Mastocytosis ke molomo. Ho utloa hore uena kapa moratuoa oa hau o na le boemo bo bongaka boo u sa kang ua bo utloisisa bo ka tšosa. Mastocytosis e ka boela ea bitsoa mast cells lefu. Lisele tse kholo ke mofuta oa sele e tšoeu ea mali ea sesole sa hau sa 'mele. Li amana le basophil 'me li ameha ho hola habonolo le anaphylaxis (e leng tšoaetso e matla).
Ha e le hantle, lisele tse ngata li hohela lisele tse ling tsa 'mele tsa ho itšireletsa mafung ho ea libakeng tsa lisele tse hlokahalang ka ho lokolla lik'hemik'hale. Ha palo e ngata haholo ea methapo ea mast e bokana hammoho ka lik'hemik'hale, e bitsoa mastocytosis. Ha methapo ea mast e bokella letlalong feela e bitsoa mastocytosis e nyenyane. Ha e hlaha likarolong tse 'maloa e bitsoa systemic mastocytosis. Mastocytosis e hlophisitsoeng e nkoa e le mokuli oa myeloproliferative neoplasm .
Likotsi, Lipontšo le Matšoao a Mastocytosis
Hobane mastocytosis ke mathata a sa tloaelehang joalo ha ho tsejoe hore na batho ba bangata ba na le eng. Ho bonahala banna le basali ba amehile ka tsela e tšoanang. Ho bana, boholo ba makhetlo a mangata ha a le ho batho ba baholo tsamaiso ea mastocytosis e tloaelehile.
Hobane mastocytosis e ka hlaha likarolong tse ngata, matšoao a bontšang a ka fapana haholo. Bongata ba matšoao a hlaha ha lisele tse ngata li lokolla histamine le lik'hemik'hale tse ling.
Lintho tse fumanoang ke letlalo ke tse tloaelehileng ka ho fetisisa '
- Rash: Ho na le mefuta e 'maloa ea li-mastocytosis. Ka linako tse ling e ka 'na ea e-ba libaka tse mongobo / tse sootho tse shebahalang joaloka li-freckles. Ka linako tse ling ho bonahala eka matheba a khubelu letlalong.
- Ho hlatsoa (pruritus): Lebelo le ka 'na la senya haholo ha le halefisitsoe (le tsitsitsoe) kapa ha le hlahetsoe ke phetoho ka tšohanyetso (joalo ka metsi a chesang ka ho sesa). Sena se bitsoa letšoao la Darier.
- Ho ruruha (letlalo le khubelu, le futhumetseng)
- Blisters
Matšoao a mang a bonahatsa setho le libaka tsa mast cell infiltration. Matšoao a mafu a tloaelehileng a tloaelehile 'me a kenyelletsa bohloko ba mpeng, ho nyekeloa ke pelo, ho hlatsa le letšollo. Ho kenngoa ha mesifa le masapo ho ka hlahisa bohloko kapa osteopenia / osteoporosis (matla a fokolang a bone). Matšoao a hlaselang le lik'hemik'hale tsa anaphylactic li ka boela tsa etsahala ka khatello e tlase ea mali (hypotension), syncope (ho tepella), mokhathala (ho khathala), ho fokola kapele, ho ruruha kapa ho ruruha ha mahlo, melomo, leleme kapa 'metso. Hase bakuli bohle ba nang le matšoao ana.
Ke Eng e ka Etsang Matšoao Mastocytosis?
Batho ba nang le mastocytosis ha ba na matšoao nako eohle. Ka linako tse ling matšoao a bakoa ke lintho tse ling.
- Meriana: meriana ea bohloko (morphine, codeine), meriana e sa thibeleng ho ruruha (non-steroidal anti-inflammatory (NSAIDs, joaloka ibuprofen), vancomycin (antibiotic) kapa mesifa e phopholetsang
- Koetlisa
- Ho hloekisa letlalo, haholo-holo lebelo le amanang le mastocytosis
- Mocheso o feteletseng o fetoha
- Lijo tse monate
- Joala
- Matšoao
- Ho buuoa
- Ho loma le ho lla ho tloha linotši, litlhoko, liphahlo tse tšehali, hornets, likokoanyana, jellyfish kapa linoha
- Khatello ea maikutlo
Tlhahlobo ea Mastocytosis
Ho hlahlojoa ha mastocytose ho lebisitsoe haholo ho li-biopsies tsa sebaka seo se amehileng (letlalo, bone la mokopa, pampiri ea meno, joalo-joalo).
Hobane matšoao a ka fapana haholo ho fumana lefu lena le ka ba thata.
Mokhatlo oa Lefatše oa Bophelo (WHO) o phatlalalitse melao ea ho hlahloba mastocytosis e hlahlojoang ka tlase.
Mastocytosis e fokolang: Matšoao le botlalo ba letlalo li lumellana le mastocytosis e senyehileng 'me ha ho na lintho tse lumellanang le systemic mastocytosis.
Mastocytosis e hlophisitsoeng: E tlameha ho ba le moelelo o moholo le mohato o le mong o nyane kapa bonyane mohato o monyenyane o mararo.
- Tlhaloso e kholo: Tlhahisoleseding (e tsoang ho bone marrow kapa litho tse ammeng) ho tlameha ho ba le libaka tse ngata tsa mast cell infiltration (e hlalosoang e le ≥15 mast cells).
- Tlhaloso e nyenyane:
- Ho biopsy (ebang ke bone ea 'mele kapa setho se amahanngoeng): Ho feta karolo ea 25 lekholong ea lisele tsa mast e kenang ka tlaase ho tsona li na le matla a tlhaho (ha li shebahale eka li tloaelehile)
- Ho lemoha phetoho ea liphatsa tsa lefutso e amanang le mastocytosis ka mali, masapo a mongoli kapa lisele tse ling ( KIT ea liphatsa tsa lefutso).
- Lisele tse nyenyane ka ho hlahisa limela li hlalosa likarolo tse ling tse eketsehileng (CD2 le / kapa CD25) tseo lisele tse tloaelehileng tsa mast li sa li tsebeng.
- Tryptase (e leng enzyme e senyang protheine e fumanoang meleng e mengata) e fetang 20 ng / mL. Hangata litekanyetso tsena li phahame ka ho fetisisa meleng ea kankere ea kankere ea mali, e leng mofuta o mong oa kankere oa lefu la mast cell.
Litlhare
Ho tšoana le maemo a mang a tšoanang, bothata ba maloetse bo etsa hore phekolo e hlokahale. Litekanyetso tse tloaelehileng tsa phekolo li tšoana le mastocytosis e fokolang le e sebetsang.
- Qoba lits'ebetso tse hlalositsoeng ka holimo.
- Ho itokiselitse banaphylaxis (bohato bo matla ba ho itšoara hantle). Sena se kenyelletsa ho ba le liente tsa epinephrine tse teng lapeng bakeng sa kalafo ea kapele haeba ho hlokahala.
- Ho thibela phekolo ha batho ba tsejoang ba ke ke ba qojoa. Sena se ka kenyelletsa prednisone le anti-histamines (ranitidine, diphenhydramine) pele ho hlahisa likokoana-hloko joaloka liente le opereishene.
- Immunotherapy : Immunotherapy, eo hape e tsejoang e le letšoao le fokolang la likokoanyana tse kang ho linotši, likokoanyana le likokoanyana li ka sebelisoa.
- Antihistamines: Hobane matšoao a amana le ho lokolloa ha histamine ho li-cell mast, ha ho makatse hore ngaka ea hau e ka fana ka meriana e bitsoang antihistamine. Tsena li tla kenyelletsa meriana e sebelisoang ho phekola menoana kapa ho hlohlona joaloka letirizine le hydroxyzine. Meriana e meng e kenyelletsa ranitidine le cimetidine, tseo hangata li sebelisetsoang ho phekola reflux ea gastroesophageal.
- Cromolyn: Cromolyn ke meriana ea molomo e sebelisoang ho tšoara matšoao a moriana joaloka bohloko ba mpeng le letšollo le hlahisitsoeng ke cell cell release of histamine. Ka linako tse ling meriana e ka 'na ea kenngoa ka mafura bakeng sa lisele tsa letlalo.
- Meriana ea Antileukotriene: Meriana ea Antileukotriene e kang montelukast le zileuton e ka sebelisoa haeba matšoao a sa laolehe ka antihistamine.
- Aspirin: Aspirin e ka sebelisoa ho phekola ho phatloha ha letlalo.
- Tyrosine Kinase Inhibitors (TKI): Melao ea liphatsa tsa lefutso tsa KIT e ama liprotheine tse bitsoang tyrosine kinase. Phetoho e fumanoang mastocytosis e eketsa lisele tse ngata tse bokellaneng ka mahlaseli. Meriana e bitsoa tyrosine kinase inhibitors e ka 'na ea e-ba ea bohlokoa. TKI e rutehileng haholo, imatinib, ha e sebetse mastocytosis. E 'ngoe TKI, midostaurin, e ntse e ithutoa ka mastocytosis.
- Chemotherapy: Mefuta e meng e mabifi ea mastocytosis e ka hloka phekolo ka chemotherapy e kang hydroxyurea le cladribine.
Mastocytosis e ka ba boloetse bo thata ho utloisisa. U ka 'na ua hloka lingaka tse khethehileng tsa lingaka hore u fihlella lefu lena le ho ntlafatsa phekolo ea hau.
> Mehloli:
> Castells MC le Akin C. Mastocytosis (cutaneous and systemic): Epidemiology, pathogenesis, le litlhaloso tsa meriana, Mastocytosis (cutaneous and systemic): Hlahloba le ho hlahlojoa ho batho ba baholo, Mastocytosis e fokolang: Kalafo le boleloa, le Mastocytosis ea Ts'ebetso. Ka ho: UpToDate, TW Post (Ed), UpToDate, Waltham, MA.