Anemia mai juyayi tare da fashewa mai tsanani, ko RAEB, yana nufin ɓarna daga cikin kwayoyin jini. RAEB na ɗaya daga cikin nau'o'i bakwai na irin wannan cuta, ko kuma ƙwararrun myelodysplastic (MDS), wanda Masanin Lafiya ta Duniya (WHO) ya gane, wanda ya bambanta tsakanin sassa biyu na RAEB: RAEB-1 da RAEB-2.
Dukansu suna nuna nau'i mai mahimmanci: yanayin rayuwar rayuwa wanda aka wallafa (a yanzu yana da kwanan baya) ya kasance daga watanni 9-16.
Har ila yau, RAEB yana haɗuwa da haɗari mai zurfi na ci gaba ga cutar cutar sankarar myeloid mai zurfi -a ciwon daji na jikin jini wanda ke da karfin jini .
Ganin RAEB, irin MDS
Myelodysplastic syndrome, ko MDS, tana nufin iyali da cututtukan cututtuka na jini wanda ƙashin ƙasusuwan ba ya samar da cikakken jinin jinin jinin, kwayoyin jinin jini ko platelets. RAEB wani nau'in MDS ne wanda ya saba da shi, kuma rashin alheri, shi ne babban haɗarin MDS.
Kamar sauran nau'o'in MDS, RAEB yana shafar mutane da yawa a cikin shekaru 50, amma zai iya faruwa a kananan yara, kuma a halin yanzu ba a sani ba.
Lokacin da mutum yana da nau'i na MDS irin su RAEB, kasusuwa na kasuwa zai iya haifar da ƙananan waɗanda ba su da tushe, ko maras tabbas, kwayoyin da sukan saba da siffofi, girma ko bayyanuwa, idan aka kwatanta da masu lafiya. Wadannan farkon, ƙananan yara, nau'i na jini suna kira ƙwayoyin ƙuƙwalwa - kalmar da aka yi amfani dasu sosai sau da yawa a cikin tattaunawa game da cutar sankarar bargo.
Hakika, a yau masana kimiyya da yawa suna ganin MDS a matsayin jini da ƙwayar ciwon daji.
An yi amfani da tsarin rarrabe daban-daban don waɗannan cututtuka. Tsarin tsarin tsarawa na WHO ya yi ƙoƙarin warware irin nauyin MDS, tare da kulawa da ƙaddamarwa ga rashin lafiya. WHO a halin yanzu tana gane nau'o'in MDS guda 7, tare da asusun RAEB-1 da RAEB-2 na kimanin kashi 35-40 na dukkan lokuta na MDS.
- Cytopenia mai juyayi tare da dysplasia na unilineage (RCUD)
- Anemia mai juyayi tare da ƙwayar zuciya ta tsakiya (RARS)
- Cytopenia mai juyayi tare da dysplasia multilineage (RCMD)
- Anemia mai juyayi tare da haɗari-1 (RAEB-1)
- Anemia mai banƙyama tare da ƙananan iska-2 (RAEB-2)
- Magungun ƙwayar ƙwayar cuta, wanda ba a ƙaddara ba (MDS-U)
- Abun ciwon myelodysplastic da ke haɗuwa da del (5q)
Wadannan sunayen sunaye sune sau da yawa game da yadda jini da kasusuwan ɓawon jini suka bayyana, lokacin da aka bincika su a karkashin microscope. Sunan karshe a cikin jerin da aka sama, duk da haka, an bayyana ta wani maye gurbin, ko canzawar chromosome, a cikin kwayoyin halitta na ɓawon jini na kasusuwan jini.
A cikin yanayin RAEB (duka nau'o'i), sunan yana da sassa biyu: anemia mai rikitarwa; da kuma hare-hare. Abun cutar, a gaba ɗaya, rashin rashin jinin jini ne. Maimakon ƙin jini yana nufin cewa anemia ba saboda wani sanadiyar sanadin cutar anemia ba kuma anyi amfani da cutar anemia kawai tare da karfin jini. Lokacin da mutum yana da mummunan anemia da gwaje-gwaje ya nuna mafi yawan adadin ƙwayoyin ƙwayar cuta fiye da yadda al'ada yake, yana da mummunan anemia tare da fashewa.
Zai yiwu mutum wanda ke da RAEB yana da ƙananan ƙididdigewa a cikin wasu kwayoyin halittar da ta samu daga kasusuwa.
Mutanen da ke da RAEB na iya samun mummunan anemia (ƙananan jinin jini), kwayoyin neutropenia mai rikitarwa (ƙananan neutrophils), mai ɓarya na thrombocytopenia (lowletslets), ko hade da uku.
RAEB Tsarin MDS ne mai girma
Ga marasa lafiya da aka gano tare da MDS, yana da muhimmanci a ƙayyade ƙimar hadarin. Wasu nau'i na MDS suna da haɗari, wasu ƙananan haɗari, wasu kuma haɗari. Dukkan RAEB da RCMD suna dauke da nau'i na MDS. Duk da haka, ba dukkan marasa lafiya da RAEB suna da irin wannan bayanin ba. Wasu dalilai sun shiga wasanni, kamar shekaru, lafiyar jiki, siffofin cututtukan, da kuma kwayoyin jikinsu na kwayoyin halitta.
Sanin asali
Lokacin da aka yi la'akari da MDS, za a yi amfani da kasusuwan maniyyi da kaso. Wannan ya shafi samo samfurori na kasusuwa na kasusuwa kuma aika su zuwa dakin gwaje-gwaje don bincike da fassarar.
An gane ganewar asali akan yadda kwayoyin suna fitowa a ƙarƙashin microscope, yadda zasu zama masu kama da launuka da alamomi da suka hada da amfani da kwayoyin cutar kamar tags, kuma, a yanayin saurin ƙaddara na MDS, wani abu da ake kira cytometry mai gudana . Gudun ruwan tafiye-tafiye ne wata hanya da ta ba da damar sel tare da wasu halaye don ganowa kuma an ware su daga yawan yawan yawan kwayoyin halitta a cikin samfurin da aka ba su.
Iri
Dukansu siffofin (1 da 2) na RAEB suna haɗuwa da haɗarin ci gaban cigaba da cutar cutar sankarar myeloid (AML). Bugu da ƙari, mai haƙuri wanda ke da ƙananan MDS irin su RAEB zai iya jurewa cin hanci da rashawa, ba tare da ci gaba ga AML ba, saboda haka yanayin yana sau da yawa barazanar rayuwa, ba tare da cigaba da cutar sankarar bargo ba.
RAEB-Related Terminology
Ra'ayin RAEB ya dogara ne da fahimtar wasu kalmomi:
- Ƙarƙashin ƙwayar murmushi : An samo samfurin kabar nama, kuma yawan nau'in mahaukaci, ƙananan ƙwayar ƙwayoyin suna auna.
- Rashin jinin jinin jini: An samo samfurin jinin jinin daga gindin mai amfani da allura, kuma yawan nau'in mahaukaci, ƙananan ƙwayar ƙwayoyin suna auna.
- Ƙarƙwarar ƙira : Wannan wani abu ne likitoci zasu nema idan sun ga fitowarka a karkashin microscope. Duk da cewa an kira su Auer "sanduna," sun zo ne da yawa da siffofi daban-daban. Su ne kananan-karami fiye da tsakiya, kuma ana samun su a cikin cytoplasm. Sau da yawa suna da siffar allurar ƙira mai ma'ana, amma suna iya zama nau'i-nau'i, mai siffar lu'u-lu'u, ko kuma mai tsawo.
Bisa ga kasancewar ko babu abubuwan da aka sama, an ƙaddara mutum don samun ko RAEB-1 ko RAEB-2 kamar haka:
Ana bincikar marasa lafiya tare da RAEB-1 idan suna da ko dai (1) ƙaddarar kashi kashi tsakanin kashi 5 zuwa 9 na akalla 500 kwayoyin kididdiga ko (2) ƙididdigar ƙirar jini tsakanin 2 da 4 bisa dari na akalla ɗari 200 aka ƙidaya, da kuma (3) babu sanduna Auer. Maganin ko wane sashe 1 ko 2 da 3 sun ware wani rahoton MDS kamar RAEB-1.
Ana iya kimanta chances na RAEB-1 zuwa mai cutar cutar sankarar myeloid mai zurfi a kimanin kashi 25.
Ana bincikar marasa lafiya tare da RAEB-2 idan suna da ko dai (1) ƙididdigar karfin kashi kashi 10 zuwa 19 bisa dari na akalla 500 kwayoyin kididdiga ko (2) ƙididdigar jini tsakanin 5 zuwa 19 bisa dari na akalla 200 kwayoyin kidaya, ko (3) Ana iya gano sandunan Auer. Gabatarwar kowane sha'ani na 1, 2 ko 3 ya ware wani rahoton MDS kamar RAEB-2.
Ana kiyasta cewa chances na RAEB-2 zuwa mai cutar cutar sankarar myeloid mai tsanani zai iya zama kamar yadda kashi 33 zuwa 50 cikin dari.
Menene RAEB-T?
Hakanan zaka iya haɗu da kalmar "mummuna mai rikitarwa tare da tashin hankali a cikin canji," ko RAEB-T. An riga an watsar da wannan kalmar a halin yanzu na WHO na ƙwayoyin cuta na myelodysplastic.
Yawancin marasa lafiya da suka kasance a cikin wannan rukunin yanzu an classified suna da ciwon cutar sankarar myeloid mai tsanani. A cikin tsarin daban-daban, Faransanci-Amurka-Birtaniya (ƙungiyar FAB), an sanya marasa lafiya zuwa ga RAEB-T idan suna da ko dai (1) ƙaddara mai karfin kashi kashi tsakanin 20 zuwa 30 bisa dari, (2) ƙididdigar ƙirar ƙirar aƙalla akalla kashi 5, ko (3), Ƙarƙashin magunguna, wanda ba tare da la'akari da ƙididdiga ba.
Akwai ci gaba da yin gardama game da muhimmancin rarraba RAEB-T a cikin tsarin FAB, dabam daga "AML-20-30," kamar yadda a tsarin WHO. Da yawa daga cikin gwaje-gwajen gwaji a cikin 'yan shekarun nan sun yi amfani da kalmar RAEB-T, duk da sauye-sauye a cikin tsarin ƙididdigar WHO. Ƙasar ga marasa lafiya da masu kula da kiwon lafiya suna da alama cewa yana da muhimmanci a san cewa akwai kalmomi masu mahimmanci, don haka kada ku rasa damar yin rajista a gwaji.
Ta yaya ake kula da RAEB?
Kula da RAEB ya bambanta da yanayin da ya bambanta. Yau da lafiyar lafiyar mutum zai iya shiga cikin irin wannan yanke shawara. Magunguna da RAEB zasu karbi karin bayanai game da maganin rigakafi, kuma masu shan taba da RAEB suna karfafa su dakatar da shan taba. Alamar da RAEB ke iya cigaba da ci gaba sun hada da ciwo da yawa, da zubar da jini, da haushi, da kuma bukatar ƙarin saurin jini.
Ba duk marasa lafiya tare da MDS na buƙatar gaggawa ba, amma marasa lafiya da alamun ƙananan ƙwayoyin cuta (anemia, thrombocytopenia, neutropenia tare da ciwo mai maimaita), kuma wannan ya haɗa da mafi yawan marasa lafiya tare da MDS mai girma ko babbar mawuyacin hali (ciki har da RAEB-2, wanda ya wakilta mafi girma SAS na MDS tare da mafi girman talauci).
Gudanar da Sharuɗɗa na Ƙungiyar Ciwon Kankara ta Kasa (NCCN) ta ƙunshi cikakken lafiyar mutum da kuma aikinsa, Ƙarƙashin Ƙarƙashin Ƙarƙashin Ƙarƙashin Ƙasa (IPSS) da kuma asusun na IPSS (IPSS-R) MDS, da sauran cututtukan cututtuka don taimakawa wajen jagorancin yanke shawara. Babu wata hanya daya da ta dace da kulawa ga mutane tare da RAEB.
Akwai hanyoyi uku na jiyya : kulawa da goyan baya, hanyoyin kwantar da hanzari, da kuma hanyoyin tsabtace jiki. Wadannan jiyya suna bayyana a kasa:
- Taimakon tallafi yana hada da maganin cututtuka don cututtuka da kuma jan kwayoyin jini da karfin jini don nuna alamun ƙananan ƙidaya.
- Harkokin ƙananan ƙwayoyin cuta sun haɗa da wasu kwayoyin halittar jini, wasu magunguna irin su azacitidine da decitabine, maganin immunosuppressive, da ƙananan ilimin chemotherapy. Wadannan jiyya za a iya tsĩrar da su a kan asibiti kuma za su iya inganta bayyanar cututtuka da ingancin rayuwa, amma ba su warke yanayin ba.
- Harkokin gwagwarmaya masu tsanani sun hada da haɗuwa da haɓaka da kuma allogeneic kashi mai laushi. Wadannan hanyoyin kwantar da hankali suna buƙatar samun asibiti da kuma haɗari da haɗarin rayuka, amma su ma zasu iya inganta yawan jinin jini fiye da rashin lafiyar marasa lafiya kuma zai iya canza hanyar da yanayin zai ci gaba. Kawai wasu mutane ne masu neman ƙwayar cutar.
Gwajen gwajin gwaji kuma wani zaɓi ne ga wasu marasa lafiya. Ba da daɗewa ba, a gaskiya ma, akwai gwajin gwaji na gwaji da ƙaddarawa, idan aka kwatanta da kulawa mafi kyau, a cikin marasa lafiya da ke dauke da cutar anemia tare da haɗari da yawa a canji (RAEBt).
Kalma Daga
Idan an gano ku tare da RAEB-1, RAEB-2, ko kana da wani nau'i na MDS wanda za'a yi la'akari da haɗari, magana da ƙungiyar kiwon lafiyarku game da zaɓuɓɓuka.
Ga marasa lafiya da MDS mafi girma, azacitidine (5-AZA, Vidaza) da decitabine (Dacogen) su ne kwayoyi guda biyu da FDA ta amince don MDS cewa mai kula da kulawa zai iya la'akari. Wadannan kwayoyi sune ake kira hypomethylating jamiái.
Kungiyoyin kungiyoyi da dama sun nuna cewa, saboda MDS mafi girma, hawan HSCT (ƙuƙwalwar karfin jini) ko farfesa tare da wakilai mai tsabta ya kamata a fara su nan da nan. Allogeneic HSCT (ƙuƙwalwar karɓin jini ne daga mai bayarwa) shine kawai tsarin kulawa da ƙwayar cuta ga MDS, amma, da rashin alheri, wannan zaɓi ne na ainihi ga marasa lafiya marasa lafiya, saboda yawan shekarun da aka samu na MDS, tare da hadin gwiwar lafiya yanayi da wasu dalilai masu haƙuri.
> Sources:
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> Germing U, Cutar C, Kujallar A, et al. Anemia mai juyayi tare da haɗari na hargitsi (RAEB): nazari na ƙaddarawa bisa ga shawarar WHO. Br J Haematol. 2006; 132 (2): 162-7.
> Holkova B, Supko JG, Ames MM, et al. Wani lokaci na gwaji na vorinostat da alvocidib a marasa lafiya tare da sake komawa baya, mai rikitarwa ko rashin lafiya maras lafiyar cutar cutar sankarar bargo, ko mummunar cutar anemia tare da haɗari-2. Ciwon Cutar Cancer. 2013; 19 (7): 1873-1883.
> Jiang Y, Dunbar A, Gondek LP, et al. Halittar DNA methylation shine mahimmin tsari a MDS ci gaba zuwa AML. Jinin jini . 2009; 113 (6): 1315-1325.
> Manyan L, Burgstaller S, Stauder R, et al. Labaran Azacitidine a cikin marasa lafiya 339 tare da cututtukan myelodysplastic da mikiloid cutar sankarar bargo: kwatanta na Faransanci-Amurka-Birtaniya da Ƙungiyar Lafiya ta Duniya. J Hematol Oncol. 2016; 9: 39.