndi anti-histamines (ranitidine, diphenhydramine) musanayambe kuwoneka ngati katemera ndi opaleshoni.
- Immunotherapy : Immunotherapy, yomwe imadziwikanso ngati zizindikiro zazing'ono zomwe zimatulutsa njuchi ngati njuchi, mavu, ndi nyerere zingagwiritsidwe ntchito.
- Antihistamines: Chifukwa chakuti zizindikiro zimagwirizana ndi kutuluka kwa histamine ku maselo akuluakulu, n'zosadabwitsa kuti dokotala wanu angapereke mankhwala otchedwa antihistamines. Izi ziphatikizapo mankhwala ochizira matendawa kapena kuyabwa monga cetirizine ndi hydroxyzine. Mankhwala ena amaphatikizapo ranitidine ndi cimetidine, zomwe zimagwiritsidwa ntchito kwambiri pochiza matenda a gastroesophageal reflux.
- Cromolyn: Cromolyn ndi mankhwala ochiritsira pakamwa omwe amagwiritsidwa ntchito pochizira m'mimba zizindikiro monga kupweteka kwa m'mimba ndi kutsekula m'mimba kumene kunayambitsa khungu la histamine. Nthawi zina mankhwala akhoza kuwonjezeka kukhala mafuta onunkhira a khungu.
- Mankhwala a Antileukotriene: Mankhwala a Antileukotriene monga montelukast ndi zileutoni angagwiritsidwe ntchito ngati zizindikiro sizikulamulidwa ndi antihistamines.
- Aspirin: Aspirin ikhoza kugwiritsidwa ntchito poyeretsa pakhungu.
- Tyrosine Kinase Inhibitors (TKI): Mavitamini a KIT adakhudzidwa ndi mapuloteni otchedwa tyrosine kinase. Kusinthika kwapezeka mumastocytosis kumapangitsa misa kusungunuka mu matenda. Mankhwala otchedwa tyrosine kinase inhibitors angakhale othandiza. TKI yophunzira bwino kwambiri, imatinib, sichitha mastocytosis. TKI ina, midostaurin, ikuphunziridwa mu mastocytosis.
- Chemotherapy: Mankhwala oopsa kwambiri a mastocytosis angafune mankhwala ndi chemotherapy monga hydroxyurea ndi cladribine.
Mastocytosis akhoza kukhala vuto lovuta kumvetsa. Mungafunike madokotala ambiri apadera kuti muwathandize kupeza chithandizochi ndikukwaniritsa chithandizo chanu.
> Zotsatira:
> Castells MC ndi Akin C. Mastocytosis (cutaneous and systemic): Epidemiology, pathogenesis, ndi mawonetseredwe am'chipatala, Mastocytosis (cutaneous and systemic): Kufufuza ndi kuganizira kwa akuluakulu, Mastocytosis ochepetsedwa: Kuchiza ndi kufotokoza, ndi Masokacytosis Wachilengedwe: Kuchiza ndi kufotokoza. Mu: UpToDate, Post TW (Ed), UpToDate, Waltham, MA.