Waldenstrom's macroglobulinemia: zviratidzo, zvinokonzera, kufungidzira

Zvirwere zvinokonzerwa neropa hazvishamisi, asi zvinowedzera njodzi kuvanhu. Chimwe chezvirwere izvi ndiVandelstrom's macroglobulinemia.

Izvo zvakaratidzwa sei?

Pasi pechirwere ichi mune mushonga wemazuva ano kunonzwisiswa nhaka yehematologic syndrome, pamwe chete nekuvapo muropa re macroglobulin. Iyi proteni inoumbwa semugumisiro webasa re monoclonal B-lymphoid zvimwe zveropa (B-lymphocytes).

Kazhinji, aya masero anokonzera kushandiswa kweM-globulin muropa. Apo pfupa remashonga rinoshanda rinokanganiswa (kazhinji nekukura kwemaitiro ekutura), maitiro eamino acid uye kuenzaniswa kwegeni kunoshanduka, izvo zvinotungamirira kushandiswa kwe macroglobulin, i.e. Waldenstrom's macroglobulinemia inokura.

Chirwere chacho hachiwanzoitiki, zvinenge zvitatu pamamiriyoni evanhu. Inonyanya kunyanya kune varume vanopfuura makore makumi mashanu. Kusvikira kumakore makumi mana, chirwere ichi hachina kuwanikwa.

Chirwere chacho chinoratidzika sei pamagetsi uye mavara? Ndezvipi zvivako zvinobatwa kutanga uye zvinoitika sei semugumisiro?

Pathogenesis yechirwere

Iyo pathogenesis yechirwere ichi inobva pakuwedzera kwemasero ejekiti yakaipa ye plasma zvinogona kugadzira immunoglobulins yekirasi M. Aya masero anopinda mumapfupa emapfupa, chiropa, spleen, apo pathogenic macroglobulin inotanga kuwedzera uye kuvhara. Nokudaro, maWaldenstrom's macroglobulinemia inokura.

Kubatana kwepathogenic protein muropa kunotungamirira kukurudzirwa kwezvisikwa zvayo. Somugumisiro, muyero weropa unoyerera mukati memidziyo unoderera, izvo zvinotungamirira kumararamiro ayo. Mukuwedzera, zvinhu zvinodzivirira plasma zvinodzingwa (kuburikidza nekuvhara uye kuvadzivisa nema macroglobulins). Nemhaka yeizvi, hutachiona hwema thrombus hunovhiringidzwa, izvo zvinozoita kuti kuwedzere kubuda ropa.

Maziso, kana iwe uchidzidza pasi pe microscope masero epafupa, iwe unogona kuona masango e "plasma" lymphocytes, nhamba yakawanda yeB-lymphocytes yakakura uye kuvhara mutevedzeri wekuvhiringidza kwemasero emasero. Zvose izvi masero zvinoratidza kuti Waldenstrom macroglobulinemia inoitika.

Zviratidzo

Ndezvipi zvekuchipatara zvinokwanisika kufungidzira kubatanidzwa kwechirwere ichi?

Chokutanga pane zvose, varwere vacharamba vachinyunyuti pamusoro pezviratidzo zvisingaoneki - kushaya simba kwose, pasi-grade fever kwenguva yakareba, kutuka, kuora muviri kwechikonzero chisingaoneki.

Chiratidzo chikuru, pamusana pekuti isu tinogona kufungidzira kuti kufambiswa kweVandelstrom's macroglobulinemia, inowedzera kubuda kubva mumhino uye mucous membrane yemasamu. Kazhinji kacho kazhinji kacho akacherechedza marwadzo echisimba uye matambudziko.

Nokufamba kwenguva, kukura kwe hepatosplenomegaly uye lymphadenopathy (yakawedzera lymph nodes) inogoneka. Izvo zvinokwanisika kuwedzera matambudziko e macroglobulinemia.

Kana pasina nguva yakakodzera yezvechiremba, kune ngozi yekuda mumurwere uye kunyange rufu (zvisinei, varwere havawanzofa zvakanyanya kubva pakuvandudzwa kweWaldenstrom's macroglobulinemia). Zvinokonzerwa nekufa kwavo ndezvikonzero zvezvinetso zvakagadzirwa.

Matambudziko echirwere chacho

Ndezvipi zvinogona kukonzera chirwere ichi?

Chokutanga, mhepo yemwoyo inobatanidzwa mukugadzirisa zvirwere, iyo midziyo miduku - capillaries, arterioles nemavuta. Nemhaka yekuderedza kubuda kweropa, retinopathy uye nephropathy zvinowanikwa. Itsvo dzakabatwa. Muchiitiko ichi, kukurudzirwa kwekudzivirira kwemidziyo ye glomerular uye urolithiasis nekuiswa kwehutano chinhu.

Waldenstrom's macroglobulinemia inoratidzwa nekuderera kwehutachiona uye kuwedzera kwehutachiona hunobatsira hunoomesa dzidzo uye kuongororwa kwechirwere chepfungwa.

Sezvo urongwa hwacho huri kufambira mberi, kudzvinyirirwa kwenzvimbo dzose dzezvirwere zve hemopoiesis uye kukurudzirwa kwepancytopenia kunoonekwa. Unhu hunoitika kukura kweamyloidosis uye kukanganisa kwepuroteni iyi yehutachiona yemidzi yeropa yechiropa uye spleen, iyo inowedzera kuipa basa ravo rakatodzwa.

Diagnostics

Ndezvipi zviratidzo zvichabatsira kunzwisisa kuti macroglobulinemia yakagadzirwa?

Chokutanga pane zvose, iwe unofanirwa kubhadhara kuongorori rekuongorora ropa. Zviratidzo zvikuru zvinoratidza chirwere ichi chichava kuwedzera kwe ESR uye kuumbwa kwe "mahwendefa" chaiwo - akabatanidzwa pamwechete erythrocytes. Ikokurocyte maitiro anowanzoratidza kuwedzera kwenhamba ye lymphocyte uye kuonekwa mukutsvaga kwemaitiro asina kuchena emalaukocyte.

Immunoelectrophoresis inoratidzwa nokuda kwekusimbiswa kwehutano. Mushure mokunge uchitora tsvakurudzo yakapiwa zvinokwanisika kuzivisa yakawanda yakakwira muropa re immunoglobulins yekirasi M.

Monoclonal immunoglobulins inowanikwa muropa mushure mekuwedzera kwezvinyorwa zita.

Zvimwe asi zvisingaoneki zviratidzo ndezvekuwedzera kwechiropa uye spleen, kuonekwa kweamyloid mu renal capillaries ne biopsy, uye kuderera kwenhamba yezvinhu zvinodzima plasma (kunyanya, chikonzero 8).

Kurapa

Muchikamu chekutanga chechirwere, kana pasina chiremera chakakosha chemuviri wechipfuva, mushonga chaiwo hauna kuratidzwa. Kazhinji varwere vakadai vari kutarisirwa kune dzimwe nzvimbo kune imwe nzvimbo yezvematema.

Nechitarisiko chekutanga zviratidzo uye kusimbisa kuti chirwere ichi - Waldenstrom's macroglobulinemia, tanga chirwere chakanangana ne cytostatics. Nokushandisa kurapwa semishonga yakadai se "Chlorbutin", "Cyclophosphanum". Vane chinokonzera chetostatic uye vanodzvinyirira basa reMalignant B-lymphocyte clone.

Ita panzvimbo yekutanga "Chlorbutin" ye 6 mg zuva nezuva mukati mavhiki 3-4. Kuwedzera kwenzara kunowanikwa nekufambisa kwemafupa aplasia. Mushure mokupfuura gwara guru rekurapa sarudza kugadzirisa doses rechirwere (2-4 mg) imwe neimwe zuva.

Kuvandudza rheological parameters yeropa, plasmapheresis inoshandiswa. Nzira yekurapa ne plasmapheresis inoitwa panguva yekushandiswa kwe cytostatics. Nzira yacho inoratidza kubviswa kwemasviki maviri emaplasma, kuitora iyo nemupiro, isingaiti.

Kudzivirira

Sezvo chirwere chacho chiri nhaka, zvakaoma munzira ipi neipi yekukanganisa kukura kwayo, nokuti yakatoiswa mukati memajeni. Nzira imwe chete yekudzivisa hutungamiri hwayo ndiyo nguva yekuongorora chirwere ichi uye chirongwa chinoronga chirongwa.

Kukanganisa kwakashata pane chirwere kunogona kuonekwa nehupenyu hwakanaka, kudzivisa kusvuta uye doro, kurega kusvuta uye zvinonhuwira ndiro.

Sezvo chirwere chacho chiri chimiro, dzimwe nguva mamiriro ezvinhu anokuvadza anogonawo kumutsa kukwidziridzwa kwayo - kushanda ne aniline dyes, peint, varnishes.

Kakawanda zvirwere zvehutachiona zvinogonawo kuchinja gome revanhu. Kurapa kwezvirwere zvakadaro panguva yakakodzera kunobatsira kudzivirira kuchinja kwemajini uye kudzivirira kukura kwe macroglobulinemia.

Matanho ekudzivirira anosanganisira kudzidzira vanachiremba vane hutano hwechirwere uye vachivamanikidza kutevera mabasa ehupenyu hwehupenyu.

Zviratidzo zvechirwere

Chii chakamirira varwere vakagadzira Waldenstrom macroglobulinemia? Kufanirwa kwechirwere ichi kunobva paukukutu hwechirwere, kukura kwezvinetso uye nguva yekurapa yakatanga.

Kana chirwere ichi chiri padanho rekutanga rekufambira mberi, mumatambudziko mazhinji zvinogona kuvharidzira huri kufambira mberi. Varwere vakadaro, kunyange kana vasina mukana wekurapa kwakakwana, kushandiswa kwekuchengetedza kwema cytostatics kuchiri kubvumira kunyanya kuwedzera nguva yehupenyu hwavo.

Zvinhu zvakanyanya kuipisisa zviri kune avo vakambove vakaoma veWalddenstrom macroglobulinemia. Zviratidzo, izvo zvinoratidzirwa zvisinganzwisisiki - kuora kweamyloid pachiropa uye neitsvo, kubuda ropa nguva dzose uye nekuvandudzwa kwemavara emapapu. Kana iwe usingaiti rubatsiro rwakakodzera kune vakadai vakadaro, mukana wekufa kunowedzera.

Ivhareji yekutarisira upenyu kune varwere vane chirwere ichi ndeye makore 4-5. Nerongwa hwakarongwa hwekurapa, zvinokwanisika kuwedzerwa iyo kusvika makore 9-12.

Ngozi yezvirwere

Zvichipa kuti chirwere ichi chinowanzosawanzoitika, zvakaoma zvikuru kufungidzira kufambira mberi kwayo kumunhu asina kumboonana naye. Zvichibva zvave kuti vesayendisiti vedunhu vanosangana nemurwere pakutanga, pane pamafudzi avo avo vane mhosva panguva yakakodzera uye vanofungidzirwa Waldenstrom macroglobulinemia. Chirwere ichi chii, kwete vese vanachiremba vachayeuka, asi kufunga kuti murwere ane chirwere ichi, achifunga zvose zviratidzo zvekurapa, anofanirwa nemumwe mushandi.

Mune ramangwana, murwere uyu acharamba ari hematologists, asi kunyanya kuongororwa kunongova pamapfudzi echiremba pa polyclinics.

Kutsunga kusina nguva kwechirwere ichi kunotungamirira kumigumisiro yakaipisisa apo chemotherapy haisisiri yakashanda. Ndicho chikonzero iwe unofanirwa kuziva nezvechirwere ichi zvose, kuitira kuti urege kuchitadza uye usingaiti kuti uende pasi.