Rashin lafiya na Richter (RS), wanda aka fi sani da gyaran Richter, yana nufin sauyawa na musamman na jini na jini a cikin daban-daban, mafi mawuyacin hali.
RS na nufin ci gaba da lymphoma wanda ba Hodgkin ba a cikin mutumin da ke dauke da cutar kankarar lymphocytic na kullum (CLL) / ƙananan lymphocytic lymphoma (SLL) . Sauran nau'ikan RS ɗin ma an san su faruwa, kamar su canji zuwa lymphoma Hodgkin.
Bayanin waɗannan sharuddan da muhimmancin su.
Bayani
RS yayi tasowa a cikin wanda ya rigaya yana da ciwon daji na jikin jini. Wannan ciwon daji na farko yana da sunaye guda biyu, dangane da inda aka gano ciwon daji: An kira CLL idan yawancin ciwon daji ke samuwa cikin jini da kasusuwan jini ko SLL idan aka samu mafi yawa a cikin ƙwayoyin lymph .
An yi amfani da CLL don rufe dukkanin ƙungiyoyi, ci gaba a cikin wannan labarin.
Ba Kowane mutumin da yake tare da CLL ya tasowa Ciwo mai tsanani
Gyara RS a cikin mutanen da ke tare da CLL ba su da sananne. Rahoton da aka buga a shekara ta 2016 shine cewa canjin Richter na faruwa ne a kusan kashi 5 cikin 100 na marasa lafiya tare da CLL. Sauran hanyoyin suna nuna bambanci tsakanin 2 da 10 bisa dari. Idan RS ya faru da ku, yana da banbanci cewa zai faru a lokaci guda CLL an gano shi. Mutanen da suka inganta RS daga CLL suna yin haka shekaru da yawa bayan ganewar CLL.
Sabuwar Ciwon Ƙwayar tana da ƙwaƙwalwa
Sabuwar ciwon daji ke faruwa a yayin da mutumin da yake tare da CLL ya ci gaba da inganta abin da aka sani a matsayin canji, mafi sau da yawa zuwa lymphoma non-Hodgkin (NHL) mai girma. "Matsayi mai mahimmanci" na nufin ciwon daji yana ci gaba da girma da sauri kuma ya zama mafi muni. Lymphoma ciwon ciwon daji ne na ƙwayoyin jini na lymphocyte.
Bisa ga binciken daya, kashi 90 cikin dari na canje-canje daga CLL sune irin nau'in NHL da ake kira lymphoma mai girma B-cell (DLBCL), yayin da kimanin kashi 10 ke canza zuwa lymphoma na Hodgkin.
An zahiri shi ne "Hodgkin variant na rashin lafiya na Richter (HvRS)" a wannan yanayin, kuma ba a bayyana ko bayyanar ba ta bambanta da lymphoma Hodgkin. Sauran canje-canjen daga CLL suna yiwuwa.
Me yasa ake kira Hadisin Richter?
Wani mutum mai suna Maurice N. Richter ya fara bayyana rashin lafiya a shekarar 1928. Ya rubuta game da malamin mai shekaru 46 wanda aka shigar da shi a asibitin kuma ya ci gaba da tafiya a hankali. A cikin bincike na autopsy, ya ƙaddara cewa akwai wani ɓarna a cikin kwanan nan wanda ba a ciki ba, amma daga ciki, wani ɓangaren rashin lafiya ya yi kama da cewa yana da karuwa wanda ya ci gaba da sauri kuma ya lalata da abin da tsohon tsohon CLL ya rushe.
Ya san cewa CLL ya wanzu fiye da kowa ya san game da wannan cuta, kuma ya rubuta game da ciwon daji guda biyu, ko raunuka, yana cewa, "Yana yiwuwa yiwuwar ci gaban daya daga cikin raunuka ya dogara ne akan wanzuwar ɗayan . "
Halaye
Mutanen da ke tare da RS suna ci gaba da ciwo mai tsanani tare da hanzari ƙara yawan ƙwayoyin lymph, kara girma da ƙwaƙwalwa da hanta, da kuma ƙananan matakan alamar alamar jini da aka sani da lactate dehydrogenase, ko LDH.
Taruwar Survival
Kamar yadda dukkanin lymphomas, yawan kididdigar rayuwa zasu iya wuyar fassara.
Kwararrun mutane daban-daban sun bambanta a lafiyar su da kuma karfi kafin su gane asali. Bugu da ƙari, ko da magunguna biyu da wannan zai iya nuna bambanci sosai a cikin mutane daban-daban. Amma tare da RS, duk da haka, sabon ciwon daji ya fi tsanani. A wasu mutane tare da RS, an bayar da rahoton rayuwa tare da matsakaicin adadi na kimanin watanni 10 daga ganewar asali. Duk da haka, wasu nazarin sun nuna rayayyen rayuwa na tsawon watanni 17, kuma wasu mutanen da ke tare da RS zasu iya rayuwa; Tsarin suturar kwayar halitta zai iya ba da dama don rayuwa mai tsawo.
Alamai da cututtuka
Idan CLL ya canza zuwa DLBCL, za ka lura da yadda ake nuna damuwa game da bayyanar cututtuka.
Ayyukan RS sun hada da ci gaba da ciwon ciwon tumowa tare da ko ba tare da shigarwar extranodal ba-wato, sababbin ci gaba zasu iya kasancewa a cikin ƙwayoyin lymph, ko kuma ciwon daji zai iya haɗawa da wasu kwayoyin da suka hada da lymph nodes, irin su yatsun da hanta.
Kuna iya samun:
- Hanyar kara girma cikin ƙananan hanyoyi
- Raunin rashin tausayi na ciki wanda ya danganci kararraki da hanta da ake kira hepatosplenomegaly
- Hanyoyin cututtuka na ƙananan jinin jini (anemia), kamar jijiyar gajiya, kodadde fata, rashin ƙarfi na numfashi
- Kwayoyin cututtuka na ƙananan platelet (thrombocytopenia), kamar sauƙaƙa da sauƙi da rashin jini
- Alamar alamar extranodal ciki har da wasu shafukan yanar gizo, irin su kwakwalwa, fata, tsarin gastrointestinal, fata, da huhu
Abubuwan Dama don Canji
Rashin haɓaka RS daga CLL ba shi da alaka da mataki na cutar sankarar bargo, tsawon lokacin da ka samu, ko kuma irin amsa ga farfado da ka samu. A gaskiya ma, masana kimiyya ba su fahimci abin da ke haifar da canji.
Kwanan nan, wasu binciken sun bayyana cewa marasa lafiya wadanda kwayoyin CLL sun nuna wani alamar alama mai suna ZAP-70 na iya samun ƙarin haɗarin canji. Sauran alamomi kamar marasa lafiya wadanda ke da maye gurbin NOTCH1 a ganewar asali sun kasance daga neman bincike. Duk da haka, wasu nazarin sun nuna cewa marasa lafiya CLL marasa lafiya - wato, ƙananan yara fiye da 55 - na iya samun haɗari mai yawa.
Wani ka'ida shine cewa lokaci ne mai tsawo tare da tsarin maye gurbin daga CLL wanda ke haifar da canji. A wasu nau'ikan marasa lafiya wadanda suka rage yawan aikin da ba su da kariya, kamar su cutar kwayar cutar mutum (HIV) ko kuma a cikin mutanen da suka yi suturar kwayoyin halitta, akwai kuma haɓaka ƙari na bunkasa NHL.
Duk abin da ya faru, ba ya bayyana cewa akwai wani abu da za ka iya yi don hana ko hana CLL daga canzawa.
Jiyya da Fahimci
Yin magani na RS yakan haɗa da ladabi na chemotherapy da aka saba amfani dashi ga NHL. Wadannan tsarin sun haifar da yawan yawan masu amsawa game da kashi 30 cikin dari. Abin takaici, yawancin rayuwa tare da chemotherapy na yau da kullum ba shi da kasa da watanni shida bayan gyaran RS. Sabbin hanyoyin kwantar da hankali da kuma haɗuwa suna ci gaba da gwadawa a gwaje-gwaje na asibiti, duk da haka.
A cikin 'yan shekarun nan, nazarin sun bincika yin amfani da ladabi na Fludarabine chemotherapy yayin da aka nuna su don inganta sakamakon a marasa lafiya tare da CLL mai wahala . Yawancin rayuwa da irin wannan chemotherapy an ƙara zuwa watanni 17 a binciken daya.
Wani abu kuma wanda ke faruwa shi ne amfani da theatumumab - wani ɗan adam anti-CD20 monoclonal antibody wanda ke sa ido a kan mahimmanci a kan ƙwayoyin B. Nazarin binciken na CHOP -O yana kimanta lafiyar, iyawa, da kuma aikin da cutar CHOP ta haɗu tare da inatumumab a cikin shigarwa da kuma kulawa na ƙarshe ga marasa lafiya da sababbin RS. A cikin bincike na tsawon lokaci, fiye da 7 daga cikin masu halartar farko 25 sun cimma cikakkiyar amsawa ko bangare bayan an baje kolin CHOP-O.
Wasu ƙananan binciken sun dubi amfani da tsire-tsire na suturar sel don bi da wannan yawan. Yawancin marasa lafiya a cikin wannan binciken sun karu da yawa kafin cutar chemotherapy. Daga irin nau'in sassan kwayoyin da aka gwada, wanda ba shi da myeloablative dashi yana da ƙasa da guba, mafi kyawun rubutu, da yiwuwar gyarawa . Za a buƙaci ƙarin nazarin don ganin idan wannan zaɓin mai yiwuwa ne ga marasa lafiya na RS.
Bincike na gaba
Domin inganta rayuwar mutane marasa lafiya tare da RS, masana kimiyya na bukatar fahimtar abin da ke haifar da canji daga CLL. Tare da ƙarin bayani game da RS a matakin ƙwayar salula, ƙwarewar da aka fi dacewa za a iya bunkasa akan waɗannan ƙananan hauka. Duk da haka, masanan sunyi tsammanin cewa tun da akwai wasu canje-canje masu rikitarwa masu rikitarwa da suka haɗa da RS, bazai taba zama "manufa ɗaya" wanda ake nufi da magani ba kuma cewa kowane daga cikin waɗannan magunguna zai iya buƙatar haɗuwa tare da chemotherapy na yau da kullum don samun sakamako mafi kyau. Yayinda masana kimiyya suka bayyana asirin RS, suna ganin cewa RS ba daidai ba ce ko tsari.
A halin yanzu, ana kwantar da marasa lafiya da suka yi CLL zuwa RS don su shiga cikin binciken binciken asibiti a kokarin da za su inganta zaɓuɓɓukan magani da kuma sakamakon daga halin yanzu.
Sources:
Richter, M. Generalized cell cellular sarcoma na ƙwayoyin lymph da ke hade da cutar sankarar jini lymphatic . The American Journal of Pathology. 1928; 4; 4. 285-292.
Sabbin Sabbin Kwayoyin Cikin Ciwo mai Cin Hanci. Eyre TA, Clifford R, Roberts C, et al. Neman hannu na NCRI na biyu na binciken CHOP a hade tare da Ofatumumab a cikin shigarwa da kuma kulawa ga marasa lafiya da cutar lafiya ta Richter. BMC Kankara . 2015; 15: 52.
> Parikh SA, Habermann TM, Cha ee KG, et al. Hodgkin canza na kullum lymphocytic cutar sankarar bargo: Dalili, sakamakon, da kuma kwatanta zuwa novo Hodgkin lymphoma. Am J Hematol. 2015; 90: 334-38.
Rossi D, Gaidano G. Richter ciwo. Adv Exp Med Medl . 2013; 792: 173-91.