Uke wezwa ngalo mdlavuza wegazi oyingozi? (Acute Promyelocytic Leukemia - APL) Ake sixoxe ngokuningiliziwe!

Wake wazizwa ukhathele kakhulu ngokuzumayo, waba nemivimbo embalwa, noma waba nokuphuma kwegazi njalo kwezinsini? Nakuba singazinaki kakhulu lezi zinto, ezimweni ezingavamile, zingaba izimpawu zesimo esibucayi kakhulu, njenge -Acute Promyelocytic Leukemia (APL) . Ngakho-ke, ungakhathazeki, namuhla sizokhuluma ngalomdlavuza wegazi obizwa ngokuthi i-APL ngendlela ecacile nelula.

Kuyini i-acute promyelocytic leukemia (APL)?

Kalula nje, i-APL uhlobo lomdlavuza wegazi olungavamile kakhulu . Empeleni luyingxenye yeqembu elikhulu lama-leukemia abizwa ngokuthi i-Acute Myeloid Leukemia . Indawo eyinhloko lapho igazi lenziwa khona emzimbeni wethu yi -bone marrow . Lapha kulo mnkantsha, ngenxa yoshintsho lwezakhi zofuzo, okungukuthi, ukuguquka kwezakhi zofuzo , amangqamuzana amhlophe egazi angajwayelekile aqala ukwakheka. Lawa maseli angajwayelekile ahlukana futhi aphindaphindeke ngokungalawuleki, asakazeke kuwo wonke umnkantsha wethambo. Ngezinye izikhathi odokotela babiza lokhu ngokuthi i-APL leukemia, noma i-M3-leukemia .

I-APL iyisimo esibucayi. Izimpawu zayo zingavela ngokuzumayo futhi zibe zimbi ngokushesha. Ukopha ngokweqile kuyisici esikhulu sengozi. Kodwa kunezindaba ezinhle! Ngezindlela ezintsha zokwelapha, okuhlanganisa i-chemotherapy kanye nemithi emisha engeyona i-chemotherapy, odokotela bayakwazi ukulawula i-APL futhi bavame ukuyilapha ngokuphelele.

Sivame kangakanani lesi sifo?

I-APL empeleni iyisifo esingavamile kakhulu. Isibonelo, ezweni elifana ne-United States, abantu ababalelwa ku-30,000 kuphela abatholakala benalesi sifo minyaka yonke. Ngokuvamile, i-APL itholakala kubantu abaseminyakeni yabo yo-30 .

Ziyini izimpawu ze-APL?

Izimpawu ze-APL zenzeka lapho umongo wakho wethambo ungakwazi ukwenza amangqamuzana abomvu egazi aphilile, amangqamuzana amhlophe egazi, nama -platelet ngendlela evamile. Lokhu kuncipha kwazo zonke lezi zinhlobo zamangqamuzana egazi kubizwa ngokuthi i-pancytopenia . Uma lokhu kwenzeka, ungaba nezimpawu ezinkulu ezifana ne -anemia , izinkinga zokopha ngenxa yokujiya kwegazi, kanye nezifo ezivamile.

Ezinye izimpawu ezivamile ze-APL yilezi:

• Ukuzizwa ukhathele kakhulu futhi ukhathele ngenxa yokwehla kwamangqamuzana abomvu egazi (okungukuthi, i-anemia).
• Ukutheleleka okuvamile okubangelwa amangqamuzana amhlophe egazi aphansi alwa nezifo. Izinto ezifana nomkhuhlane kanye nomkhuhlane zingavela njalo.
• Ngenxa yokuthi i-metabolism yomzimba wakho iyasheshiswa futhi amandla avela ekudleni ashiswa ngokushesha,Ukwehlisa isisindo ngokungahlosile.

Izimpawu zokopha

Umuntu one-APL unenani eliphansi lama-platelet nezinto eziqinisa igazi ezisiza ekuqhekekeni kwegazi . Uyazi, ama-platelet yiwo asiza ekumiseni noma ekunciphiseni ukopha. Ngakho-ke, uma lawa ephansi, inkinga iqala.

Nazi ezinye izimpawu zokopha okubangelwa yi-APL:

• Ukopha kungenzeka noma kuphi emzimbeni. Isibonelo, ukuphuma kwegazi ezinsinini , ukuphuma kwegazi ekhaleni njalo, kanye, kwabesifazane, ukuphuma kwegazi okukhulu kokuya esikhathini .
• Igazi liqoqana ngaphansi kwesikhumba futhi libangele ukuklwebheka ezindaweni ezahlukene emzimbeni . Ngisho nokuklwebheka okuncane kungabangela ukuklwebheka okukhulu.
• Ngezinye izikhathi ukopha kungenzeka ngaphakathi kobuchopho (i-intracranial hemorrhage) . Uma lokhu kwenzeka, ungase ube nobunzima bokuhambisa izitho zakho zomzimba, uzwe ubuhlungu bekhanda obukhulu, futhi ungase uzwe izinguquko embonweni wakho. Lesi yisimo esiphuthumayo!
• Uma indle yakho imnyama noma inemivimbo yegazi, kungenzeka ukuthi ibangelwa ukopha ohlelweni lokugaya ukudla (ukopha kwamathumbu) .

Yini ebangela i-APL?

Lesi sifo sibangelwa izakhi zofuzo ezimbili ezilawula ukukhiqizwa kwamangqamuzana egazi ethu ahlangana ukuze akhe isakhi sofuzo esingavamile esibizwa ngokuthi i-PML-RARa . Okubalulekile, lokhu kuguqulwa kwezakhi zofuzo akuyona into oyizuza njengefa kubazali bakho . Kwenzeka ngokungahleliwe, okusho ngaphandle kwesizathu, ngesikhathi sokuphila kwakho. Ochwepheshe abasazi kahle ukuthi yini ebangela lolu shintsho lwezakhi zofuzo.

Lokhu kuguquka kubangela ukuthi amangqamuzana amhlophe egazi akhule ngokungalawuleki, esikhundleni sokukhula kahle, futhi esikhundleni salokho akhe amangqamuzana amhlophe egazi angakavuthwa (ama-promyelocyte) . Lawa mangqamuzana angakavuthwa agcwalisa umongo wethambo, aminyanise isikhala samangqamuzana egazi anempilo nama-platelet.

Yiziphi izinkinga ze-APL?

I-APL iyisimo esisongela ukuphila. Lokhu kungenxa yokuthi ukuphuma kwegazi ngokweqile okwenzekayo kungaba yingozi ngokushesha. Uma ungakwazi ukuyeka ukuphuma kwegazi ngisho noma unesilonda esincane, uma unegazi eliningi endle noma emchameni wakho lapho uya endlini yangasese, noma uma ungakwazi ukuyeka ukuphuma kwegazi ezinsinini zakho, kufanele ubone udokotela noma uye egumbini lezimo eziphuthumayo esibhedlela ngokushesha .

Khumbula: Uma unesifo sokopha esingalawuleki, ungalokothi usidebesele. Ukwelashwa okusheshayo kubalulekile.

Itholakala kanjani i-APL?

Odokotela bavame ukwenza izivivinyo eziningana ukuze bathole lesi sifo.

• Ukubalwa Kwegazi Okuphelele (i-CBC): I-APL ibangela ukwakheka kwamaseli amhlophe egazi angajwayelekile. Ukuhlolwa kwe-CBC kungabheka izinhlobo ezahlukene zamaseli egazi kanye nenani lama-platelet kusampula yegazi lakho.
• Ukugcotshwa Kwegazi Okungaphandle:Kulokhu, kuthathwa isampula yegazi bese ihlolwa ngaphansi kwe-microscope. Bese, babheka ukuthi kukhona yini ama-granule amancane amaningi ngaphakathi kwalawo maseli amhlophe egazi angakavuthwa (ama-promyelocyte), noma ukuthi kukhona yini izinto ezikhethekile ezibizwa ngokuthi ama-Auer rods . Lezi ziyisici se-APL.
• I-Bone Marrow Biopsy: Kulolu vivinyo, kuthathwa isampula encane yomnkantsha wakho bese kuhlolwa amangqamuzana awo.
• I-Flow Cytometry: Kulolu vivinyo, ososayensi bezifo bafuna amaphethini amaprotheni athile ebusweni bamaseli angajwayelekile. Lawa maphethini angaqinisekisa i-APL.
• Ukuhlolwa kwe-Polymerase Chain Reaction (PCR): Lokhu kuhlolwa kungabona ngokunembile ukuba khona kwe-gene engavamile (PML-RARa) ebangela i-APL.
• I-Cytogenetics: Lokhu kuhlola izinguquko ezithile kuma -chromosome amaseli angajwayelekile. Ukuthola lezi zinguquko kuyindlela eyinhloko yokuqinisekisa ukuxilongwa kwe-APL.

Odokotela bahlukanisa i-APL njengengozi ephansi noma ingozi ephezulu ngokusekelwe enanini lamaseli akho amhlophe egazi. Abantu abane-APL eyingozi ephezulu banamathuba amaningi okuba nomdlavuza ophindaphindayo noma ukuphinda babuye .

Iphathwa kanjani i-APL?

I-APL yelashwa ngenhlanganisela yemithi yokuhlukanisa , i-chemotherapy, kanye nokwelashwa okuqondiwe . Eqinisweni, selokhu kwatholakala lezi zindlela zokwelapha ngawo-1980, lesi sifo, esasicatshangwa ukuthi siyabulala, manje sesiyisifo esingelapheka. Lokhu kuyimpumelelo enkulu!

Imithi yokuhlukanisa amaseli ayimithi engeyona eye-chemotherapy esiza amaseli amhlophe egazi angajwayelekile, angavuthiwe ukuba ahlukane abe amaseli amhlophe egazi ajwayelekile, avuthiwe. Le mithi engeyona eye-chemotherapy, ebhalwe ngezansi, iholele ekukhululweni nasekuphulukisweni okungaphezu kwama-95%.

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

Uma udokotela wakho esola ukuthi une-APL, angase akuqalise nge -ATRA ngisho nangaphambi kokuba ezinye izivivinyo ziqinisekise lesi sifo. Lokhu kungenxa yokuthi ukuqala ukwelashwa kusenesikhathi kunganciphisa ingozi yokopha okusongela ukuphila.

Izigaba zokwelashwa kwe-APL

Ukwelashwa kuvame ukwenziwa ngezigaba ezintathu: Ukuqala, Ukuhlanganisa, kanye Nokugcina. Ukwelashwa kungahluka kuye ngezinga lengozi.

• Isigaba sokuqala (Ukungeniswa):Umgomo oyinhloko walesi sigaba ukubulala amangqamuzana e-leukemia anele ukufaka i-APL yakho ekuphumuleni . Ukuxolelwa kusho ukuthi awunazo izimpawu futhi azikho izimpawu ze-leukemia ezingatholakala ezivivinyweni. Lokhu kwenziwa ngokusebenzisa imithi engeyona eye-chemotherapy, i-chemotherapy, kanye nemithi yokwelapha eqondiwe. Phakathi nalesi sikhathi, kuzodingeka uhlale esibhedlela, ngokuvamile amasonto amane kuya kwayisithupha.
• Isigaba sokuhlanganiswa: Udokotela wakho womdlavuza angase futhi akubize ngokuthi ‘ukwelashwa kwangemva kokuxolelwa’. Lokhu kwelashwa kugcina i-APL isesimweni sokuxolelwa futhi kuyaqhubeka nokubulala noma yimaphi amangqamuzana e-leukemia asele. Lona umuthi ofanayo owawusetshenziswa esigabeni sokuqala. Lokhu kwelashwa kungahlala cishe izinyanga eziyisishiyagalombili, ngokwelashwa njalo ezinyangeni ezimbili. Ungase ube namasonto amane okwelashwa kulandelwe amasonto amane okuphumula. Lo muthi unganikezwa njengephilisi noma njengokwelashwa nge-intravenous (IV) .
• Isigaba sokunakekela: Lokhu ukwelashwa okuqhubekayo, kodwa umuthi unikezwa ngomthamo ophansi kunasezigabeni zokuqala nezokuzinzisa. Lokhu kwelashwa kokunakekela kuvame ukunikezwa cishe unyaka.

Udokotela wakho wezifo zomdlavuza angase futhi akunikeze ukwelashwa okusekelayo , njengokumpontshelwa igazi, kanye nalokhu kwelashwa.

Izinkinga zokwelashwa

Inkinga evame kakhulu, futhi engathi sína, ibizwa ngokuthi i-differentiation syndrome . Leli yiqembu lokusabela okukhulu emithini ye-APL. Lokhu kusabela kuvame ukwenzeka phakathi kwamasonto amathathu okuqala okuqala kokwelashwa. Izimpawu zingaba zincane noma zibe zimbi kakhulu. Ezinye zazo zifaka:

• Ukukhwehlela.
• Uketshezi olwengeziwe oluzungeze inhliziyo namaphaphu akho (i-pleural effusion) .
• Ukwehluleka kwezinso .
• Umfutho wegazi ophansi (hypotension) .
• Ukwehla kwamazinga omoya-mpilo egazini (i-hypoxemia) .
• Ubunzima bokuphefumula (ukuphelelwa umoya) .
• Ukuvuvukala/ ukuvuvukala kwezingalo zakho, imilenze, kanye nentamo.
• Umkhuhlane oza ngaphandle kwesizathu.
• Ukwanda kwesisindo ngaphandle kwesizathu.

Uma uba nalesi sifo sokuhlukanisa, udokotela wakho angase ayeke ukwelashwa kwakho isikhashana. Bangase basebenzise neminye imithi, njenge -hydroxyurea, ukuze banciphise inani lamaseli akho amhlophe egazi.

Yini umuntu one-APL angayilindela?

Ngokuvamile, isibikezelo salesi sifo sihle.Nakuba isimo somuntu wonke sihlukile, izifundo zibonise ukuthi phakathi kuka-90% no-95% wabantu abane-APL bayalulama. Kodwa-ke, i-APL ingabuya ngemva kokwelashwa. Phakathi kuka-5% no-10% wabantu bazophinde babuye, ngokuvamile phakathi neminyaka emithathu yokuqala ngemva kokwelashwa. Bazodinga ukwelashwa okwengeziwe noma okuhlukile.

Amanani okusinda

Ngenxa yokuthi i-APL iyisifo esingavamile, esikwaziyo ngamanani okusinda kuvela ezivivinyweni zemitholampilo ezibandakanya iziguli ze-APL ezisengozini encane nezisengozini enkulu.

Ukuhlaziywa okukodwa kukhombisa ukuthi ama-99% eziguli ze-APL ezisengozini encane ziyaphila eminyakeni emine ngemuva kokwelashwa. Okunye ukuhlaziywa kweziguli ze-APL ezisengozini enkulu kukhombisa ukuthi ama-86% ayaphila eminyakeni emihlanu ngemuva kokwelashwa. Lena imiphumela emihle ngempela!

Ngizinakekela kanjani?

Ngenxa yokuthi i-APL ingaphinde ivele, kubaluleke kakhulu ukuthi ubonane nodokotela wakho ngesikhathi (ama-aphoyintimenti okulandelela) .

Uzohlolwa njalo ngenyanga noma ezimbili ngonyaka wokuqala ngemuva kokwelashwa. Ngemva konyaka wokuqala, kuzodingeka ubone udokotela wakho cishe njalo ezinyangeni ezintathu kuya kwezine eminyakeni emibili ezayo. Ukuhlolwa okufana ne-CBC, i-PCR, kanye ne-bone marrow biopsy kungenziwa kulokhu kuvakasha.

Kufanele ngiye nini egumbini lezimo eziphuthumayo?

I-APL ingabuya futhi ngemva kokwelashwa, futhi izimpawu zingaba zimbi kakhulu ngokushesha. Uma usukhululekile ngenxa ye-APL, iya ekamelweni eliphuthumayo ngokushesha uma uhlangabezana nanoma yikuphi kwalokhu okulandelayo:

• Uma uphuma igazi ngendlela engalawuleki .
• Uma uvuvukala ngokuzumayo ngenxa yobuhlungu emilenzeni noma esiswini esingezansi .

Ekugcineni, umyalezo obalulekile

I-acute promyelocytic leukemia (APL) ingumdlavuza wegazi ongavamile owawubhekwa njengesifo esibulalayo, kodwa manje usuyisifo esingelapheka ngenxa yokwelashwa okuthuthukile.

Kodwa-ke, lesi kuseyisifo esibi kakhulu. Uma singatholakali futhi selashwe ngokushesha, singasongela impilo . Ngakho-ke, uma unezimpawu ezifana nokopha okungalawuleki, ungalokothi uzishaye indiva. Into ebaluleke kakhulu ukufuna iseluleko sezokwelapha futhi uthole ukuxilongwa ngokushesha okukhulu. Ungesabi, yiba nesibindi, ngoba manje kunezindlela zokwelapha ezinhle zalokhu!

I- Leukemia, i-APL, Umdlavuza Wegazi, Umdlavuza Wegazi, Umongo Wethambo, I-Anemia, i-ATRA, Ukwelashwa Ngemithi Yekhemo, I-Acute Promyelocytic Leukemia, i-M3-Leukemia, i-PML-RARa, Ukopha