So mogya mu kokoram yɛ tebea a ɛyɛ nwonwa a nnuru na ɛde ba? Momma yɛnka Differentiation Syndrome ho asɛm!

Sɛ wɔresa wo anaa obi a wunim no yare bi te sɛ mogya mu kokoram a, ebia wubenya ɔhaw bi a wonhwɛ kwan. Ɛnnɛ, yɛbɛka nneɛma a anibere wom a ebetumi aba wɔ nnuru a wɔde di dwuma ho, titiriw nea wɔde ma mogya mu kokoram ahorow abien ho asɛm. Nnuruyɛfo frɛ eyi Differentiation Syndrome . Esiane sɛ ebetumi ayɛ aniberesɛm koraa nti, nnuruyɛfo taa fi ase sa yare no ho sɛnkyerɛnne ahorow no ansa na wɔatumi asi so dua denneennen, na ɛno betumi atew asiane a ɛwɔ hɔ sɛ wobenya ɔhaw ahorow a ɛde nkwa to asiane mu no so.

Dɛn ne Nsonsonoe Ho Ayaresa?

Sɛ yɛbɛka no tiawa a, Differentiation Syndrome yɛ nneyɛe a emu yɛ den kuw bi a nipadua no nya wɔ nnuru bi a wɔde sa mogya mu kokoram ahorow abien, a ɛne Acute Promyelocytic Leukemia (APL) ne Acute Myeloid Leukemia (AML) . Ɛte sɛ nea nkwammoaa a ɛwɔ yɛn nipadua mu no bi "bɔ dam" wɔ saa nnuru yi ho na wofi ase gyae nneɛma pii prɛko pɛ. Eyi na ɛde sɛnkyerɛnne ahorow no mu biara ba.

Fa no sɛ w’adamfo bi ahu sɛ ɔwɔ APL na ofi ase sa yare. Wɔ nna kakraa bi mu no, ne ho tɔ no ara kwa, ɛyɛ den ma no sɛ ɔbɛhome, na ne ho ahonhon kakra. Saa bere no, nnuruyɛfo susuw sɛ eyi yɛ Differentiation Syndrome.

Dɛn ne saa Nsonsonoe Nneɛma yi?

Nnuru a ɛma nsonsonoe yɛ nnuru bi a nnuruyɛfo de sa APL. APL yɛ nea ɛte nka kɛse sen AML ahorow afoforo wɔ nnuru a ɛma nkwammoaa nyin, anaasɛ ɛyɛ soronko ho . Saa nnuru yi boa ma kokoram nkwammoaa (blast cell stage) no nyin bɛyɛ mogya mu nkwammoaa fitaa a ɛteɛ na ɛte apɔw. Ɛno nti na nnuruyɛfo de nnuru a wɔde kyerɛ nsonsonoe yi di dwuma sen sɛ wɔbɛma wɔn nnuru a wɔde sa yare tẽẽ no.

Te sɛ kokoram nnuru afoforo no, saa nnuru yi boa ma kokoram nkwammoaa nyin anaasɛ ɛma ɛyɛ brɛoo. Nnuruyɛfo betumi de nnuru a ɛma nsonsonoe yi nkutoo adi dwuma, de afra wɔn ho wɔn ho, anaasɛ wɔde aka nnuru a wɔde sa yare ho. Wobetumi asa APL yiye. Nanso, nnipa binom a wɔwɔ APL betumi anya nnuru yi mu nsunsuanso bɔne a emu yɛ den.

Nnuru bɛn na ebetumi ama obi anya Differentiation Syndrome?

Saa tebea yi betumi afi nsonsonoe a wɔde ma APL no mu aba. Nhwɛso ahorow bi ne:

• All-trans-retinoic acid (ATRA) yɛ vitamin A bi.
• Arsenic Trioxide (ATO) yɛ aduru bi a wɔde yɛ arsenic.

Afei nso, wɔ nnipa binom a wɔwɔ AML fam no, nnuruyɛfoIsocitrate Dehydrogenase (IDH) inhibitors yɛ nnuru a wɔde sa yare a wɔde di dwuma. Saa nnuru a esiw IDH ano (ivosidenib) ne (enasidenib) nso betumi de Differentiation Syndrome aba. Ɛnyɛ ɛno nko, na mmom nnuru a esiw FLT3 ano (e.g. (midostaurin) , (gilteritinib) ) nso betumi de tebea yi aba.

Henanom na ebia wobehia nnuru a esiw IDH ano?

• Nnipa a wɔn kokoram asan aba bio.
• Nnipa a wɔwɔ kokoram a ɛyɛ den sɛ wɔbɛsa.
• Nnipa a wɔwɔ awosu mu nkwaadɔm mu nsakrae pɔtee bi (IDH1 anaa IDH2 mutation).
• Wɔn a wontumi nnye nnuru a wɔde sa yare a wɔtaa de di dwuma no ntom.

Hena na ɛda adi sɛ obenya Differentiation Syndrome kɛse?

Nnipa pii a wɔwɔ APL no mogya mu nkwammoaa fitaa dodow (WBC) sua . Nanso, wɔn a wɔn WBC dodow kɔ soro no wɔ asiane kɛse mu sɛ wobenya Differentiation Syndrome. Wɔ tebea horow yi mu no, nnuruyɛfo taa kyerɛw aduru bi a wɔfrɛ no Prednisone anaa Dexamethasone na ama asiane a ɛwɔ hɔ sɛ wobenya saa yare yi so atew.

Differentiation Syndrome abu so dɛn?

Eyi yɛ tebea a ɛntaa nsi koraa ankasa . AML ne mogya mu kokoram a ɛtaa ba mpanyimfo mu, ɛwom sɛ ɛyɛ kokoram nyinaa mu bɛyɛ 1% de. Wɔ United States no, APL ba nnipa bɛyɛ 250,000 biara mu biako mu.

Efi wɔn a wɔwɔ AML ne APL hɔ:

• APL ayarefoɔ a wɔanya ATRA ne ATO nnuru no mu bɛyɛ 25% anya Differentiation Syndrome.
• Wɔabɔ saa tebea yi ho amanneɛ wɔ AML ayarefo a wɔnom nnuru a esiw IDH ano no mu 14% kosi 19% mu.

Dɛn na ɛde Nsonsonoe a Ɛma Nsonsonoe yi ba?

Nhwehwɛmufo da so ara nnim nea enti a nnipa binom yɛ wɔn ade wɔ APL ayaresa ho saa kwan yi so no ankasa. Nhwehwɛmu ahorow a edi kan kyerɛ sɛ kokoram nnuru no ma mogya mu kokoram nkwammoaa no ma nipadua no mu nkwammoaa a ɛko tia nyarewa no mu nnuru pii fi adi mpofirim. Wɔfrɛ eyi cytokine release syndrome . Saa ɔfe a wontumi nni so yi de yare no ho sɛnkyerɛnne ahorow ba, efi nea emu nyɛ den so kosi nea emu yɛ den so.

Dɛn ne Differentiation Syndrome ho sɛnkyerɛnne ahorow?

Mpɛn pii no, obi a ɔwɔ APL fi ase nya Differentiation Syndrome ho sɛnkyerɛnne wɔ dapɛn biako anaa abien akyi bere a wafi ATRA ne/anaasɛ Arsenic Trioxide ase no. Nanso, sɛ worenom IDH inhibitor aduru a wɔde sa AML a, ebia ebegye bere tenten kakra ansa na sɛnkyerɛnne ahorow no ada adi.Ebetumi akɔ (saa sɛnkyerɛnne ahorow yi betumi ada adi wɔ baabiara fi da koro kosi asram anum kosi asia wɔ bere a wɔafi ase de aduru no adi dwuma akyi).

Eyinom ne sɛnkyerɛnne ahorow a wɔtaa hu:

• Ɛwa .
• Nsu foforo a ɛbɛboaboa ano atwa wo koma ne w’ahurututu ho ahyia (Pleural Effusion) .
• Adwene mu Nneɛma a Ɛnyɛ Yiye .
• Mogya mmoroso a ɛba fam (Hypotension) .
• Oxygen dodow a ɛba fam wɔ mogya mu (Hypoxemia) .
• Ɔhome a ɛyɛ den (Dyspnea) .
• Nsa, anan, ne kɔn a ɛho ahonhon (ɔfe) .
• Atiridiinini a ntease nnim.
• Mu duru a ɛkɔ soro a ntease biara nnim.

Nea ɛho hia: Sɛ wowɔ saa sɛnkyerɛnne ahorow yi mu biako anaa nea ɛboro saa a, ɛnkyerɛ sɛ akyinnye biara nni ho sɛ wowɔ Differentiation Syndrome. Nanso, ɛho hia sɛ wobɔ wo duruyɛfo amanneɛ ntɛm ara sɛ wuhu saa sɛnkyerɛnne ahorow yi bi a.

Dɛn ne nsɛnnennen a ebetumi afi Differentiation Syndrome mu aba?

Ɛtɔ mmere bi a, saa tebea yi betumi de tebea horow a emu yɛ den paa aba. Nhwɛsoɔ:

• Koma a Ɛyɛ Fɛ .
• Adwene mu Nneɛma a Ɛnyɛ Yiye .
• Ahurututu a Ɛnyɛ Yiye .
• Mogya a ɛtɔ fi ahurututu mu (Pulmonary Hemorrhage) .
• Ahurututu mu yare .
• Sepsis ( ɔyare mmoawa a emu yɛ den a ɛtrɛw kɔ nipadua no mu nyinaa).

Sɛ wuhu saa sɛnkyerɛnne ahorow yi bi a, frɛ wo duruyɛfo ntɛm ara . Sɛ ɛyɛ den ma wo sɛ wobɛhome anaasɛ wo koko yɛ wo yaw a, frɛ 911 (anaa wo mpɔtam hɔ nɔma a egye ntɛmpɛ) ntɛm ara . Wɔtaa sa nnipa a wɔwɔ APL yare wɔ ayaresabea kosi sɛ asiane a ɛwɔ hɔ sɛ wobenya differentiation syndrome no bɛtwam.

Ɔkwan bɛn so na wohu Differentiation Syndrome?

Ebetumi ayɛ den sɛ wobehu tebea yi wɔ obi a ɔwɔ APL mu, efisɛ tebea afoforo pii wɔ hɔ a ebetumi de sɛnkyerɛnne a ɛte saa ara aba. Wɔ tebea horow a emu yɛ den mu no, nnuruyɛfo betumi afi ase asa yare no ho sɛnkyerɛnne no bere a wosusuw sɛ Differentiation Syndrome bi wɔ hɔ ara pɛ, ansa na wɔatumi asi so dua mpo sɛ ɛyɛ nea ɛfata.

Wo duruyɛfo bɛkamfo akyerɛ sɛ wɔnyɛ nhwehwɛmu pii de si tebea no so dua na wɔayi aduruyɛ mu tebea afoforo afi hɔ. Ebia eyinom bi ne:

• Mogya a Wɔkan a Edi Mu (CBC) .
• Koko so X-ray a wɔyɛ .
• Echocardiogram (sɔhwɛ a wɔde hwɛ sɛnea koma no yɛ adwuma).
• Chest CT scan a wɔyɛ no .
• Bronchoscopy (sɔhwɛ a wɔde mfoninitwa afiri di dwuma de hwɛ ahurututu no mu).
• Mogya mu nhwehwɛmu afoforo (Bloodwork).

So aduru bi wɔ hɔ a wɔde sa Differentiation Syndrome?

Mpɛn pii no, nnipa a wɔwɔ acute promyelocytic leukemia (APL) betumi anya ahoɔden koraa afi differentiation syndrome mu sɛ wonya ayaresa ntɛm na ɛfata a . Mprempren APL yɛ yare a wotumi sa no kɛse. Enti biribiara nni hɔ a ɛsɛ sɛ yesuro.

Ɔkwan bɛn so na wɔsa Differentiation Syndrome?

Nnuruyɛfo taa de aduru bi a wɔfrɛ no corticosteroid anaa glucocorticoid te sɛ dexamethasone sa nsonsonoe a ɛwɔ nipadua no mu ho sɛnkyerɛnne ahorow. Wɔ tebea horow a emu yɛ den mu no, ebia wo duruyɛfo nso bɛkamfo akyerɛ sɛ gyae wo kokoram ayaresa no bere tiaa bi kosi sɛ wo yare no ho sɛnkyerɛnne no bɛbrɛ ase.

Wɔsa nnipa dodow no ara a wɔwɔ APL yare wɔ ayaresabea kosi sɛ asiane a ɛwɔ hɔ sɛ wobenya differentiation syndrome no bɛtwam. Sɛ woyɛ ɔyarefo a wɔda ayaresabea na wosusuw sɛ nsonsonoe a ɛda wo ntam no adi a, ebia wo nnuruyɛfo besi gyinae sɛ wɔbɛma wo akɔ ayaresabea anadwo ma wɔahwɛ wo anaa wɔahwɛ wo. W’aduruyɛfo kuw no bɛboa ma wɔasi wo yare no so dua na wɔasa tebea biara a ɛde wo sɛnkyerɛnne ahorow no reba no yiye.

Dɛn na metumi ayɛ na ama asiane a ɛwɔ hɔ sɛ menya Differentiation Syndrome no so atew?

Biribiara nni hɔ ankasa a wubetumi ayɛ de atew asiane a ɛwɔ hɔ sɛ wubenya saa tebea yi so. Sɛ worenom nnuru anaa kokoram nnuru afoforo a, ka sɛnkyerɛnne biara a wubehu kyerɛ wo duruyɛfo ntɛm ara . Afei nso, hu sɛnkyerɛnne ahorow a ɛsɛ sɛ wohwɛ yiye (sɛnea yɛaka ho asɛm wɔ atifi hɔ no) na sɛ wowɔ biribi a ɛhaw wo a, wo ne wo duruyɛfo nkasa. Eyi ne ade a ɛho hia sen biara.

Dɛn ne Differentiation Syndrome ho anidaso?

Mpɛn pii no, adwene a ɛwɔ hɔ ma differentiation syndrome no ye ma nnipa dodow no ara. Mpɛn pii no, wubetumi atoa wo ayaresa a wode ko tia kokoram no so, bere a wode steroid resa no anaasɛ bere a wode steroid resa wo no. W’aduruyɛfo kuw no ne wo bɛkasa afa ayaresa a wubetumi apaw ho.

Bere bɛn na ɛsɛ sɛ wohwehwɛ nnuruyɛfo afotu wɔ Differentiation Syndrome ho?

Sɛ wuhu nsonsonoe ho sɛnkyerɛnne biara bere a woreyɛ kokoram ayaresa a, frɛ wo duruyɛfo ntɛm ara . Ebia ɛbɛyɛ den ama wo anaa wo duruyɛfo sɛ wubehu sɛ ebia kokoram ayaresa no na ɛde wo sɛnkyerɛnne ahorow no ba anaasɛ tebea foforo bi. Ayaresa a wɔde sa yare ntɛm no betumi aboa ma yare no ho sɛnkyerɛnne ahorow no afi hɔ na atew asiane a ɛwɔ hɔ sɛ ɔhaw ahorow bɛba no so.

Kokoram ayaresa betumi ayɛ den. Nanso, ebetumi ayɛ abasamtu sɛ wubehu sɛ ayaresa no ankasa wɔ ɔhaw ahorow. Differentiation Syndrome yɛ nneyɛe kuw bi a ebetumi de nkwa to asiane mu wɔ mogya mu kokoram ayaresa ho. Nanso, sɛ wohu na wɔsa no ntɛm a, wobetumi asa . Esiane sɛ yare no ho sɛnkyerɛnne betumi asɛe ntɛmntɛm nti, ebia nnuruyɛfo befi ase asa wo yare ansa na wo nhwehwɛmu aba no asi so dua sɛ wowɔ Differentiation Syndrome. Ayaresa a wɔde sa wo ntɛm no betumi aboa ma wo yare no ho sɛnkyerɛnne no afi hɔ na ama woasan akɔ ɔkwan a ɛkɔ wo ho so no so.

Nkrasɛm a Wɔde Kɔ Fie

• Differentiation Syndrome yɛ adeyɛ a emu yɛ den a ebetumi aba esiane nnuru bi a wɔde sa mogya mu kokoram te sɛ APL ne AML nti.
• Nsɛnkyerɛnne te sɛ atiridiinini, ahome a ɛyɛ den, ne nipadua a ɛhoa betumi aba.
• Sɛ wuhu saa sɛnkyerɛnne ahorow yi a, ɛho hia sɛ wobɔ wo duruyɛfo amanneɛ ntɛm ara .
• Sɛ wohu ntɛm na wɔde nnuru te sɛ Dexamethasone sa a, mpɛn pii no wobetumi asa.
• Mma wo ho nnyɛ wo hu bere a wote eyi no. Wo nnuruyɛfo kuw no bɛboa wo. Ade a ɛho hia sen biara ne sɛ wobɛma wɔahu nsakrae biara, sɛnkyerɛnne biara.

` Nsonsonoe a ɛba, mogya mu kokoram, kokoram ayaresa, nea efi mu ba, APL, AML, ATRA, sɛnkyerɛnne ahorow