Kwanin cutar sankarar jini na yau da kullum, ko CMML, shine ciwon daji na jikin jini wanda ke zaune a cikin kututture. A cikin CMML, ƙwayoyin mahaukaci a cikin kwayoyin jini suna shafar ƙwayar kasusuwa - suna sa mutum yayi da yawa monocytes-irin nau'in jini na jini-kuma wannan yana shafar wasu sassan jikin.
CMML vs. CML
CMML yana tsaye ne ga myelomonocytic cutar sankarar bargo, yayin da CML na tsaye ga myeloid cutar sankarar bargo (kuma ake kira na kullum myelogenous cutar sankarar bargo).
Akwai kamance tsakanin CML da CMML dangane da binciken farko, amma waɗannan cututtuka biyu sun bambanta.
Daga cikin guda biyu, CMML kullum tana ɗauke da maganin da ya fi kyau fiye da CML, ko da yake yawancin dalilai daban-daban na iya taimakawa wajen kwatantawar mutum da kuma rayuwa kuma akwai yiwuwar maganin warkarwa wanda ya dace dangane da kowane mutum.
Dalili da Dalili Dama
CMML yana faruwa ne a cikin tsofaffi, yawancin mutane waɗanda suka kai shekaru 65.
A mafi yawan lokuta na CMML, hanyar ba ta sani ba, kuma babu wata hanyar da za ta hana shi. Wasu mutane suna inganta CMML bayan sun karbi chemotherapy da radiation a matsayin ɓangare na maganin ciwon daji. A wasu lokuta, ana iya yin ƙoƙari don kauce wa magungunan ƙwayoyin cutar shan magani wanda zai iya haifar da CMML, amma ana iya buƙatar waɗannan kwayoyi, maganin warkar da rai a wasu lokuta.
Tsarin jima'i
Ba daidai ba ne ainihin abin da ke faruwa na CMML. Gaba ɗaya, CMML ba wani ciwon daji ne na kowa ba; Duk da haka, shine mafi yawan nau'in jini a cikin sashinta, MDS / MPN, wanda aka bayyana a ƙasa.
Ayyukan
Cikakken tsari na CMML, irin na sauran cututtuka na jini, ya shafe wasu sauye-sauye a tsawon shekaru, a cikin layi tare da ci gaba da fahimtar ilimin kimiyya mafi kyau na cutar.
A yau, ana gane CMML a matsayin cike da halaye na nau'i na nau'i biyu na cutar jini: myelodysplastic ciwo da kuma myeloproliferative neoplasm .
- Myelodysplastic Syndrome (MDS) wani rukuni ne na cututtuka da ke shafar cutar da jini ta al'ada a cikin kututture. A MDS, ƙwayar kasusuwa tana samar da ƙarancin abu mara kyau, ƙananan jini wanda ake kira ƙwayoyin iska. Wadannan ƙwayoyin ƙwaƙwalwa sun kasa cin nasara sosai kuma basu iya aiki a cikin matsayin da suke da shi na jini.
- Myeloproliferative neoplasm (MPN) tana nufin cututtuka da ƙwayar nama ta sa jini da jini mai yawa, platelets, ko daban-daban nau'in jini.
Mutane tare da CMML suna da kwayoyin halitta masu kamala (dysplastic) a cikin kututtukansu na fata, kuma, don haka, na dogon lokaci, an dauke CMML wani nau'i na ciwon myelodysplastic.
Duk da haka, mutanen da ke tare da CMML suna da nauyin jini na jini guda daya, kuma mahimmin siffar MDS yana da ƙananan jini a cikin jini, don haka CMML ba ta da kyau sosai ga tsarin MDS.
Myelodysplastic / myeloproliferative neoplasms (MDS / MPN)
Ga waɗannan dalilai da sauran dalilai, tsarin kulawa da Lafiya ta Duniya (WHO) ya hada da CMML a cikin wani nau'in dukansu: MDS / MPN, kamar yadda aka nuna a kasa.
CMML yana daga cikin nau'o'in MDS / MPN da dama.
- Kwarar cutar sankarar bargo (CMML)
- Magungunan cutar sankarar myeloid mai yawan gaske (aCML), BCR-ABL1 -
- Kwayar cutar sankarar bargo da yara (JMML)
- MDS / MPN tare da wayoyin zobe da thrombocytosis (MDS / MPN-RS-T)
- MDS / MPN, unclassifiable
CMML shine mafi yawan cutar a wannan rukuni. Yawancin cututtuka da yawa a cikin wannan rukuni sune marasa lafiya mai ciwon zuciya da cutar mukamarin cutar sankarar bargo da kuma mielomonocytic cutar sankarar bargo. Duk waɗannan cututtuka suna samar da jini mai yawa.
A cikin sabuntawar da aka bayar a shekara ta 2016, WHO, wani abu da ake kira anemia mai banƙyama tare da raƙumar zobe mai haɗawa da alamar thrombocytosis (RARS-T) an gabatar da shi a matsayin cikakkiyar karɓa, sabon lokacin bincike, wanda ake kira MDS / MPN tare da gefen ringroblasts da thrombocytosis.
Alamai da cututtuka
Samun da yawa na monocytes a kan tsawon lokaci, yawanci, watanni uku shine alamar ta CMML.
Maganin monocytes shine zargi ga yawancin alamun bayyanar. Ƙarin monocytes zai iya tafiya da kuma sanyawa zuwa gabobi biyu a cikin ciki, hanta, da kuma ƙuƙwalwa, inda zasu iya haifar da fadada da wasu alamun bayyanar.
- Tsarin ƙararraki , ko ɓarna, zai iya haifar da ciwo a cikin hagu na hagu na ciki. Ƙara ƙãra a cikin wannan yanki na iya sa mutane su ji cike da sauri lokacin da suke cin abinci.
- Idan hanta ya kara girma, wanda ake kira hepatomegaly, wannan zai iya haifar da rashin jin daɗi a cikin ciki, a cikin ɓangaren dama na dama.
Ko da yake mutane tare da CMML sunyi yawa da jini guda daya, amma wasu lokuta za'a iya samun raguwa da sauran nau'in launin jan da fararen fata a matsayin wani ɓangare na wannan tsari, kuma waɗannan ƙananan za su iya samar da wasu alamomin CMML.
- Rashin jinin jini, ko anemia, zai iya haifar da gajiya sosai, da kuma rauni, rashin ƙarfi, da kodadde fata.
- Ƙididdigar yawan tsaran jini na jini, ko leukopenia, zai iya haifar da cututtuka mai tsanani ko mai tsanani.
- Rashin gajerun jini, ko thrombocytopenia, zai iya haifar da zub da jini da ƙyama. A cikin mutumin da yake tare da CMML, ana iya lura da shi azaman ƙwaƙwalwar ƙwaƙwalwar ƙwayar jini ko ƙananan jini.
Sauran cututtuka na iya haɗa da asarar rashin nauyi, zazzabi, da hasara na ci.
Alamomi da alamun cututtuka na iya bayar da alamomi, amma basu isa don ganewar asirin CMML ba.
Bincike da Bayani
Sakamakon ganewa na CMML ya ƙunshi nazari na musamman na kwayoyin daga jini da kututtukan kasusuwa. Wannan yana nufin kasusuwan kasusuwan maniyyi yana cikin ɓangaren kimantawa, baya ga ƙarin samo jini da aka samo daga wani nau'i mai amfani da allura.
Bisa ga binciken binciken farko, akwai kuma tsarin kawarwa da ke faruwa, tun da yawancin cututtuka daban-daban na iya haifar da waɗannan bayyanar cututtuka da kuma binciken binciken kwarewa sosai.
2016 Harkokin Cibiyar Harkokin Wajen Kimiyya ta CMML
- Tsarancin (fiye da watanni 3) wuce haddi na monocytes (monocytosis) a cikin jinin jiki fiye da 1000 / microL.
- Bayanan Monocytes fiye da kashi 10 cikin 100 na dukkanin jini mai tsabta (WBC) bambanci.
- Ba saduwa da ka'idodin WHO don sauran cututtuka na jini: BCR-ABL1 tabbataccen cutar cutar sankarar bargo, myelofibrosis na farko, polycythemia vera, ko muhimman thrombocytosis
- Kashi na nau'in bala'in da ba a cikin jini da hauka: kasa da kashi 20 cikin dari na myeloblasts + monoblasts + promonocytes a cikin jini da jini da kasusuwa.
- Canje-canje na dysplastic (siffofi mara kyau da bayyanuwa a ƙarƙashin microscope) a cikin ɗaya ko fiye da iyalai na jini masu tasowa a cikin "gidan iyali myeloid." A cikin iyalin iyalin myeloid, "rassan" sun haɗa da layin salula wanda ke raba da girma don samar da monocytes, macrophages, neutrophils, basophils, eosinophils, kwayoyin jinin jini, kwayoyin dendritic, da megakaryocytes.
Kwayoyin cututtuka tare da binciken da suka dace da CMML sau da yawa dole ne a yi sarauta, wasu lokuta likitoci zasuyi ƙarin gwaje-gwajen da aka ba da shawarar kafin maganin marasa lafiya tare da CMML, tare da aikin farko, tun da sun riga sun samo ɓawon jini da jini don ganewar asali.
Idan wasu canje-canje na halitta ko canje-canje na wasu kwayoyin suna samuwa a cikin mawuyacin kwayoyin (wanda ba a sani ba a cikin mutane tare da monocytosis, amma sun hada da PDGFRA ko PDGFRB, FGFR1, ko PCM1-JAK2) sannan akwai bambancin WHO wanda aka ba, kuma akwai alamun magani tare da wani wakili mai suna imatinib.
CMML Categories
Ƙungiyar WHO ta kara bambanta mutane tare da CMML a cikin nau'o'i daban-daban guda uku da suka danganci alamar bincike:
- CMML-0: Fiye da kashi 2 cikin dari a cikin jinin jini da kuma kasa da kashi 5 cikin dari a cikin karfin.
- CMML-1: 2 zuwa kashi 4 cikin dari na fashewar jini da jini / / 5 zuwa 9 bisa dari a cikin karfin.
- CMML-2: 5 zuwa 19 bisa dari a cikin jini, kashi 10 zuwa 19 bisa dari a cikin karfin, da kuma / ko kasancewar daya ko fiye da sandunan Auer (burbushin launuka masu launin burgundy mai launin launin fata wanda ya samar da siffar elongated, wanda aka gani a cikin cytoplasm myeloid blasts).
Jiyya
Kashi mai laushi wanda aka samo daga mai bayarwa (allogeneic hematopoietic cell transplantation) shine kawai magani mai magani ga marasa lafiya tare da CMML. Lokacin da ya kasance wani zaɓi, wannan yanke shawara ya zama sakamakon sakamakon likita-haƙuri.
Gwaji na Clinical
A dangane da ilimin chemotherapy, ya zuwa yanzu, babu "bulletin sihiri ga CMML" da aka samo. Hanyoyin da ke samuwa ba su da tasiri sosai kan ci gaba da cutar, saboda haka mutane da CMML suna ƙarfafawa don la'akari da shiga cikin gwaji, idan akwai.
Ga mutanen da ba su zuwa dashi ba, kuma ga waɗanda ba su shiga rajistar ba, akwai wasu zaɓuɓɓuka don maganin rashin lafiya, ba tare da maganin warkar da su ba, ciki har da farfadowa da maganin cututtuka.
Gudanar da Ƙaddamarwa da Cibiyar Taimako
Jagoran kwararru sun ce marasa lafiya ba tare da bayyanar cututtuka ya kamata su kula da su ba tare da nazarin lokaci na zamani da kuma nazarin gwaje-gwaje. Sun kuma bayar da shawarar cewa za a sake inganta rigakafin rigakafi kuma idan mai haƙuri ne mai fure, an dakatar da shan taba.
Idan ba a samu gwaji ba, akwai sauran kwayoyi da za'a iya amfani da su don "kayar da" yawan kwayoyin halitta marasa mahimmanci (hanyoyin cytoreductive) kamar hydroxyurea, azacitidine, ko decitabine.
Taimakon tallafi ga marasa lafiya tare da CMML na kama da abin da aka yi wa marasa lafiya tare da MDS. Ana ba da karusar juyyoyin kwayoyin jan kwayoyin jini da kuma platelets, kuma ana iya yin amfani da magungunan erythropoiesis-stimulating agents, da kuma maganin cututtukan kwayoyin cututtuka.
Domin CMML mai rikitarwa, ko lokuta inda aka yi magunguna amma sun kasa, ana ƙarfafa marasa lafiya zuwa shiga gwaji a duk lokacin da suke samuwa.
Faɗakarwa
Babu wani abu mai kyau wanda zai iya rayuwa, saboda mutane da CMML na iya samun irin abubuwan da suka faru tare da cutar.
Mawallafin da aka wallafa (ko, statistically, lambar tsakiya a cikin jerin lokuta na rayuwa) yana da kusan tsawon watanni 30 daga lokacin ganewar asali. Akwai bambanci mai yawa a cikin wannan tsakiyar, duk da haka, da kuma kididdigar rayuwa, a gaba ɗaya, ba a fahimci sababbin jiyya ba wanda babu bayanai a yanzu. Ana nazarin ganewa akan ƙarin bayani a ƙasa, da zarar an kwatanta kamfanoni daban-daban na CMML.
Rashin tabbas akan kididdiga na iya sa ku ji damu. Maganar budewa da likita za ta taimake ka ka fahimci batunka. Akwai hanyoyi masu yawa da sauye-sauye tare da CMML, kuma akwai akalla darussan bambance-bambance tara don kokarin taimakawa wajen ƙayyade tsarin bincike wanda ke amfani da abubuwa kamar siffofin asibiti da binciken binciken, kuma wasu suna nazarin kwayoyin halittar kwayoyin halitta .
Za a iya zama manyan bambance-bambance a hanyar yadda masu shan magani ke kulawa da CMML haɗari, dangane da ma'aikata, amma babu wani bayanan da ya nuna cewa yana nuna hanya ɗaya mafi kyau don tantance hadarin.
Lissafi daga Cibiyar Kankarar Amurka ta yanzu suna da ɗan lokaci, amma suna taimakawa wajen nuna bambancin da sassan CMML-1 da CMML-2, kuma suna nuna yadda wasu kungiyoyi a cikin dukan jama'a suna son su yi fiye da sauran.
A cikin binciken daya daga cikin marasa lafiya CMML da aka bincikar da su tsakanin 1975 da 2005, lokutan tsakiyar rayuwa tare da CMML-1 da CMML-2 sun kasance watanni 20 da 15. Duk da haka, wasu marasa lafiya sun rayu tsawon lokaci. Kimanin kashi 20 cikin dari na marasa lafiya CMML-1 kuma kimanin kashi 10 cikin dari na marasa lafiya CMML-2 sun rayu fiye da shekaru biyar. Har ila yau, marasa lafiya tare da CMML-2 zasu iya ci gaba da ciwon cutar sankarar bargo kamar marasa lafiya tare da CMML-1. A cikin wannan binciken, kashi 18 cikin dari na marasa lafiya CMML-1 da kashi 63 cikin 100 na CMML-2 marasa lafiya sun sami ciwon mikiya mai tsanani a cikin shekaru biyar na CMML ganewar asali.
Kalma Daga
Ga mutumin da yake tare da CMML, ƙwarewar zata iya dogara da dalilai da yawa, kuma shekarun mutum da kuma lafiyar lafiyarsu duk suna da muhimmanci. Saboda wannan cutar tana shafar mutane da yawa waɗanda suka kamu da cututtuka, yawancin magani da kuma abin da ke da magungunan karuwanci - ba koyaushe wani zaɓi ba.
Kyakkyawan matakan tallafi suna samuwa, amma CMML wani cututtuka ne wanda ke alama a gefen wani binciken da ake bukata a warkewa. Kamar yadda irin wannan, abin da aka buga a jiya zai yiwu ko ba gaskiya bane, don haka duba duk zaɓinku kuma ku yi la'akari da shiga cikin gwaji na asibiti.
> Sources:
> Arber DA, Orazi A, Hasserjian R, et al. Rajista ta 2016 zuwa Ƙungiyar Lafiya ta Duniya ta ƙayyade ƙwayoyin cuta da ƙwayar cutar sankarar jini. Jinin jini . 2016; 127: 2391-2405.
> Elena C, Gall A A, Irin wannan E, et al. Haɗaka siffofin asibiti da raunin kwayoyin a cikin hadarin kima na marasa lafiya tare da cutar cutar sankarar myelomonocytic na kullum. Blood 2016, 128 (10): 1408-1417.
> Zeidan AM, Hu X, Long JB, et al. Amfani da hypomethylating farfadowa da rayuwa a cikin marasa lafiya marasa lafiya tare da cutar sankarar myelomonocytic na kullum a Amurka: Babban binciken jama'a. Ciwon daji . 2017 Oktoba 1; 123 (19): 3754-3762.