ImpiloIzifo nemibandela

Macroglobulinemia Waldenstrom sika: izimpawu, izimbangela, onalesi sifo

izifo igazi Ukubambezeleka ezingajwayelekile, kodwa bayingozi abantu. Omunye isifo ezinjalo kuba Vandelstrema macroglobulinemia.

Kunalokho libhekene?

Ngaphansi kwalesi sifo ngo-inqubo yokwelapha yesimanje libhekisela a syndrome lezinceku hematological ephelezelwa khona igazi macroglobulin. Leli namaprotheni by umsebenzi monoclonal egazi B-lymph (B-lymphocyte).

Ngokuvamile, la mangqamuzana banesibopho synthesis igazi-M globulin. Lapho yimuphi umsebenzi umnkantsha (ngokuvamile ekuthuthukiseni yenqubo isimila) ushintsho acid acid ukwakheka kanye ukulandelana isakhi sofuzo, okuholela zamagama macroglobulin, isb ekuthuthukiseni macroglobulinemia Waldenstrom sika.

Lesi sifo ngempela ezingavamile, mayelana 3 amakesi isigidi sabantu. Kutfutfukisa ikakhulukazi amadoda phezu kweminyaka eyi-55. lokhu zokugula cishe ingekho kufika eminyakeni engu-40.

Pho-ke, masibonise isifo emazingeni yeselula nezicubu? Yikuphi izakhiwo iyathinteka kwasekuqaleni nokuthi ngenxa yalokho kukhona?

I pathogenesis lesi sifo

At inhliziyo patogenza lesi sifo singumbulali ekwandeni abulalayo plasma amaseli Clone amaseli uyakwazi ukuthuthukisa immunoglobulin M. La mangqamuzana bahlasele umnkantsha, isibindi, ubende, lapho aqala ukuba ngenkuthalo ziyanda futhi secrete macroglobulin pathogenic. ekuthuthukiseni Ngakho macroglobulinemia Waldenstrom sika.

Ukubuthelela amaprotheni pathogenic egazini kuholela ukuthuthukiswa viscosity yayo. Ngenxa encishisiwe igazi ukugeleza rate ngokusebenzisa izitsha, okuholela yokujama tsi yayo. Ngaphezu kwalokho, engasenamahloni plasma ezijiyisa igazi (ngenxa kwemibhalo kanye okunqanda kubo macroglobulins). Ngenxa yalokhu kwaphazamisa ihlule kumiswa evamile okwagcina kuholela ukopha anda.

Ngokubukeka, lapho kuhlolwa ngesibonakhulu, amaseli umnkantsha, kungabonwa kunenqwaba "plazmatizirovannyh" lymphocyte, inani elikhulu ovuthiwe B-lymphocyte futhi secreting balamuli ukuvuvukala we insika amaseli. Konke lokhu amaseli kusikisela ukuthi kukhona macroglobulinemia Waldenstrom.

izimpawu

kanjani emtholampilo zixwaywe kwalesi sifo?

Iziguli lokuqala lizoba esekhala izimpawu nonspecific - ubuthakathaka jikelele, ongaphakeme-ebangeni imfiva isikhathi eside, ukujuluka, kwesisindo ngaphandle kwesizathu.

Uphawu oyinhloko, ngesisekelo esingakwazi usola ukuthi savela Vandelstrema macroglobulinemia, is kukhuphuke ngopha emakhaleni, izinsini nolwelwesi lwamafinyila. Okuningi kancane njalo waphawula nemihuzuko ongama nemihuzuko.

Ngokuhamba kwesikhathi, kungenzeka ukuthuthukisa hepatosplenomegaly futhi lymphadenopathy (ukuvuvukala zamanzi). Kungenzeka futhi ukuthuthukiswa yezinkinga macroglobulinemia.

Lapho ukwehluleka ukunakekela okuhle kwezokwelapha ngesikhathi esifanele kunobungozi yesiguli ikhoma, ngisho nokufa (kodwa ukuba izigulane ezifayo ngokuvamile hhayi kakhulu kulokho bezwa, bayahlupheka yini kusukela macroglobulinemia Waldenstrom sika). Izimbangela beshona kukhona ngenxa yobunzima athuthukile.

yezinkinga lesi sifo

Kungashiwo okufanayo nzima kanjani lesi sifo?

Ngokuyinhloko abahilelekile zokucabanga okubangelwa isifo esimiso senhliziyo nemithambo yegazi, okungukuthi izitsha ezincane - capillary, arteriole futhi ama-venule. Ngokunciphisa ukugeleza kwegazi waphawula ukuthuthukiswa retinopathy, nephropathy. ukulimala kwezinso. Kulokhu libhekene ukuthuthukiswa glomerular occlusion semithambo kanye urolithiasis nge sebethule urate.

Macroglobulinemia Waldenstrom libhekene ukungatheleleki ongaphakeme nokujoyina izifo ezingosomathuba nzima nokuhlola lesi sifo ecashile.

Njengoba ukuqhubeka nqubo ukucindezelwa okuphawulekayo cishe wonke amagciwane of hematopoiesis kanye nokuthuthukiswa pancytopenia. Amyloidosis libhekene ukuthuthukiswa kanye izilonda sokugembula Ngokusho amaprotheni isibindi nezitsha ubende igazi, elibuye siba sibi futhi ngakho umsebenzi wabo kahle.

ukuhlola

Yini amamethrikhi kuzokusiza uqonde ukuthi savela macroglobulinemia?

Okokuqala, kufanele sinake CBC. Izinkomba oyinhloko sifo iyokwanda esikhathini erythrocyte sedimentation izinga kanye kumiswa "rouleaux" ethize - ukuhlala ndawonye kwamangqamuzana egazi abomvu. WBC ngokuvamile sibonisa inani elikhulu lama-lymphocyte ekucubunguleni futhi ukubukeka leukocyte beya amafomu.

Ukuze uqinisekise uphethwe ngokuqhuba izifundo Immuno. Ngemva kokwenza lokhu cwaningo awukwazi ukubona isamba zandiswe immunoglobulin igazi M

immunoglobulin, ayisiza Monoclonal zinqunywa egazini ngemva kwalokho sera ilebula.

izimpawu engeziwe, kodwa hhayi othize zihlanganisa i isibindi lakhulisiwe futhi ubende, ukuthola amyloid e biopsy kappillyarah izinso, kanye ukunciphisa isibalo plasma ezijiyisa igazi (ngokukhethekile, isici 8).

ukwelashwa

Esikhathini kusaqalwa isifo, ngaphandle uma kunezizathu abalulekile yangaphakathi isitho ukuzibandakanya, uhlobo oluthile lokwelapha aluboniswa. Ngokuvamile, lezi iziguli ngaphansi kweso lwezokwelapha ngesikhathi eyisazi wesifunda.

Lapho izimpawu zokuqala bese uqinisekisa ukuthi isifo - Waldenstrom macroglobulinemia, baqale ukwelashwa oluthile cytostatics. Ukuze ukusetshenziswa ukwelashwa izidakamizwa ezifana "hlorbutin", "Cyclophosphamide". Sinenkosi nomphumela cytostatic nokucindezela umsebenzi Clone abulalayo B-lifotsitov.

Esimisiwe endaweni "hlorbutin" owokuqala 6 mg nsuku zonke ngomlomo for 3-4 amasonto. Ukwandisa umthamo igcwele ukuthuthukiswa umnkantsha aplasia. Emva kokuphothula Inkambo yokwelashwa ebekiwe yesondlo Ukweqiwa izidakamizwa (4.2 mg) Njalo ngemva kosuku olulodwa.

Ukuze uthuthukise igazi imingcele rheological esetshenziswa plasmapheresis. ukwelashwa plasmapheresis Kwenziwa ngoba ukusetshenziswa kwezidakamizwa cytotoxic. Ukuze inqubo ukususwa kuboniswe 2 amalitha plasma nge donor esikhundleni ku inactivated yayo.

zokuvimbela

Ngoba lesi sifo sitholakala ngofuzo, kunzima nganoma iyiphi indlela ezithinta ukuthuthukiswa kwawo, ngoba kakade wabeka phansi ezakhini zofuzo. Okuwukuphela kwendlela yokugwema ukuthuthukiswa ukuxilongwa esifike ngesikhathi lesi sifo futhi icebo kahle ungenisa yokwelashwa.

Ngokungaqondile kuthinte isifo kungaba kuncike ngendlela enempilo, ukuyeka ukubhema utshwala, umkhawulo ngibhema futhi izitsha okubabayo.

Njengoba lesi sifo zofuzo, ke kungase kubangele ukuthuthukiswa futhi kwezinye izimo ezimbi yayo - ukusebenza aniline udayi, upende, varnishes.

izifo njalo viral bayakwazi ukushintsha izakhi zofuzo zomuntu. Ukwelashwa kusenesikhathi lezi zifo kuzosiza ukuvimbela izinguquko ezakhini zofuzo futhi kuvimbele ukuthuthukiswa macroglobulinemia.

Izinyathelo akunakubangelwa yazi iziguli kanye izici isifo nokukhuthaza imisebenzi yabo ukuze ihambisane nendlela yokuphila enempilo.

Izibikezelo

Yini elindela iziguli abaye bahlakulela Waldenstrom macroglobulinemia? Izibikezelo lesi sifo incike ngobucayi isifo eziye nezinkinga zempilo futhi ukwelashwa esifike ngesikhathi.

Uma lesi sifo ngo isigaba saso iqale isencane, ezimweni eziningi kungenzeka ukuvimbela intuthuko yayo. Iziguli ezinjalo ngisho nethuba lokuthi uzophulukiswa ephelele, ukusetshenziswa nokugcinwa esenikwe cytostatics namanje ukwandisa ukuphila kwabo isilinganiso sobude.

Kuyinto zimbi kakhulu kulabo nge macroglobulinemia kuyinkimbinkimbi Waldenstrom sika. Izimpawu, onalesi sifo okuyinto esidumazayo kakhulu - isibindi amyloid nezinso, ukopha nokuthuthukiswa njalo ikhoma paraproteinemic. Uma isikhathi kungasizi iziguli ezinjalo, awokuthi ukufa.

Isikhathi esivamile okulindeleke ukuba iziguli lesi sifo cishe sekudlule iminyaka engu 4-5. Lapho ukudweba kahle lapho kwenziwa uhlelo lokusetshenziswa ukwelashwa kungase kwandise ke iminyaka engu-9-12.

Ingozi yesifo

Njengoba sazi ukuthi isifo kwenzeka akuvamile, kunzima ukuba usola kuye ukuthuthukiswa indoda owacishe ungakaze uhlangane naye. Njengoba sazi ukuthi ukuthintana wokuqala nge abelaphi wendawo lesiguli, kuba emahlombe abo, futhi unesibopho sokuqinisekisa ukuthi ngesikhathi esifanele kusolakala futhi kwatholakala ukuthi nginesifo macroglobulinemia Waldenstrom sika. Siyini ngempela lesi sifo, ngeke zikhunjulwe, akubona bonke Nokho odokotela, ukuba ucabange ukuthi isiguli usanda inikezwe zokugula, ikhumbula zonke zako yomtholampilo, kufanele iyiphi owelapha.

Esikhathini esizayo, lolu hlelo nesineke kuzoholela zegazi, kodwa uphethwe eyinhloko oba kuphela emahlombe emitholampilo odokotela.

ukuhlonza Late lesi sifo kuholela nemiphumela eqatha lapho ngelashwa nangemithi ayisasebenzi. Yingakho kufanele wazi ngalesi sifo zonke ungalahlekelwa ke futhi ungasingenisi ukuba sesimeni sokunganakwa.

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