Mastocytosis is mouthful. Kunzwa kuti iwe kana mudiwa wako ane chiremba iwe usati wambonzwa nezvazvo unogona kutya. Mastocytosis inogonawo kunzi mast cell disease. Mast masero ndiwo rudzi rwechichena chemuviri weropa remuviri wako. Izvo zvakabatana nebasophil uye zvinobatanidzwa mumakemikari uye anaphylaxis (yakanyanya kukanganiswa kwehutano).
Chaizvoizvo, mast masero anotema mamwe masero omusununguko kumativi emaviri kana zvichidiwa nekusunungura makemikari. Apo nhamba yakawandisa yemasero emasero anoungana pamwechete mumatumbu, inonzi mastocytosis. Apo mast masero anounganidza muganda chete anonzi cutaneous mastocytosis. Apo zvinoitika munhengo dzakawanda inonzi systemic mastocytosis. Systemic mastocytosis inoonekwa semetroproliferative neoplasm .
Njodzi, Zviratidzo, uye Zviratidzo zveMastocytosis
Nemhaka yokuti mastocytosis inetso dzakadaro dzisingazivikanwi hazvizivikanwi kuti vangani vanhu vanezvo. Vanhurume nevakadzi vanoita sevanokanganiswa. Mune vana, maitiro akawanda anowanikwa muhutano apo muvanhu vakuru systemic mastocytosis yakawanda.
Nokuti mastocytosis inogona kuitika munhengo dzakawanda, zviratidzo zvinoratidza zvinogona kusiyana zvikuru. Zvizhinji zvezviratidzo zvinowanikwa apo mamasi masero anosunungura histamine nemamwe makemikari.
Zviwanikwa zveganda ndizvo zvakanyanya uye zvinosanganisira:
- Rash: Kune mhando dzakasiyana dzekumhanya mumastocytosis. Dzimwe nguva zvinogona kuva matani / maruva nzvimbo dzinotaridzika sejasi. Dzimwe nguva inoratidzika semaronda matsvuku paganda.
- Itching (pruritus): Rush rinogona kunyanya kunyanya kana ratsamwiswa (kubviswa / rakatsvaira) kana kana rikazoshandurwa kamwe kamwe mumamiriro ekushisa (semvura inopisa mumvura). Izvi zvinonzi Darier chiratidzo.
- Kushambadza (tsvuku, ganda rinodziya)
- Blisters
Zvimwe zviratidzo zvinoratidzira nhengo pamwe nenzvimbo dzemasero cell infiltration. Zviratidzo zvepabonde zvinowanzosanganisira kurwadziwa kwepamuviri, kunyorera, kusuruvara, uye kurara. Kubatanidzwa kwemasumbu nemapfupa kunogona kuuya nekutambudzika kana osteopenia / osteoporosis (yakaderera masimba emapfupa). Allergic reactions uye anaphylactic reactions zvinogonawo kuitika nekuderera kweropa (hypotension), syncope (kupera simba), kuneta (kuneta), kupera kwemafupa, kuputika kana kuvhara kwemaziso, miromo, rurimi kana huro. Havasi varwere vose vane zviratidzo izvi.
Chii Chinogona Kutora Zviratidzo muMastocytosis?
Vanhu vane mastocytosis havana zviratidzo nguva yose. Dzimwe nguva zviratidzo zvinokonzerwa nezvimwe zvinhu.
- Mishonga: marwadzo anorwadza (morphine, codeine), maitiro asina-steroidal anti-inflammatory (NSAIDs, kufanana ne ibuprofen), vancomycin (antibiotic) kana musimba unozorodza
- Dzidzira
- Rubbing skin, kunyanya kuputika kunobatanidza mastocytosis
- Kunze kwekushisa kunoshanduka
- Zvokudya zvinonaka
- Doro
- Zvirwere
- Surgery
- Kunyorera uye kunamira kubva kune nyuchi, mavhinga, jekete jena, hornets, ants, jellyfish kana nyoka
- Kushungurudzika mupfungwa
Kuongororwa kweMastocytosis
Kuongororwa kwemastocytosis kunonyanya kuiswa pazvinhu zvinokanganisa (nzvimbo, ganda, marora, maitiro emimba, nezvimwewo).
Nokuti zviratidzo zvinogona kusiyana zvakasiyana nekuwana chirwere chinogona kuva chakaoma.
World Health Organization (WHO) yakabudisa zviyero zvekutsvaga mastocytosis iyo inoratidzwa pasi apa.
Mastocytosis Yakagadzikana: Zviratidzo uye ganda bhiopsy zvinopindirana nemastocytosis akacheka uye hapana maitiro akaenderana ne systemic mastocytosis.
Systemic Mastocytosis: Inofanirwa kuva nechigarire chikuru uye imwe tsanangudzo duku kana zvishoma zvitatu zviduku.
- Mutsara mukuru: Pamusoro pemafupa (zvichibva kumapfupa emafuta kana nhengo yakagadzikana) inofanira kuva nenzvimbo dzakawanda dzemasero cell infiltration (inotsanangurwa se ≥15 mamita masero).
- Tsanangudzo shoma:
- Pamusoro pehupopi (kana marota marora kana nhengo yakagadzirirwa): Inopfuura 25 muzana yemasero emasera ari kupinda mukati anonyanya kutarisa (haafaniri kutaridzika senguva dzose mast cells)
- Kucherechedza chimiro chechimiro chinosanganiswa nemastocytosis muropa, marora kana mamwe mhuka ( KIT gene).
- Mast masero mu biopsy anotaura mamwe mavara (CD2 uye / kana CD25) iyo yakakwana yemasero masero haaiti.
- Tryptase (an enzyme inoputsa mapuroteni anowanikwa mumasero masero) ayo anopfuura 20 ng / mL. Aya mazinga anowanzonyanya kukwirira zvikuru mumasero cell leukemia, chirwere chekenza chinonzi mast cell disease.
Mishonga
Kufanana nedzimwe mamiriro ezvinhu akafanana, kuoma kwechirwere kunogadzira kurapa kunoda. Kazhinji kurapwa kwezviyero zvakafanana nechepakati uye yakagadzirwa mastocytosis.
- Dzivisa zvikonzero zvinongororwa pamusoro apa.
- Kuva wakagadzirira vanaphylaxis (yakaoma yega inogadzirisa). Izvi zvinosanganisira kuva nemajekiseni epinephrine awanikwa kumba kuti awane chirwere chinokurumidza kana zvichidiwa.
- Kuchengetedza kurapwa painozivikanwa zvinokonzera hazvidziviriki. Izvi zvinosanganisira prednisone uye anti-histamines (ranitidine, diphenhydramine) kusati kwaita kujekesa semishonga uye chirwere.
- Immunotherapy : Immunotherapy, inozivikanwawo sezvenyeredzi shots yezvipembenene zvinoputika sezvinoita nyuchi, marwadzo, uye zvipembenene zvinogona kushandiswa.
- Antihistamines: Nokuti zviratidzo zvakabatana ne histamine yakasununguka kubva mumasero emasero, hazvishamisi kuti chiremba wako anogona kupa mishonga inonzi antihistamines. Izvi zvinosanganisira mishonga inoshandiswa kurapa mishonga yepamusoro kana kuchenesa seti cetirizine uye hydroxyzine. Mimwe mishonga inosanganisira ranitidine uye cimetidine, iyo inowanzoshandiswa kurapa gastroesophageal reflux.
- Cromolyn: Cromolyn inorapwa mishonga inoshandiswa kuporesa zviratidzo zvepamuviri sekurwadziwa kwepamuviri uye chirwere chinokonzerwa nemasero cell release of histamine. Dzimwe nguva mishonga inogona kuiswa mukati mehuni yezvipembenene zveganda.
- Antileukotriene mishonga: Antileukotriene mishonga se montelukast uye zileuton inogona kushandiswa kana zviratidzo zvisingaitwi ne antihistamines.
- Aspirin: Aspirin inogona kushandiswa kurapa kuputika kweganda.
- Tyrosine Kinase Inhibitors (TKI): I- KIT gene inobata zvinyorwa zveprotini inonzi tyrosine kinase. Kushandurudza kwakawanikwa mumastocytosis kunowedzera mast cell accumulation in tissues. Mishonga inonzi tyrosine kinase inhibitors inogona kubatsira. TKI yakanyatsodzidza zvikuru, imatinib, haisi yakasimba mumastocytosis. Mumwe TKI, midostaurin, iye zvino ari kuidzidzwa mumastocytosis.
- Chemotherapy: Zvimwe zvechisimba zvemastocytosis zvingada kurapwa nemakemikirasi se hydroxyurea uye cladribine.
Mastocytosis inogona kuva dambudziko rakaoma kunzwisisa. Iwe unogona kutsvaga huwandu hwehutano hwechiremba kuti uwane hutachiona uye uwedzere kurapa kwako.
> Sources:
> Castells MC uye Akin C. Mastocytosis (cutaneous and systemic): Epidemiology, pathogenesis, uye kliniki zviratidzo, Mastocytosis (cutaneous and systemic): Kuongorora uye kuongororwa kune vanhu vakuru, Mastocytosis Yakagadzirwa: Kurapa uye kufungidzira, uye Systemic Mastocytosis: Kurapa uye kufungidzira. Mu: UpToDate, Post TW (Ed), UpToDate, Waltham, MA.