I-Acute Myeloid Leukemia

Uhlobo Lwegazi Lomdlavuza Ubonakala Kubantu Abadala

I-myeloid leukemia (AML) enamandla kakhulu uhlobo lomdlavuza oqala ekuqaleni emnothweni wethambo lapho ukhiqizwa amangqamuzana egazi khona bese ushukumisela masinyane kumaseli egazi ngokwawo. Ukusuka lapho, umdlavuza ungasakazeka kwezinye izingxenye zomzimba kuhlanganise nesibindi, uhlangothi, isikhumba, ubuchopho, nomgogodla.

I-AML ithinta abantu abangaba yizigidi ngonyaka futhi iholela ekufeni kwabantu abangaba ngu-150 000.

E-United States kuphela, kutholakala amacala angama-10 000 no-18 000 ngonyaka.

Ngokungafani nezinye izinhlobo ze-leukemia , ezivame ukushaya abasha, i-AML ngokuvamile ihlasela abantu abadala abangaphezu kuka-65. Phakathi kwaleli qembu lobudala, isilinganiso seminyaka emihlanu sokusinda sibuhlwempu, sibheke cishe ngamaphesenti amahlanu kuphela. Amazinga okuguqulwa phakathi kwabantu abadala asebekhulile athambekele kangcono nanoma yikuphi ukusuka kuma-25% ukuya kuma-70% ekufezeni ukukhululwa okuphelele ngemuva kwe-chemotherapy.

Izinkinga zezifo

I-leukemia iyinhlangano ehlukene yezincane ezithinta ama-tissue amabili okwenza igazi kanye namaseli egazi ngokwabo. Nakuba lesi sifo sithinta kakhulu amangqamuzana egazi omhlophe , ezinye izinhlobo zezifo ezihlasela ezinye izinhlobo ze-cell.

Endabeni ye-AML, igama elithi "acute" lisetshenziselwa ngoba umdlavuza uqhubeka ngokushesha, kanti "imyeloid" ibhekisela kokubili umnkantsha wamathambo kanye nezinhlobo ezithile zamaseli egazi athola umnkantsha.

I-AML ivela esitokisini esigazini segazi esibizwa ngokuthi i-myeloblast.

Lawa angamaseli ukuthi, ngaphansi kwezimo ezivamile, ayezovuthwa ekufakeni ngokugcwele amangqamuzana egazi amhlophe njenge- granulocytes noma ama-monocytes . Kodwa-ke, nge-AML, ama-myeloblasts azosebenza "efriziwe" esimweni sabo esishadile kodwa aqhubeke nokuphindaphindiwe angahlosiwe.

Ngokungafani namaseli avamile anempilo ethile, amangqamuzana omdlavuza "angabhubhi" futhi azoqhubeka ephindaphinda ngaphandle kokuphela.

Nge-AML, amangqamuzana egazi omdlavuza azogcina ekuxosheni izinto ezijwayelekile futhi aphazamise nokuthuthukiswa kwamaseli amhlophe egazi amasha, amangqamuzana egazi abomvu (ama-erythrocytes), namaplatelets ( thrombocyte ).

I-AML ayifani nomzala wayo we -lymphocytic i-leukemia (KONKE) ethinta olunye uhlobo lweseli elimhlophe legazi elibizwa ngokuthi i- lymphocyte . Ngenkathi i-AML ithinta ngokuyinhloko abadala asebekhulile, BONKE bahlasela izingane phakathi kweminyaka emibili kuya kwemihlanu.

Izimpawu Zokuqala Nezibonakaliso

Izimpawu ze-AML zihlobene ngokuqondile nokufuduka kwamangqamuzana egazi avamile ngabadlavuza. Ukungabikho kwamaseli ejwayelekile egazi kungashiya umuntu obhekene nengozini yokutheleleka kanye nezinye izifo umzimba ongazivimbela.

Ngokomfanekiso, amangqamuzana egazi amhlophe aphakathi komzimba omzimba. Amangqamuzana egazi abomvu, ngokuphambene, anesibopho sokuthwala i-oksijeni nokususa i-carbon dioxide ezicukwaneni, kuyilapho amaplatelets ayisihluthulelo sokuvala igazi.

Ukunciphisa kwanoma yiliphi lala maseli kungabangela ukuhlaselwa kwezimpawu, ngokuvamile okungezona okucacile futhi okunzima ukuyihlolisisa. Izibonelo zifaka:

• Ukuntuleka kwamangqamuzana amhlophe wegazi kungandisa ingozi yezifo ezingeke zisuke. Lokhu kufaka phakathi izimpawu ezihlobene nokungabi nalutho lwe-leukocytes (leukopenia) noma i-neutrophils ( neutropenia ).

• Ukuntuleka kwamangqamuzana egazi abomvu kungaholela ekudleni kwegazi okungabonakaliswa ngezimpawu zokukhathala, ubuhlungu, ukuphefumula, ukuphathwa ikhanda, isizungu, nobuthakathaka.
• Ukuntuleka kwamaplatelets kungaholela ekutheni i- thrombocytopenia kanye nokuthuthukiswa kwezinsini eziphuma egazini, ukulimala ngokweqile noma ukuphuma kwegazi, noma ukuphuza ngokweqile noma okunzima kakhulu.

Izimpawu zokuhamba kwesikhathi kamuva

Njengoba lesi sifo sithuthuka, ezinye, izimpawu ezitshela ngaphezulu zingaqala ukuthuthukisa. Ngenxa yokuthi amangqamuzana e-leukemia amakhulu kunamaseli egazi amhlophe ajwayelekile, cishe angabanjwa ezintweni ezincane zesistimu yokujikeleza noma ukuqoqa izitho ezihlukahlukene zomzimba.

Kuncike lapho ukuvinjelwa kwenzeka, umuntu angase athole:

• Ama-chloromas , iqoqo eliqinile lamaseli angathuthuka abe, ngokuhlukile, inqwaba efana ne-tumor ngaphandle komnyosa we-bone, ukuqhuma okufana ne-plaque, noma ukuphuma okubuhlungu nokuvuvukala kwezinsini
• I-Leukostasis , isimo esiphuthumayo sezokwelapha lapho ukuvinjelwa kungabangela izimpawu ezifana nesifo sohlangothi
• I-Sweet's syndrome , ukuphuza isikhumba ebuhlungu kakhulu kubonakala ezingalweni, enhloko, emilenzeni nasesitini
• I-thrombosis ye-vein deep (DVT) lapho i-vein izovinjelwa khona, kaningi emlenzeni
• I-embolism ye-pulmonary (PE) , ukuvimba kwe-artery emaphashini
• Ukuvalelwa kwesisu ngenxa yokuqoqwa kwamangqamuzana e-spleen nesibindi
• I-meningeal leukemia ebonisa ukukhathazeka okuphakathi kwezinzwa ezifana nokukhanda ikhanda, ukuhlanza, umbono ongenalutho, ukuhlukunyezwa, ukuxubha inkathazo, nokungafihli ubuso

Ngokuvamile, ama-AML angathinta izinso, amantombazane amancane, amehlo, noma amaconsi.

Izimbangela Nezici Zengozi

Kunezinhlobo eziningi zobungozi ezihlobene ne-AML. Kodwa-ke, ukuba neyodwa noma ngisho nalezi zici ezimbalwa akusho ukuthi uzothola i-leukemia. Kuze kube manje, asikaqondi ngokugcwele ukuthi kungani amanye amangqamuzana azophenduka ngokungazelelwe abe nomdlavuza kuyilapho abanye bengaboni.

Lokho esikwaziyo ukuthi i-khansa ibangelwa iphutha lokubhala i-genetic elingase livele ngezinye izikhathi uma iseli lihlukanisa. Sibhekisela kulokhu njengenguquko. Ngenkathi iningi lezinguquko zingadlulisi emdlalweni, kunezikhathi lapho iphutha "liyovala" into ebizwa ngokuthi isifo sofuba esisindayo esichaza ukuthi ihlala isikhathi eside kangakanani. Uma lokhu kwenzeka, i-cell engavamile ingahle iphinde iphinde iphelelwe yisilawuli.

Kunezinhlobo eziningi zobungozi ezihlobene nalokhu:

• Ukubhema
• Ukuvezwa kwemisebenzi yamakhemikhali, ikakhulukazi ku-benzene
• Ezinye izidakamizwa zamakhemikhali we-cancer ehlanganisa i- cyclophosphamide , mechlorethamine, procarbazine, i-chlorambucil, i-melphalan, i-busulfan, i-carmustine, i-cisplatin, ne-carboplatin
• Ukuvezwa kwemisebe ephezulu, njengomdlavuza we-radiotherapy
• Ukuba nezinkinga ezithile zegazi ezingapheli ezifana nesifo se-myeloproliferative (MPS) noma i- myelodysplastic syndromes (MDS)
• Ukuba nezinkinga ezithile zokubeletha ezifana ne- Down syndrome , i- Fanconi anemia , ne- neurofibromatosis uhlobo 1

Ngenxa yezizathu ezingaziwa, amadoda angamaphesenti angu-67 amathuba okuthola i-AML kunabesifazane.

Ukuxilongwa

Uma kukhishwa i-AML, ukuxilongwa kuzovame ukuqala ngokuhlolwa ngokomzimba nokubuyekeza umlando womuntu wezokwelapha nomndeni. Ngesikhathi sokuhlolwa, udokotela uzoziqaphela ngokucophelela izibonakaliso ezinjengobuhlungu obuningi, ukuphuma kwegazi, ukutheleleka, noma ukungajwayelekile kwamehlo, umlomo, isibindi, uhlangothi, noma i-lymph nodes. Inani eliphelele legazi (CBC) lizokwenziwa futhi ukukhomba noma yikuphi okungavamile ekubunjweni kwegazi.

Ngokusekelwe kulokho okutholwe, udokotela angase alawule izibalo eziningi ukuqinisekisa ukuxilongwa. Lokhu kungafaka:

• I-bone yomnkantsha okufisa lapho amangqamuzana omnkantsha wamathambo akhishwa ngokufaka inaliti ende emathanjeni, ngokuvamile eseduze nesikhumba
• I-bone yomnkantsha we-bone lapho inaliti enkulu efaka khona ngaphakathi emathanjeni ukuze ikhiphe amaseli
• Ukukhishwa kwe-Lumbar (umgogodla wempuphu) lapho inaliti encane ifakwa phakathi kwamathambo o ikholomu yomgogodla ukukhipha cerebrospinal fluid (CSF )
• Ukucubungula ukuhlolwa okufana ne-X ray, i-ultrasound, noma ukuskena kwe- tomography (CT )
• I-bloodpress blood smear lapho igazi lihlolwe ngaphansi kwe-microscope, ngokujwayelekile ngamadayi angagcizelela kuphela amangqamuzana e-leukemia kodwa asize ukuhlukanisa phakathi kwe-AML ne-ALL
• I-cytometry egeleza lapho amaprotheni avikelayo, okuthiwa ama-AML antibodies, aqaliswa egazini noma e-CSF isampula ukuqinisekisa ukuthi kukhona ama-AML cells
• I-cytogenetics lapho amangqamuzana e-leukemia "akhulile" ebhodini bese ehlolwe ngaphansi kwe-microscope ye-electron ukukhomba ukuguqulwa okuqondile ngamaphethini abo okuchithwa kwe-chromosomal

Isiteji

Ukumiswa komdlavuza kwenziwa ukuze kunqume ukuthi umdlavuza usakaze kangakanani. Lokhu, nakho, kusiza udokotela ukuba anqume inkambo efanele yokwelashwa ukuze umuntu angaphathwa kabi noma angaphathwa kabi. Isiteji sisiza futhi ukubikezela ukuthi umuntu angasinda isikhathi eside kangakanani ngemuva kokwelashwa.

Ngenxa yokuthi i-AML ayifaki ukubunjwa kwesifo esibuhlungu esibonakala kwezinye izinhlobo zomdlavuza, ayikwazi ukuhlelwa nge- TNM ( indlela yokulimala / i-lymph node / malignancy ) ye-classic.

Kunezinqubo ezimbili ezahlukene okwamanje ezisetshenziselwa isigaba se-AML: isigaba samaFrench-American-British (FAB) se-AML kanye nesigaba se-World Health Organization (WHO) se-AML.

Ukufakwa kwe-FAB

Isigaba samaFrench-American-British (FAB) sakhiwa eminyakeni yama-1970 kanye nezigaba lesi sifo esekelwe ngohlobo nokuvuthwa kweseli elichaphazelekayo.

Isizathu sokwenza isilula silula: i-AML izovame ukulandela iphethini lapho ama-myeloblasts angakazalwa amaseli okuqala angathinteka. Njengoba lesi sifo siphuthuka, sizoqala ukuphazamisa ama-myeloblasts ezinyathelweni ezilandelayo zokuvuthwa, bese uthuthukela emaqenjini amhlophe egazi amhlophe (njengama-monocytes nama-eosinophils) ngaphambi kokuthuthela emangqamuzana egazi abomvu (erythrocytes) futhi ekugcineni megakaryoblasts (amangqamuzana e-platelet amancane).

Lokhu kuqhubeka kuzohlinzeka ngolwazi oludingekayo ukuze ukwazi ukuthi umdlavuza uphambili kangakanani.

Izindawo ze-FAB zokulinganisa ukusuka ku-M0 (nge-AML yokuqala) ukuya ku-M7 (ye-AML ethuthukile) kanje:

• I-M0: i-leukemia e-myeloblastic engafanele
• I-M1: i-leukemia e-myeloblastic acute ene-maturation encane
• I-M2: i-leukemia ephawulekayo ye-myeloblastic ne-maturation
• I-M3: i-acute promyelocytic leukemia
• I-M4: i-myelomonocytic i-leukemia
• I-M4 eos: i-myelomonocytic i-leukemia elula ne-eosinophilia
• I-M5: i-leukemia enkulu ye-monocytic
• I-M6: i-leukemia e-erythrocytic elula
• I-M7: i-leukemia ye-megakaryoblastic acute

I-WHO Classification

I-World Health Organization yaqala izindlela ezintsha zokwenza i-AML ngo-2008. Ngokungafani nesistimu ye-FAB, ukuhlukaniswa kwe-WHO kuhlolisisa ukushintshashintsha kwe-chromosomal okutholakala ngesikhathi kuhlaziywa kwe-cytogenetic. Kubuye kubhekane nezimo zezokwelapha ezingase zithuthukise noma zibhebhethekise umbono (ukubikezelwa) komuntu othintekile.

Uhlelo lwe-WHO luyinto enamandla kakhulu ekuhloleni kwalo isifo futhi lungabhidliza kabanzi kanje:

• I-AML enezinhlobo ezivamile zezofuzo (okusho okucacile, izici ezithinta izakhi zofuzo)
• I-AML nezinguquko ezihlobene nemyelodysplasia (okusho ukuthi kukhona i-MDS, i-MDP, noma ezinye izifo ezithinta i-myeloblastic)
• Amaphilisi amakhemikhali ahlobene ne-thérapie (okuhlobene okuhlobene ne-chemotherapy yangaphambili noma ukwelashwa kwama-radiation)
• I-syeloid sarcoma (okusho ukuthi i-AML ihambisane ne-chloroma)
• Ukwanda kweMyeloid okuhlobene ne-Down syndrome
• I-plastic plasmacytoid dendritic cell neoplasm (uhlobo olunonya lomdlavuza olubonakala ngezilonda zesikhumba)
• I-AML ayihlukaniswe ngenye indlela (ngokuyisisekelo uhlelo lwe-FAB lwesigaba esinezigaba ezimbili ezengeziwe zezifo)

Ukwelapha

Uma kutholakala nge-AML, ifomu kanye nobude besikhathi sokwelapha kuzokwenziwa ikakhulukazi ngesigaba somdlavuza kanye nempilo ejwayelekile yomuntu ngamunye.

Ngokuvamile, ukwelashwa kuzoqala nge-chemotherapy. Lokhu kungabandakanya izidakamizwa ezizukulwaneni zakudala ezingaba nomthelela kokubili amangqamuzana anomdlavuza nabangewona umdlavuza kanye nezidakamizwa ezihlosiwe ezizukulwaneni ezishayelwayo ezingenayo amangqamuzana omdlavuza yedwa.

Imithi ejwayelekile ye-chemotherapy ibizwa ngokuthi "i-7 + 3" ngoba imithi ye-chemotherapy eyaziwa ngokuthi i- cytarabine inikezwa njenge-infusion eqhubekayo (IV) izinsuku ezingu-7 ezilandelwa izinsuku ezintathu zokulandelana komunye umuthi obizwa ngokuthi i- anthracycline . Kuze kube ngamaphesenti angu-70 abantu abane-AML bayothola ukukhululwa okulandelayo ngemuva kokwelapha "7 + 3".

Uma kuthiwa, inani elincane lamangqamuzana e-leukemia cishe lizohlala lilandela i-chemotherapy, okuholela ekuphindeni emuva kwamacala amaningi. Ukuze ugweme lokhu, odokotela bazobeka ukwelashwa okuqhubekayo ngokusekelwe emiphumeleni yomuntu ngemuva kokuphathwa kanye nesimo sezempilo.

Kubantu abanezinkomba zokuhlonza kahle, ukwelashwa kungabandakanya izifundo ezintathu kuphela eziyisihlanu zamakhemikhali aqinile, okubizwa ngokuthi i-chemotherapy ehlanganisiwe.

Kulabo abasengozini enkulu yokuphindaphinda, ezinye, zokwelashwa okudinga ukwedlulela kungadingeka kufaka phakathi ukufakelwa kwe-stem cell uma umnikeli engatholakala. Kungavamile ukuhlinzwa, ukuhlinzwa noma imithi yokwelapha.

Ngenxa yokuthi i-AML chemotherapy ivame ukuholela ekucindezelweni okukhulu kwamagciwane, iziguli ezindala zingase zingakwazi ukubekezelela ukwelashwa futhi kungenzeka zinikezwe i-chemo encane noma ukunakekelwa kwe-palliative .

Ukusinda

Umbono womuntu oye waphathwa ukwelashwa kwe-AML ungashintsha ngokuphawulekayo ngokusekelwe esigabeni somdlavuza ngesikhathi sokuxilongwa. Kodwa, kunezinye izici ezingase zibikezele ukuthi kungenzeka yini. Phakathi kwazo:

• Abantu abathintekayo abane-MDS kanye ne-MPD banesikhathi sokusinda kusukela ezinyangeni eziyisishiyagalolunye kuya ku-11.8 iminyaka kuye ngokuthi ubuchopho bubucayi kangakanani.
• Izinguquko ezithile ze-chromosomal ezikhonjiswe yi-cytogenetics zingaholela emalini ominyaka emihlanu okusinda aphansi ngamaphesenti angu-15 ukuya phezulu ngamaphesenti angu-70.
• Abantu abangaphezu kuka-60 abaye baphakamisa ama- lactate dehydrogenase (okukhombisa umonakalo omkhulu wesikhumba) ngokuvamile banemiiphumela empofu.

Ngokuvamile, izinga lokuphulukisa isilinganiso le-AML liphakathi kwamaphesenti angu-20 namaphesenti angu-45. Izindleko zokuxoshwa ezixhaswe zivame ukuphakama kubantu abasha abakwazi ukubekezelela ukwelashwa.

Izwi elivela

Uma uthola ukuthi une-AML, uzobhekana nezinselele ezingokomzwelo nezingokomzimba okungase kube nzima ukunqoba. Ungayi yedwa. Amathuba akho okuphumelela azoba ngcono nakakhulu uma wakhe inethiwekhi yokwesekwa eyenziwe ngabathandekayo, ochwepheshe bezempilo, nabanye abaye bahamba noma bawela ukwelashwa komdlavuza.

Ngisho nangemva kokuthola ukwelashwa, ukwesaba ngokuphindaphindiwe kungase kuphele izinyanga noma ngisho neminyaka. Ngokusekelwa, ekugcineni uzowunqoba lezi zikhathazo futhi ufunde ukuqapha impilo yakho ngokuvakashelwa odokotela njalo. Ngokuvamile, uma ubuyela emuva akuzange kwenzeke phakathi neminyaka embalwa, akunakwenzeka ukuthi i-AML izoke ibuye.

Nakuba kungekho lutho ongayithatha ukuvimbela ukubuyela emuva, indlela yokuphila enempilo ingathuthukisa kakhulu izimo zakho. Lokhu kuhlanganisa ukunikeza imikhuba emihle yokudla, ukuzivocavoca njalo, ukuyeka ukubhema, nokuthola okuningi ukugwema ukucindezeleka nokukhathala.

Ekugcineni, kubalulekile ukuthatha izinto usuku ngalunye futhi ube nomuntu ongaziphendulela uma ngabe udinga ukwesekwa.

> Imithombo:

> I-American Cancer Society. "Ukusinda Izibalo ZeMyelodysplastic Syndromes." Washington, DC; ibuyekeziwe ngoJanuwari 22, 2018.

> De Kouchenovsky, I. no-Abdul Hay, M. "I-leukemia eqondile ye-myeloid: ukubuyekezwa okunzulu nokubuyekezwa kuka-2016." Igazi elinguJ 2016; 6; e441.

> Döhner, H .; I-Weisdorf, D .; neBloomfield, C. "I-Acute Myeloid Leukemia." I-Engl J Med entsha . 2015; 373 (12): 1136-52. I-DOI: 10.1056 / NEJMra1406184.