Ngaba ukhe weva ngalo mhlaza wegazi unzima? (Acute Promyelocytic Leukemia - APL) Masithethe ngokweenkcukacha!

Ngaba wakha waziva udiniwe kakhulu ngequbuliso, waba nemivumbo embalwa, okanye uphuma igazi rhoqo kwiintsini? Nangona singazinaki kakhulu ezi zinto, kwiimeko ezingaqhelekanga, zinokuba ziimpawu zesimo esibi ngakumbi, njenge -Acute Promyelocytic Leukemia (APL) . Ngoko ke, ungakhathazeki, namhlanje siza kuthetha ngalo mhlaza wegazi obizwa ngokuba yi-APL ngendlela ecacileyo nelula.

Yintoni i-acute promyelocytic leukemia (APL)?

Ngamafutshane, i-APL luhlobo olungaqhelekanga kakhulu lomhlaza wegazi . Enyanisweni ikwiqela elikhulu lee-leukemias ezibizwa ngokuba yi -Acute Myeloid Leukemia . Indawo ephambili apho igazi lenziwa khona emzimbeni wethu yi -bone marrow . Apha kule bone marrow, ngenxa yotshintsho lwe-genetic, oko kukuthi, utshintsho lwe-genetic , iiseli ezimhlophe zegazi ezingaqhelekanga ziqala ukwakheka. Ezi seli ezingaqhelekanga ziyahlukana kwaye zande ngokungalawulekiyo, zisasazeka kuyo yonke i-bone marrow. Ngamanye amaxesha oogqirha babiza le APL leukemia, okanye i-M3-leukemia .

I-APL yimeko enzima kakhulu. Iimpawu zayo zinokuvela ngequbuliso kwaye zibe mandundu ngokukhawuleza. Ukopha kakhulu yingxaki enkulu. Kodwa kukho iindaba ezimnandi! Ngonyango olutsha, kuquka i-chemotherapy kunye namayeza amatsha angengowe-chemotherapy, oogqirha bayakwazi ukulawula i-APL kwaye bahlala beyinyanga ngokupheleleyo.

Ixhaphake kangakanani le sifo?

I-APL sisifo esingaqhelekanga ngokwenene. Umzekelo, kwilizwe elifana ne-United States, ngabantu abamalunga nama-30,000 kuphela abafunyaniswa benesi sifo minyaka le. Ngokuqhelekileyo, i-APL ifunyaniswa kubantu abakwiminyaka yabo yama-30 .

Zithini iimpawu ze-APL?

Iimpawu ze-APL zenzeka xa umongo wakho wethambo ungakwazi ukwenza iiseli ezibomvu zegazi eziphilileyo, iiseli ezimhlophe zegazi, kunye neeplatelets njengesiqhelo. Oku kuncipha kwazo zonke ezi ntlobo zeeseli zegazi kubizwa ngokuba yi-pancytopenia . Xa oku kusenzeka, unokuba neempawu ezinzulu ezifana ne -anemia , iingxaki zokopha ngenxa yokuxinana kwegazi, kunye nezifo ezixhaphakileyo.

Ezinye iimpawu eziqhelekileyo ze-APL zezi:

• Ukuziva udiniwe kakhulu kwaye udiniwe ngenxa yokuncipha kweeseli ezibomvu zegazi (oko kukuthi, i-anemia).
• Usulelo oluqhelekileyo olubangelwa ziiseli ezimhlophe zegazi ezisezantsi ezilwa nezifo. Izinto ezifana nomkhuhlane kunye nomkhuhlane zinokwenzeka rhoqo.
• Ngenxa yokuba imetabolism yomzimba wakho ikhawuleza kwaye amandla avela ekutyeni atsha ngokukhawuleza,Ukunciphisa umzimba ngengozi.

Iimpawu zokopha

Umntu one-APL unenani eliphantsi lee-platelet kunye nezinto ezibangela ukujiya kwegazi ezinceda ukujiya kwegazi . Uyazi, ii-platelet zezona zinceda ekumiseni okanye ekunciphiseni ukopha. Ngoko ke, xa ezi ziphantsi, ingxaki iqala.

Nazi ezinye iimpawu zokopha okubangelwa yi-APL:

• Ukopha kunokwenzeka naphi na emzimbeni. Umzekelo, ukopha iintsini , ukopha rhoqo ngeempumlo , kwaye, kubafazi, ukopha kakhulu ukuya exesheni .
• Igazi liqokelelana phantsi kolusu lize libangele ukukrweleka kwiindawo ezahlukeneyo emzimbeni . Nokuba likrwelekile elincinci linokubangela ukukrweleka okukhulu.
• Ngamanye amaxesha ukopha kuyenzeka ngaphakathi kwengqondo (intracranial hemorrhage) . Ukuba oku kuyenzeka, unokuba nobunzima bokushukumisa amalungu akho omzimba, ube neentloko ezibuhlungu kakhulu, kwaye unokufumana utshintsho ekuboneni kwakho. Le yimeko engxamisekileyo!
• Ukuba indle yakho imnyama okanye inemigca yegazi, isenokuba ibangelwa kukopha kwindlela yokugaya ukutya (i-gastrointestinal bleeding) .

Yintoni ebangela i-APL?

Esi sifo sibangelwa ziijini ezimbini ezilawula ukuveliswa kweeseli zethu zegazi ezidibanayo ukuze zenze ijini engaqhelekanga ebizwa ngokuba yi-PML-RARa . Okubalulekileyo kukuba, olu tshintsho lwejini aluyonto oyizuza njengelifa kubazali bakho . Lwenzeka ngengozi, ngaphandle kwesizathu, ngexesha lobomi bakho. Iingcali azikazi ukuba yintoni kanye kanye ebangela olu tshintsho lwejini.

Olu tshintsho lwenzeka lubangela ukuba iiseli ezimhlophe zegazi zikhule zingalawuleki, endaweni yokuba zikhule ngokufanelekileyo, endaweni yoko zenze iiseli ezimhlophe zegazi ezingavuthwanga (iipromyelocytes) . Ezi seli zingavuthwanga zizalisa umongo wethambo, ziminyanise indawo yeeseli zegazi ezisempilweni kunye neeplatelets.

Zithini iingxaki ze-APL?

I-APL yimeko esongela ubomi. Oku kungenxa yokuba ukopha kakhulu okwenzekayo kunokuba yingozi ngokukhawuleza. Ukuba awukwazi ukuyeka ukopha nokuba kukusikeka okuncinci, ukuba unegazi elininzi kwindle yakho okanye kumchamo xa usiya kwindlu yangasese, okanye ukuba awukwazi ukuyeka ukopha kwiintsini zakho, kufuneka ubone ugqirha okanye uye kwigumbi likaxakeka esibhedlele ngoko nangoko .

Khumbula: Ukuba ukopha kwakho akulawuleki, ungaze ukutyeshele. Unyango olukhawulezileyo lubalulekile.

Ifunyaniswa njani i-APL?

Oogqirha badla ngokwenza iimvavanyo ezininzi ukuze bafumanise esi sifo.

• Ukubalwa kwegazi okupheleleyo (CBC): I-APL ibangela ukwakheka kweeseli ezimhlophe zegazi ezingaqhelekanga. Uvavanyo lwe-CBC lunokujonga iintlobo ezahlukeneyo zeeseli zegazi kunye nenani leeplatelets kwisampuli yegazi lakho.
• Ukutshiza igazi okujikeleze i-peripheral:Kule meko, kuthathwa isampuli yegazi ize ihlolwe phantsi kwe-microscope. Emva koko, bajonga ukuba kukho iigranules ezininzi ezincinci ngaphakathi kwezo seli zegazi ezimhlophe ezingavuthwanga (ii-promyelocytes), okanye kukho izinto ezikhethekileyo ezibizwa ngokuba zii-Auer rods . Ezi ziimpawu ze-APL.
• I-Bone Marrow Biopsy: Kolu vavanyo, kuthathwa isampuli encinci yomongo wakho wethambo kwaye iiseli zawo ziyahlolwa.
• I-Flow Cytometry: Kolu vavanyo, iingcali zezifo zikhangela iipateni zeeproteni ezithile kumphezulu weeseli ezingaqhelekanga. Ezi pateni zinokuqinisekisa i-APL.
• Uvavanyo lwePolymerase Chain Reaction (PCR): Olu vavanyo lunokufumanisa ngokuchanekileyo ubukho bejini engaqhelekanga (PML-RARa) ebangela i-APL.
• I-Cytogenetics: Oku kuvavanya utshintsho oluthile kwii -chromosomes zeeseli ezingaqhelekanga. Ukufumana olu tshintsho yeyona ndlela iphambili yokuqinisekisa ukuxilongwa kwe-APL.

Oogqirha babeka i-APL njengenobungozi obuphantsi okanye obuphezulu ngokusekelwe kumanani eeseli ezimhlophe zegazi lakho. Abantu abane-APL enobungozi obuphezulu banamathuba amaninzi okuba nomhlaza ophindaphindiweyo okanye ukuphinda babuye .

Iphathwa njani i-APL?

I-APL inyangwa ngokudibanisa ii-differentiation agents , i-chemotherapy, kunye nonyango olujoliswe kuyo . Enyanisweni, ukusukela oko kwafunyanwa olu nyango ngeminyaka yoo-1980, esi sifo, esasicingelwa ukuba siyabulala ngaphambili, ngoku sibe sisifo esinyangekayo. Le yimpumelelo enkulu!

Amayeza okwahlula iiseli ngamayeza angengowe-chemotherapy anceda iiseli ezimhlophe zegazi ezingaqhelekanga, ezingavuthwanga ukuba zahlulwe zibe ziiseli ezimhlophe zegazi eziqhelekileyo, ezivuthiweyo. La mayeza angengowe-chemotherapy, adweliswe ngezantsi, abangele ukuba kubekho ukuxolelwa kunye nokuphiliswa okungaphezulu kwama-95%.

• I-All-trans-retinoic acid (ATRA) , okanye i-tretinoin (Vesanoid ®) – Olu luhlobo lwevithamini A.
• I-Arsenic trioxide (ATO) – Olu luhlobo lwe-arsenic.

Ukuba ugqirha wakho ukrokrela ukuba une-APL, banokukuqalisa nge -ATRA nangaphambi kokuba ezinye iimvavanyo ziqinisekise esi sifo. Oku kungenxa yokuba ukuqala unyango kwangethuba kunokunciphisa umngcipheko wokopha okusongela ubomi.

Amanqanaba onyango lwe-APL

Unyango ludla ngokwenziwa ngamanqanaba amathathu: Ukungenisa, Ukudibanisa, kunye nokugcina. Unyango lunokwahluka ngokuxhomekeke kwinqanaba lomngcipheko.

• Inqanaba lokuqala (Ukuqaliswa):Injongo ephambili yale nqanaba kukubulala iiseli ze-leukemia ezaneleyo ukuze i-APL yakho ibuyele esiqhelweni . Ukukhululwa kuthetha ukuba awunazo iimpawu kwaye akukho zimpawu ze-leukemia ezinokufunyanwa kwiimvavanyo. Oku kwenziwa ngokusebenzisa amayeza angengonyango lwe-chemotherapy, i-chemotherapy, kunye nonyango olujoliswe kulo. Ngeli xesha, kuya kufuneka uhlale esibhedlele, ngesiqhelo iiveki ezine ukuya kwezintandathu.
• Isigaba sokudibanisa: Ugqirha wakho we-oncologist angabiza olu 'nyango lwasemva kokuxolelwa'. Olu nyango lugcina i-APL ikwimeko entle kwaye luyaqhubeka nokubulala naziphi na iiseli ze-leukemia eziseleyo. Eli liyeza elifanayo elalisetyenziswa kwisigaba sokuqala. Olu nyango lunokuhlala malunga neenyanga ezisibhozo, kunye nonyango rhoqo emva kweenyanga ezimbini. Usenokuba neeveki ezine zonyango ezilandelwa ziiveki ezine zokuphumla. Eli yeza linokunikwa njengepilisi okanye njengonyango lwe-intravenous (IV) .
• Isigaba solondolozo: Olu lunyango oluqhubekayo, kodwa amayeza anikwa ngedosi ephantsi kunakwisigaba sokuqala nesokuzinzisa. Olu nyango lolondolozo ludla ngokunikezelwa malunga nonyaka.

Ugqirha wakho we-oncologist angakunika unyango oluxhasayo , olufana notofelo-gazi, kunye nolu nyango.

Iingxaki zonyango

Eyona ngxaki ixhaphakileyo, neyinzima kancinci, ibizwa ngokuba yi -differentiation syndrome . Eli liqela leempendulo eziqatha kumayeza e-APL. Ezi mpendulo zihlala zenzeka kwiiveki ezintathu zokuqala emva kokuqala unyango. Iimpawu zinokuba zincinci okanye zibe nzima. Ezinye zazo ziquka:

• Ukukhwehlela.
• Ulwelo olongezelelekileyo olujikeleze intliziyo yakho nemiphunga (i-pleural effusion) .
• Ukungasebenzi kakuhle kwezintso .
• Uxinzelelo lwegazi oluphantsi (uxinzelelo lwegazi oluphantsi) .
• Ukwehla kwamanqanaba eoksijini egazini (i-hypoxemia) .
• Ubunzima bokuphefumla (ukuphelelwa ngamandla) .
• Ukudumba/ ukudumba kweengalo zakho, imilenze, nentamo.
• Umkhuhlane oza ngaphandle kwesizathu.
• Ukunyuka kobunzima ngaphandle kwesizathu.

Ukuba unesifo sokwahlukana, ugqirha wakho unokumisa unyango lwakho okwethutyana. Basenokusebenzisa nezinye iindlela zonyango, ezifana ne -hydroxyurea, ukunciphisa inani leeseli zegazi ezimhlophe.

Yintoni enokulindelwa ngumntu one-APL?

Ngokubanzi, ingqikelelo yesifo ilungile.Nangona imeko yomntu wonke yahlukile, izifundo zibonise ukuba phakathi kwama-90% nama-95% abantu abane-APL baya kuphulukana nempilo yabo. Nangona kunjalo, i-APL inokubuya emva konyango. Phakathi kwama-5% nama-10% abantu baya kuphinda babuyele kwimeko yesiqhelo, ngesiqhelo kwiminyaka emithathu yokuqala emva konyango. Emva koko baya kufuna olunye unyango okanye olunye.

Amanqanaba okusinda

Ngenxa yokuba i-APL sisifo esingaqhelekanga, esikwaziyo malunga namazinga okusinda kuvela kuvavanyo lweklinikhi olubandakanya izigulane ze-APL ezinobungozi obuphantsi nezinomngcipheko ophezulu.

Olunye uhlalutyo lubonisa ukuba ama-99% ezigulane ze-APL ezisengozini ephantsi ziyaphila emva kweminyaka emine emva konyango. Olunye uhlalutyo lwezigulane ze-APL ezisengozini ephezulu lubonisa ukuba ama-86% ayaphila emva kweminyaka emihlanu emva konyango. Ezi ziziphumo ezilungileyo ngokwenene!

Ndingazinyamekela njani?

Ngenxa yokuba i-APL ingaphinda ivele, kubaluleke kakhulu ukuba ubonane nogqirha wakho ngexesha (ii-aphoyintimenti zokulandelela) .

Uza kuhlolwa ngugqirha rhoqo ngenyanga okanye ezimbini kunyaka wokuqala emva konyango. Emva konyaka wokuqala, kuya kufuneka ubone ugqirha wakho malunga neenyanga ezintathu ukuya kwezine kwiminyaka emibini ezayo. Uvavanyo olufana ne-CBC, i-PCR, kunye ne-bone marrow biopsy lunokwenziwa kwezi tyelelo.

Ndingaya nini kwigumbi likaxakeka?

I-APL ingaphinda ivele emva konyango, kwaye iimpawu zingaba mandundu ngokukhawuleza. Ukuba uphulukene ne-APL, yiya kwigumbi likaxakeka ngokukhawuleza ukuba ufumana nayiphi na kwezi zilandelayo:

• Ukuba wopha kakhulu .
• Ukuba ngequbuliso uqalisa ukudumba kunye nentlungu emilenzeni okanye esiswini esisezantsi .

Okokugqibela, umyalezo obalulekileyo

I-Acute promyelocytic leukemia (APL) ngumhlaza wegazi ongaqhelekanga owawukade uthathwa njengesifo esibulalayo, kodwa ngoku ube sisifo esinyangekayo ngenxa yonyango oluphambili.

Nangona kunjalo, esi sisifo esibi kakhulu. Ukuba asifunyaniswa kwaye sinyangwe ngokukhawuleza, sinokuba yingozi ebomini . Ke ngoko, ukuba uneempawu ezifana nokopha okungalawulekiyo, ungaze uyityeshele loo nto. Eyona nto ibalulekileyo kukufuna ingcebiso yezonyango kwaye ufumane ukuxilongwa ngokukhawuleza. Musa ukoyika, yiba nesibindi, kuba ngoku kukho unyango olufanelekileyo loku!

I- Leukemia, i-APL, umhlaza wegazi, umhlaza wegazi, umongo wamathambo, i-anemia, i-ATRA, unyango lwekhemotherapy, i-Acute Promyelocytic Leukemia, i-M3-Leukemia, i-PML-RARa, ukopha