Richter's Syndrome (RS), inozivikanwawo seRichter's transformation, inoreva kuchinjwa kwechimwe chirwere chekenza yeropa chaiyo kuva rudzi rwakasiyana, rwechisimba.
RS inotaura nezvekugadzirwa kwepamusoro-grade kwete-Hodgkin lymphoma mumunhu ane chirwere chechirwere chechirwere chechiremera (CLL) / small lymphocytic lymphoma (SLL) . Zvimwe zvakasiyana-siyana zve RS zvinonziwo zvinowanzoitika, zvakadai sokushandura kuHodgkin lymphoma.
Tsanangudzo yemashoko aya uye zvavanoreva zvinotevera.
Overview
RS inotanga kune mumwe munhu atova nekenza yemasero machena eropa. Uyu kenza yekutanga ine mazita maviri akasiyana, zvichienderana nekuti uri mumuviri kanhi inowanikwa: Inonzi CLL kana kenza inowanika muropa nepfupa remarara kana SLL kana yakawanikwa kunyanya mumakirini .
CLL inoshandiswa kuvhara zvose masangano, kuenda mberi munyaya ino.
Kwete Munhu wose ane CLL Inovandudza Richter Syndrome
Kubudirira kweR RS kune vanhu vane CLL hakuwanzoitiki. Inofanirwa kuparidzirwa muna 2016 ndeyekuti Richter's shanduko inowanikwa ne 5 muzana chete yevarwere vane CLL. Mamwe magwaro anotaura mararamiro pakati pe2 ne10 muzana. Kana RS ichiitika kwauri, hazvishamisi kuti yaizoitika panguva imwechete iyo CLL inowanikwa. Vanhu vanovaka RS kubva kune CLL vanowanzoita makore akawanda mushure meCLL kuongorora.
INew Cancer Kazhinji Behaves Aggressively
Kenza mutsva inowanikwa apo munhu ane CLL anoenderera mberi kukura izvo zvinozivikanwa sekushandura, kazhinji kacho kusvika kune yepamusoro-grade kwete yeHodgkin lymphoma (NHL). "Chikoro chepamusoro" chinoreva kanzera inowedzera kukura nekukurumidza. Lymphoma ine kenza yemasero machena eropa lymphocyte.
Maererano neimwe chidzidzo, anenge 90 muzana yemashanduro kubva kuCLL ndeye rudzi rweNHL rwakadana rukuru rweB-cell lymphoma (DLBCL), asi anenge 10 muzana inoshandura kuHodgkin lymphoma.
Iko chaizvoizvo inonzi "Hodgkin yakasiyana yeRichter syndrome (HvRS)" mumamiriro ezvinhu akadaro, uye haisi pachena kana kufanirwa kwechokusiyana kwakasiyana neHodgkin lymphoma. Dzimwe shanduko kubva kuCLL zvinogonawo.
Sei ichinzi Richter Syndrome?
Mumwe murume anonzi Maurice N. Richter akatanga kurondedzera chirwere muna 1928. Akanyora nezvemabharani ane makore 46 akaendeswa kuchipatara uye ane nzira inopfuurira mberi ichienda kurufu. Mukutsvakurudza kwekutsvaga, akafunga kuti pakange pane imwe yaimbove yakashata yakaipa asi asi kubva kune iyo, mhirizhonga itsva yakaita seyakasimuka iyo yakakurumidza kukura uye yakavharidzirwa uye ikaparadza mhuka dzakanga dzave yekare CLL.
Akaraira kuti CLL yakanga yave yakareba kwenguva yakawanda kudarika chero munhu upi zvake aiziva pamusoro pemurwere uyu, uyewo kunyora nezvemaviri kanzira, kana zvipembenene, achiti, "Zvinokwanisika kuti kukura kwechimwe chezvipembenene kwakange kuchienderana nekuvapo kweimwe . "
Mamiriro
Vanhu vane RS vanoita zvirwere zvechisimba nekukurumidza kukurudzira lymph nodes, kuwedzera kwepleen uye chiropa, uye kuwedzera kwemazivo muropa rinonzi serum lactate dehydrogenase, kana kuti LDH.
Survival Rate
Sezvo nemaronda ose, nhamba dzekupona dzinogona kuva dzakaoma kududzira.
Varwere mumwe nomumwe vanosiyana mune hutano hwavo uye simba musati vasangana. Uyezve, kunyange marariya maviri anokwana anogona kuita zvakasiyana chaizvo nevanhu vakasiyana. Asi neRR, zvisinei, kenza itsva yakanyanyisa. Mune vamwe vanhu vane RS, kupona kwave kunorondedzerwa nehuwandu hwemazana emaminitsi mashoma kusvika pakumi gumi kubva pakuongororwa. Zvisinei, dzimwe dzidzo dzakaratidza mwedzi gumi nemasere kupona, uye vamwe vanhu vane RS vanogona kurarama kwenguva yakareba; stem-cell transplantation inogona kupa mukana wekupona kwenguva yakareba.
Zviratidzo nezviratidzo
Kana CLL yako ichishandurwa kusvika kuDLBCL, uchaona kuipa kwezviratidzo zvako.
Zvinhumba zveRS zvinosanganisira kukurumidza kukura kwechirwere pamwe kana kusina kupindira kwepranodal-kureva kuti, kukura kutsva kunogona kuvharirwa kumakirini, kana kenza inogona kusanganisira nhengo dzisiri dzemakirini, sezvakaita spleen nechiropa.
Unogona kuwana:
- Kukurumidza kuwedzera lymph nodes
- Kusuruvara kwepamuviri kwakabatana nekuwedzera kwepleen nechiropa chinonzi hepatosplenomegaly
- Zviratidzo zvepasi tsvuku tsvuku yeropa (anemia), yakadai sekunzwa wakaneta zvakanyanya, ganda rinenge riripo, kupera kwepfufu
- Zviratidzo zveplateletlet count (thrombocytopenia), zvakadai sekusava nekucheka uye kusinganzwisisi kubuda ropa
- Zviratidzo zvekubatanidzwa kwemazuva ano kusanganisira munzvimbo dzisina kujairika, dzakadai seuropi, ganda, maviri, mapapu, nemapapu
Dambudziko rekuchinja
Dambudziko rekudzidzira RS kubva kuLLL harina kufananidzwa nechikwata chako cheukwepa, nguva yakareba sei iwe wakambove nayo, kana rudzi rwekupindura kurapwa kwawakagamuchira. Kutaura zvazviri, masayendisiti haanyatsonzwisisi chinokonzera kuchinja.
Munguva pfupi yapfuura, zvimwe zvidzidzo zvakaratidza kuti varwere vane maLLL masero anoratidza chiratidzo chakanzi ZAP-70 vanogona kuva nehuwandu hwekuchinja. Zvimwe zviratidzo zvakadai sevarwere vane NOTCH1 kuchinja-chinja pakuongororwa kwave kwekutsvakurudza kufarira. Kunyange zvakadaro, dzimwe tsvakurudzo dzakaratidza kuti vaduku vechidiki CLL-kureva, vaduku kune makore makumi mashanu-vanogona kunge vane ngozi yakawedzerwa zvakare.
Imwe nyanzvi ndeyokuti ndeye nguva yakareba kwenguva yakareba uye yakaora mwoyo immune system kubva kuCLL inokonzera shanduko. Mune dzimwe mhando dzevarwere vakangoderedza zvirwere zvekudzivirira muviri kwenguva yakareba, zvakadai sehutachiona hwehutachiona hwehutachiona (HIV) kana kune vanhu vane nhengo dzemuviri, kunewo mukana wekuwedzera weNHL.
Pasinei nokuti chii chingave chiripo, hazvioneki kuti kune chero chinhu chipi zvacho chaungaita kuti utadze kana kudzivirira CLL yako kushandura.
Kurapa uye Kurapa
Kurapa kweRR kunowanzobatanidza chemotherapy protocol dzinowanzoshandiswa kuNHL. Izvi zvinowanzogadzirisa zviyero zvinosvika makumi matatu kubva muzana. Zvinosuruvarisa, chiyero chekupona nekemikirini yenguva dzose iri pasi pemwedzi mitanhatu mushure meRS shanduko. Mishonga mitsva uye kusanganiswa zvinoramba zvichiri kuedzwa mumakiriniki, asi.
Mumakore achangobva kupfuura, zvidzidzo zvakatsvaga kushandiswa kweFludarabine chemotherapy protocol sezvakaratidzwa kuvandudza migumisiro kune varwere vane zvakaoma CLL . Muvhareji kurarama nemhando iyi yemakemikrasi yakawedzerwa kusvika mwedzi 17 muchidzidzo chimwe.
Chimwe chinhu chiri kuitika ndekushandiswa kweatatumab - munhu akazara anti-CD20 monoclonal antibody iyo inotarisa mutemo wakasiyana pamakirini eB. Icho CHOP -O kuongorora kuongorora kuchengetedza, kukwanisa, uye basa rechipothe chemotherapy CHOP pamwe chete neatatumum mukugadziriswa nekugadziriswa kwevarwere vane RS vachangobva kuongororwa. Pakaongororwa zvishoma, vanopfuura vanomwe vevatambi vekutanga 25 vakapindira kupindura kwakakwana kana kusarura mushure mematanhatu matanhatu e CHOP-O.
Zvimwe zvidzidzo zviduku zvakatarisa kushandiswa kwe stem cell transplant kubata chirwere ichi. Vakawanda vevarwere vari mune zvidzidzo izvi vakanga vagamuchira zvakawanda pamberi peymotherapy. Kubva pamhando dze stem cell transplants dzakaedzwa, kushandiswa kusina myeloablative kwaiva nehuwandu hwehutachiona, huri nani huwandu, pamwe nekugona kwekuregererwa . Zvimwe zvidzidzo zvichadikanwa kuti zvione kana ichi chiri chakanakidza chevarwere veR RS.
Future Research
Kuti zvive nekuvandudza kupona kune varwere vane RS, masayendisiti anoda kuwana kunzwisisa zviri nani chezvinoita kuti kushanduka kubva kuLLL kuitike. Uine humwe ruzivo pamusoro peRR pane imwe nzvimbo dzemasero, zvirwere zvinotarisirwa zvakanaka zvinogona kugadzirirwa pane izvo zvisiri izvo zvakashata. Zvisinei, nyanzvi dzinonyevera kuti sezvo pane huwandu hwakaoma hwakawanda hwema molecule huchibatanidzwa neRR, hapangambove neine "chinangwa chose" chinotarisirwa uye kuti chero ipi zvayo yemishonga inogona kudikanwa kusanganiswa nekemotherapy nguva dzose kuti iwane yavo zvakanyanya kunaka. Sezvo masayendisiti anobudisa zvikonzero zve RS, vari kuona kuti RS haisi yunifomu imwechete kana kuti inogadziriswa.
Munguva iyi, varwere vakaita kuti CLL yavo ishanduke kusvika kuR inokurudzirwa kunyoresa mune zvidzidzo zvechirwere ichi kuedza kugadzirisa zvirongwa zvekurapa uye zvabuda kubva pane zvino.
Sources:
Richter, M. Generalized reticular cell sarcoma ye lymph nodes inosangana ne lymphatic leukemia . The American Journal of Pathology. 1928; 4; 4. 285-292.
Mutsva Inovandudzwa muRichter Syndrome. Eyre TA, Clifford R, Roberts C, et al. Simba rimwe NCRI chikamu chechipiri chekudzidza che CHOP pamwe chete neOatumumab mukugadziriswa nekuchengetedzwa kwevarwere vane Hurter's syndrome. BMC Kanzura . 2015; 15: 52.
> Parikh SA, Habermann TM, Cha ee KG, et al. Hodgkin kuchinja kwechirwere lymphocytic leukemia: Kuitika, zvibereko, nekuenzanisa ne de novo Hodgkin lymphoma. Am J Hematol. 2015; 90: 334-38.
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