Richter's Syndrome (RS), yemwenso amadziwika kuti kusintha kwa Richter, imatanthawuza kusintha kwa mtundu umodzi wa khansa ya magazi kukhala mtundu wosiyana, wowawa kwambiri.
RS imatanthawuza chitukuko cha apamwamba kwambiri osati a Hodgkin lymphoma mwa munthu amene ali ndi matenda aakulu a khansa ya m'magazi (CLL) / small lymphocytic lymphoma (SLL) . Mitundu ina ya RS imadziwika kuti ikuchitika, monga kusintha kwa Hodgkin lymphoma.
Kulongosola kwa mawu awa ndi kufunika kwawo kumatsatira.
Mwachidule
RS imayamba mwa munthu yemwe ali kale ndi khansa ya maselo oyera. Khansara yoyamba ili ndi mayina awiri osiyana, malingana ndi malo omwe mumatenda khansa amapezeka: Amatchedwa CLL ngati khansara imapezeka m'magazi ndi mafupa kapena SLL ngati imapezeka makamaka m'matumbo .
CLL imagwiritsidwa ntchito poyang'anira zigawo zonse ziwiri, kupita patsogolo m'nkhaniyi.
Sikuti aliyense amene ali ndi CLL Amapanga Richter Syndrome
Kukula kwa RS mwa anthu omwe ali ndi CLL ndi wamba. Malingaliro omwe amalembedwa mu 2016 ndi kusintha kwa Richter kumachitika pafupifupi 5 peresenti ya odwala omwe ali ndi CLL. Zina zimatchula kusiyana pakati pa 2 ndi 10 peresenti. Ngati RS ikukuchitikirani, ndizodabwitsa kwambiri kuti izi zidzachitika nthawi yomweyo CLL ikupezeka. Anthu omwe amapanga RS kuchokera ku CLL kawirikawiri amatero patapita zaka zingapo pambuyo pa matenda a CLL.
Kansa Yatsopano Kawirikawiri Behaves Mwachangu
Khansara yatsopano imapezeka pamene munthu yemwe ali ndi CLL akupitiriza kukula zomwe zimadziwika kuti kusintha, nthawi zambiri kupita ku msinkhu wapamwamba wosakhala Hodgkin lymphoma (NHL). "Maphunziro apamwamba" amatanthawuza khansa ikukula mofulumira ndikukhala okhwima. Lymphoma ndi khansa ya maselo oyera a m'magazi a lymphocyte.
Malinga ndi kafukufuku wina, pafupifupi 90 peresenti ya kusintha kwa CLL ndi mtundu wa NHL wotchedwa B-cell lymphoma (DLBCL), ndipo pafupifupi 10 peresenti amasintha kupita ku Hodgkin lymphoma.
Izi zimatchedwa "Hodgkin kusiyana ndi Richter syndrome (HvRS)" choncho, ndipo sizikuwonekeratu ngati mankhwalawa ndi osiyana ndi Hodgkin lymphoma. Zosintha zina kuchokera ku CLL ndizotheka.
N'chifukwa chiyani amatchedwa Richter Syndrome?
Mwamuna wina dzina lake Maurice N. Richter anayamba kufotokoza za matendawa mu 1928. Iye analemba za mlangizi wazaka 46 yemwe anatumizidwa ku chipatala ndipo anali ndi njira yopita patsogolo kuti apite. Kufufuza kwa autopsy, iye adatsimikiza kuti pakhala pali vuto linalake lomwe linalipo kale koma kuti kuchokera pamenepo, mliri watsopano unkawoneka utakula ndipo umangowonjezera ndi kuwononga minofu yomwe inali yakale CLL.
Iye ankanena kuti CLL inalipo kwa nthawi yayitali kwambiri kuposa aliyense yemwe amadziwa za wodwala uyu, komanso kulembera za khansa ziwiri, kapena zotupa, kunena kuti, "N'zotheka kuti chitukuko cha chimodzi mwa zilondacho chimadalira kukhalapo kwa wina . "
Zizindikiro
Anthu omwe ali ndi RS amakhala ndi matenda opweteka pogwiritsa ntchito maseŵera olimbitsa thupi, kutambasula kwa nthenda ndi chiwindi, ndi kulemera kwa chizindikiro mu magazi otchedwa serum lactate dehydrogenase, kapena LDH.
Chiwerengero cha Survival
Monga momwe zimakhalira ndi mitsempha yonse, ziwerengero zapulumuka zingakhale zovuta kutanthauzira.
Odwala alionse amasiyana ndi thanzi lawo ndi mphamvu zawo asanadziwone. Kuwonjezera apo, ngakhale khansa ziwiri zofanana zimatha kukhala mosiyana kwambiri ndi anthu osiyanasiyana. Ndili ndi RS, komabe khansara yatsopano imakhala yamphamvu kwambiri. Kwa anthu ena omwe ali ndi RS, kupulumuka kwafotokozedwa ndi chiŵerengero cha chiwerengero cha miyezi yosachepera 10 kuchokera ku matenda. Komabe, kafukufuku wina wasonyeza miyezi 17 kupulumuka, ndipo anthu ena omwe ali ndi RS angakhale moyo wautali; Kutsegula maselo kumapatsa mwayi wopulumuka kwa nthawi yaitali.
Zizindikiro ndi Zizindikiro
Ngati CLL yanu yasintha n'kukhala DLBCL, mudzawona zozizwitsa zanu zoopsa kwambiri.
Makhalidwe a RS amaphatikizapo kukula msanga kwachangu komanso popanda kutulutsa mawonekedwe-kutanthauza kuti, kukula kwatsopano kumangokhala ku maselo a mitsempha, kapena khansa ikhoza kukhala ndi ziwalo zina osati zamaliseche, monga nthenda ndi chiwindi.
Mutha kuwona:
- Kukulitsa maselo amphamvu kwambiri
- Kusokonezeka kwa m'mimba mofanana ndi nthenda yothamanga ndi chiwindi chotchedwa hepatosplenomegaly
- Zizindikiro za kuchepa kwa maselo ofiira a m'magazi (kuchepa kwa magazi), monga kutopa kwambiri, khungu lotumbululuka, kupuma pang'ono
- Zizindikiro za kuchuluka kwa mbale ya thonje (thrombocytopenia), monga kuphwanyidwa kosavuta ndi kusadziwika magazi
- Zizindikiro za kugwirizanitsa kwina kuphatikizapo malo osayenera, monga ubongo, khungu, m'mimba, khungu, ndi mapapo
Zinthu Zowopsa kwa Kusintha
Kuopsa kokhala ndi RS kuchokera ku CLL sikugwirizana ndi siteji ya khansa ya m'magazi, kwa nthawi yayitali bwanji, kapena mtundu wa machiritso omwe mwalandira. Ndipotu, asayansi sadziwa bwinobwino chimene chimayambitsa kusintha.
Posachedwapa, kafukufuku wina wasonyeza kuti odwala omwe maselo a CLL amasonyeza chizindikiro china chotchedwa ZAP-70 akhoza kukhala ndi chiopsezo chowonjezeka. Zolemba zina monga odwala omwe ali ndi CHINCH1 kusintha kwadzidzidzi atakhala ndi chidwi chofufuza. Komabe, kafukufuku wina wasonyeza kuti odwala CLL aang'ono-omwe ali osakwana zaka 55-akhoza kukhala ndi chiopsezo chowonjezeka.
Nthano ina ndi yakuti nthawi yayitali ndi machitidwe a chitetezo cha mthupi kuchokera ku CLL omwe amachititsa kusintha. M'magulu ena odwala omwe adachepetsanso ntchito yoteteza mthupi kwa nthawi yayitali, monga kachilombo ka HIV kapenanso anthu omwe ali ndi ziwalo za thupi, palinso chiopsezo chowonjezeka cha kukhala ndi NHL.
Kaya zili zotani, sizikuwoneka kuti pali chilichonse chimene mungachite kuti musinthe kapena kuteteza CLL yanu kusintha.
Kuchiza ndi Kupatsirana
Chithandizo cha RS chimaphatikizapo zizindikiro za chemotherapy zomwe zimagwiritsidwa ntchito kwambiri ku NHL. Ma regimens awa akhala akutulutsa pafupifupi pafupifupi 30 peresenti. Mwamwayi, ambiri amakhala ndi mankhwala omwe amachititsa kuti miyezi isanu ndi umodzi mutembenuzidwe. Zochiritso zatsopano ndi zowonjezera zimayesedwa nthawi zonse m'mayesero a kliniki, komabe.
Zaka zaposachedwapa, kafukufuku adafufuzira kugwiritsa ntchito machitidwe a Fludarabine chemotherapy monga adasonyezedwa kuti apangitse zotsatira kwa odwala omwe ali ovuta CLL . Kawirikawiri kupulumuka ndi mtundu uwu wa chemotherapy kunakwera kufika pa miyezi 17 mu phunziro limodzi.
Chinanso chomwe chikuchitika ndi kugwiritsa ntchito yotchedwa'atatumab - antibody's anti-CD20 monoclonal antibody yomwe imalumikiza chizindikiro chapadera pa ma lymphocyte B. Phunziro la CHOP -O likuyesa chitetezo, kuthekera, ndi ntchito ya chemotherapy ya CHOP kuphatikizapo toatumumab mu kupititsa patsogolo ndikukonzekera kwa odwala omwe ali ndi RS. Pofufuza mwachidule, oposa asanu ndi awiri mwa anthu 25 oyambirirawo adalandira yankho lathunthu kapena laling'ono patatha zaka zisanu ndi chimodzi za CHOP-O.
Kafukufuku wina wazing'ono akuyang'ana kugwiritsidwa ntchito kwa kusamba kwa tsinde kuti athetse anthuwa. Ambiri mwa odwala mu maphunzirowa adalandira zambiri zam'mbuyo. Mwa mitundu ya maselo omwe anayesedwa omwe anali kuyesedwa, kusamba kosakhala myeloablative kunali kochepa poizoni, engraftment yabwino, ndi kuthekera kukhululukidwa . Maphunziro ena adzafunikanso kuti aone ngati izi ndi njira yabwino kwa odwala a RS.
Kafukufuku Wotsatira
Pofuna kupititsa patsogolo odwala omwe ali ndi RS, asayansi amafunika kumvetsetsa bwino zomwe zimachititsa kusintha kwa CLL kuchitika. Ndikumvetsetsa zambiri za RS pa mlingo wamagulu, mankhwala othandizira abwino angapangidwe motsutsana ndi zovutazo. Komabe, akatswiri akuchenjeza kuti popeza pali kusintha kochepa kwa maselo okhudzana ndi RS, sipangakhale konse "mankhwala" omwe akuwongolera komanso kuti mankhwala ena onsewa amafunika kuphatikizidwa ndi mankhwala omwe amachititsa kuti apeze mankhwalawa. zotsatira zabwino. Monga asayansi akufotokozera zomwe zimayambitsa RS, akuwona kuti RS si yunifomu imodzi yokha kapena ndondomeko yosagwirizana.
Padakali pano, odwala omwe athandiza CLL kusintha kuti afike ku RS amalimbikitsidwa kuti alowe mu maphunziro azachipatala pofuna kuyesetsa kupanga njira zothandizira chithandizo ndi zotsatirapo kuchokera pakali pano.
Zotsatira:
Richter, M. Generalized reticular cell sarcoma ya ma lymph nodes ogwidwa ndi lymphatic khansa ya m'magazi . The American Journal of Pathology. 1928; 4; 4. 285-292.
Zatsopano Zatsopano mu Richter Syndrome. Eyre TA, Clifford R, Roberts C, et al. Dzanja limodzi la NCRI gawo lachiwiri lofufuza za CHOP kuphatikizapo Ofatumumab mu kudulidwa ndi kusamalira kwa odwala omwe ali ndi matenda a Richter atsopano. BMC Khansa . 2015; 15: 52.
> Parikh SA, Habermann TM, Cha ee KG, et al. Kusintha kwa Hodgkin kwa matenda aakulu a khansa ya m'magazi: Kuchuluka, zotsatira, komanso kuyerekezera kwa novo Hodgkin lymphoma. Am J Hematol. 2015; 90: 334-38.
Matenda a Rossi D, Gaidano G. Richter. Adv Exp Med Biol . 2013; 792: 173-91.