Dɛn ne Castleman Yare? Momma yɛnka ho asɛm tiawa!

So woahyɛ no nsow pɛn sɛ wunya saa akuru nketenkete yi wɔ wo kɔn anaa wo nsateaa so? Ɛtɔ da bi a ɛba bere a woanya atiridii anaa atiridiinini no. Nanso, eyi nyɛ biribi a ɛyɛ mmerɛw te sɛ ɛno ho asɛm. Eyi yɛ tebea a ɛntaa nsi nanso ɛho hia a ɛsɛ sɛ yɛma yɛn ani da hɔ. Yɛfrɛ no Castleman Yare .

Sɛ yɛbɛka no tiawa a, Castleman Yare yɛ tebea a yɛn nipadua mu nipadua mu ahoɔden a ɛko tia nyarewa no yɛ adwuma boro so . Fa no sɛ, sɛ ɔyare mmoawa bi a ɛde nyarewa ba yɛn nipadua mu a, yɛn nipadua mu tumi a ɛko tia nyarewa no ko tia no te sɛ ɔsraani a ɔrekɔ ɔko. Sɛ wɔsa yare no a, saa ɔsraani no san yɛ komm bio. Nanso wɔ obi a ɔwɔ Castleman Yare mu no, saa nipadua no mu tumi a ɛko tia nyarewa yi ‘yɛ adwuma’ bere nyinaa. Eyi ma ɔfe kyɛ wɔ nipadua no mu, na ebetumi asɛe yɛn akwaa ahorow.

Saa bere yi, yɛn lymph nodes – saa akuru nketenkete no ne nea yɛfrɛ no – yɛ adwuma te sɛ filter. Wɔde mmoawa ne nkwammoaa a ɛko tia nyarewa no nyinaa fa mu yiyi mu. Enti, esiane sɛ nipadua no mu tumi a ɛko tia nyarewa no reyɛ adwuma bere nyinaa nti, saa ntini a ɛma mogya tu yi nso reyɛ adwuma boro so. Afei nkwammoaa a ɛwɔ mu no nyin dodo, na ntini a ɛma mogya tu no hohoro. Ɛnyɛ saa ɔfe yi nko, na mmom wɔ Castleman Yare mu no, nsakrae mpo ba saa ntini ahorow yi ntini mu.

Eyi yɛ yare a ɛntaa nsi koraa . Sɛ nhwɛso no, wɔ United States no, afe biara nnipa foforo 4,300 kosi 5,200 pɛ na wohu. Enti nnuruyɛfo da so ara resua eyi ho ade pii. Nanso, sɛ wohu sɛ wowɔ saa yare yi a, wo nsa bɛka ayaresa pɔtee bi a ɛfa wo ho.

Dɛn ne Castleman Yare ahorow atitiriw?

Saa yare yi ahorow atitiriw abien na ɛwɔ hɔ.

Castleman Yare a Ɛma Wɔn Ho Nyinaa (UCD) .

Unicentric Castleman disease (UCD) yɛ bere a ntini biako anaa kakraa bi pɛ a ɛwɔ wo nipadua no fã biako no trɛw . Susuw wo kɔn nkutoo ho, anaasɛ wo nsateaa kɛkɛ. Castleman ayarefo bɛyɛ nkyem anan mu abiɛsa hyɛ saa kuw yi mu.

Castleman Yare a Ɛwɔ Nneɛma pii (MCD) .

Multicentric Castleman disease (MCD) yɛ tebea a ɛma ntini a ɛma mogya tu no ahonhon wɔ nipadua no mmeae pii , sen sɛ ɛbɛyɛ biako pɛ. Sɛ yɛbɛka no pɛpɛɛpɛ a, nnuruyɛfo kyekyɛ MCD a ɛte sɛɛ mu bio ma ɛyɛ:

• MCD a ɛne ANOHYƐE wɔ abusuabɔ: ANOM yɛ mogya mu yare a ɛntaa nsi koraa. Ɛtɔ da bi a, ANOM tumi ka MCD ho.
• MCD a ɛne HHV-8 wɔ abusuabɔ: Saa ɔkwan yi fi ɔyare mmoawa bi a wɔfrɛ no Human Herpes Virus-8 (HHV-8) a obi nya mu . Sɛ wowɔ HIV anaasɛ wo nipadua no tumi ko tia nyarewa a, ɛda adi sɛ wubenya MCD a ɛte sɛɛ.
• MCD a wonnim nea ɛde ba (Idiopathic MCD - iMCD):Eyi ne MCD a ɛtaa ba. ‘Idiopathic’ kyerɛ sɛ wonnim nea ɛde ba ankasa.

Seesei saa nea wonnim nea ɛde ba yi iMCD nso wɔ afã nketewa abiɛsa:

• iMCD a ɛne TAFRO yare no wɔ abusuabɔ: Ɛtɔ mmere bi a, tebea bi a wɔfrɛ no TAFRO yare no betumi aka iMCD ho. Wonyaa edin TAFRO no fii nkyerɛwde a edi kan a ɛkyerɛ ne sɛnkyerɛnne ahorow no mu. Eyinom ne: Thrombocytopenia (mogya mu mogya mu nkwammoaa a ɛba fam), Anasarca (nipadua a nsu hohoro), Atiridii , Adwene (asaabo) a entumi nyɛ adwuma yiye, ne Organomegaly (akisikuru anaa mmerɛbo a ɛtrɛw).
• iMCD a Idiopathic Plasmacytic Lymphadenopathy (iMCD-IPL) ka ho: Wɔ saa ɔkwan ketewa yi mu no, mogya nkwammoaa a ɛma mogya tu no betumi akɔ soro. Ebia wo mogya mu nkwammoaa fitaa no nso bɛma nnuru a ɛko tia nyarewa pii.
• iMCD a wɔankyerɛ ɔkwan foforo so (iMCD-NOS): Wonnim nea ɛde saa ɔkwan yi ba ankasa, a wɔfrɛ no iMCD-NOS, nso, na ɛne TAFRO nni abusuabɔ.

Dɛn ne Castleman Yare no ho sɛnkyerɛnne?

Castleman yare no ho sɛnkyerɛnne gu ahorow gyina nea wowɔ no so. Mpɛn pii no, sɛ wowɔ unicentric Castleman disease (UCD) a, ebia worennya sɛnkyerɛnne titiriw biara. Ebia ntini a ɛho ahonhon nkutoo ne sɛnkyerɛnne a wubehu. Nanso, ɛtɔ mmere bi a, sɛ ntini bi a ahonhon mia akwaa foforo a ɛbɛn hɔ so a, yare no ho sɛnkyerɛnne betumi aba.

Nanso, sɛ wowɔ MCD a, ɛda adi sɛ sɛnkyerɛnne ahorow no bɛda adi kɛse. Wɔ ntini a ɛho ahonhon akyi no, ebia wubehu sɛnkyerɛnne ahorow te sɛ:

• Huraeɛ
• Ɔbrɛ (eyi betumi ayɛ mogya a ɛtɔ kɔ nipadua mu ho sɛnkyerɛnne) .
• Fifiri a ɛba anadwo
• Akisikuru ne ɔfe
• Mu duru a wɔtew so a ntease biara nnim
• Wo nan, wo nansoaa, anaa wo yafunu mu a ɛho ahonhon
• Akisikuru a ayɛ kɛse (splenomegaly) anaa mmerɛbo a ayɛ kɛse (hepatomegaly) .
• Nsa ne anan a ɛyɛ mmerɛw (peripheral neuropathy) .

Dɛn na ɛde Castleman Yare no ba?

Wonnya nhuu nea ɛde Castleman yare a ɛwɔ baabiara (UCD) ba ankasa, nanso wɔahu sɛ ɔyare mmoawa bi a wɔfrɛ no HHV-8 a wɔde ba no na ɛma obi nya Castleman yare a ɛwɔ mmeae pii (MCD).

Nyansahufo da so ara reyɛ nneɛma afoforo a ebetumi de UCD ne iMCD aba no mu. Sɛ nhwɛso no, wɔrehwɛ nyarewa afoforo a ɛnyɛ HHV-8, awosu mu nkwaadɔm mu nsakrae , ne sɛnea nipadua no tumi ko tia nyarewa .

Hena na ɔwɔ asiane kɛse sɛ obenya saa yare yi?

Wɔnnya nhuu asiane pɔtee bi a ɛde UCD anaa iMCD ba. Nanso, sɛ wowɔ HIV anaa tebea foforo a ɛma wo nipadua mu tumi a ɛko tia nyarewa no yɛ mmerɛw a, asiane kɛse wom sɛ wubenya MCD a ɛne HHV-8 wɔ abusuabɔ.

Castleman yare betumi aba wɔ mfe biara mu, nanso wɔtaa hu wɔ nnipa a wɔadi fi mfe 30 kosi 60 so .

Dɛn ne nsɛnnennen a ebetumi afi Castleman Yare no mu aba?

Castleman yare no ma asiane a ɛwɔ hɔ sɛ wubenya kokoram, titiriw lymphoma .

Afei nso, ɛwom sɛ ɛntaa nsi koraa de, nanso nnipa a wɔwɔ UCD no wɔ asiane mu sɛ wobenya tebea bi a emu yɛ den a wɔfrɛ no Paraneoplastic Pemphigus (PNP) . MCD nso ma asiane a ɛwɔ hɔ sɛ wubenya nyarewa a ebetumi asɛe w’akwaa ahorow no yɛ kɛse. Saa ɔsɛe yi betumi de nkwa ato asiane mu sɛ wɔansa no yiye a.

Mma ɛnhaw wo, wo duruyɛfo bɛhwɛ wo tebea no so bere nyinaa na waboa wo ma woasiw nsɛnnennen a ɛtete saa ano bere biara a ɛbɛyɛ yiye.

Ɔkwan bɛn so na nnuruyɛfo hu Castleman Yare no?

Castleman Yare no ho sɛnkyerɛnne betumi ayɛ te sɛ nyarewa afoforo a ɛtaa ba te sɛ atiridii anaa atiridiinini. Enti wo duruyɛfo bedi kan apow saa tebea horow no. Afei, wɔbɛyɛ nhwehwɛmu de ahu Castleman Yare no na wɔahu ɔkwan a wɔfa so yɛ no.

Sɔhwɛ ne akwan a wɔfa so yɛ no betumi ayɛ:

• Lab nhwehwɛmu: Wo duruyɛfo bɛhwɛ wo mogya mu nkwammoaa dodow a ɛnyɛ ne kwan so ne Castleman yare no ho sɛnkyerɛnne afoforo a ɛyɛ nketenkete anaa. Wobetumi nso ahyɛ sɛ wɔnyɛ HIV nhwehwɛmu . Sɛ wowɔ MCD a ɛfa HHV-8 ho a, ebia wo HIV nhwehwɛmu no yɛ papa.
• Mfoniniyɛ mu nhwehwɛmu: Nhwehwɛmu te sɛ CT scan ne PET scan betumi aboa ma woahu ntini a ɛho ahonhon wɔ wo nipadua mu, na afei nso ahwɛ sɛ ebia Castleman yare no ho sɛnkyerɛnne afoforo te sɛ mmerɛbo anaa akyi berɛmo a ayɛ kɛse anaa.
• Lymph node biopsy: Ɔkwan biako pɛ a wobɛfa so ahu sɛ wowɔ Castleman yare no yiye ne sɛ wobɛyɛ biopsy. Nea ɛka eyi ho ne sɛ wobegye ntini ketewaa bi afi ntini a ɛma mogya tu mu na wɔde afiri a wɔde hwɛ nneɛma nketenkete ahwɛ mu. Eyi na ɛbɛkyerɛ sɛ yare no ho sɛnkyerɛnne bi wɔ hɔ anaa.

Ɔkwan bɛn so na wɔsa Castleman Yare no?

Ɛsono sɛnea wɔsa Castleman yare no, na egyina nea wowɔ no so.

Castleman Yare a Ɛyɛ Unicentric (UCD) a Wɔsa .

Ayaresa titiriw a wɔde sa UCD ne sɛ wɔbɛyɛ oprehyɛn ayi ntini a ɛka ho no afi hɔ . Ɛtɔ mmere bi a ebia wubehia radiation therapy anaa immunotherapy ansa na wɔayɛ wo oprehyɛn. Saa ayaresa ahorow yi ma ntini a enyin wɔ ntini a ɛma mogya tu no mu no so tew, na ɛma ɛyɛ mmerɛw sɛ wobeyi afi hɔ.

Nanso sɛ nnuruyɛfo a wɔyɛ oprehyɛn ntumi nyi ntini a ɛka wo no, na wunni yare no ho sɛnkyerɛnne biara a, ebia wo duruyɛfo bɛhwɛ so mmom sen sɛ ɔbɛsa. Sɛ wowɔ sɛnkyerɛnne ahorow na wuntumi nyɛ oprehyɛn a, ebia wɔbɛkamfo MCD ayaresa akyerɛ wo.

Castleman yare a ɛwɔ mmeae pii (MCD) a wɔsa .

MCD yɛ den kakra sɛ wɔbɛsa sen UCD. Esiane sɛ ɛtrɛw wɔ nipadua no mu nyinaa nti, wɔntaa mfa oprehyɛn anaa mframa a wɔde sa yare nni dwuma. Mmom no, ayaresa no gyina sɛ ebia wowɔ HHV-8 yare no ne sɛnea yare no mu yɛ den no so.

Ebia wo duruyɛfo de ayaresa ahorow te sɛ:

• Corticosteroids: Saa nnuru yi boa ma ɔfe no so tew na ɛma ahotɔ fi yare no ho sɛnkyerɛnne ahorow ho.
• Nnuru a wɔde sa yare: Nnuru a wɔde sa yare no hwɛ nkwammoaa a enyin dodo wɔ wo ntini mu no so. Rituximab yɛ aduru a wɔtaa kyerɛw ma MCD a ɛne HHV-8 wɔ abusuabɔ.
• Nkwammoaa a ɛko tia nyarewa ho ayaresa: Nea ɛka ayaresa yi ho ne sɛ wɔde nneɛma te sɛ monoclonal antibodies bedi dwuma . Eyinom boa ma wo nipadua no tumi a ɛko tia nyarewa no dwo. Siltuximab (Sylvant®) nkutoo ne ayaresa a U.S. Aduan ne Nnuru Dwumadibea (FDA) apene so ama iMCD.
• Nnuru a ekum ɔyare mmoawa: Sɛ wowɔ MCD a ɛfa HHV-8 ho a, ebia wubehia nnuru a ekum ɔyare mmoawa de asa HIV anaa HHV-8 yare mmoawa.

Bere bɛn na ɛsɛ sɛ mekɔ oduruyɛfo nkyɛn?

Sɛ wuhu sɛ akisikuru anaa wo kɔn, wo nsateaa, anaa wo srɛ mu anyin a, kɔ oduruyɛfo nkyɛn ntɛm ara . Afei nso, sɛ wowɔ Castleman Yare ho sɛnkyerɛnne afoforo a yɛaka ho asɛm wɔ atifi hɔ no bi a ɛnkɔ yiye wɔ adapɛn kakraa bi akyi a, ka ho asɛm nso kyerɛ wo duruyɛfo.

Bere tenten ahe na wubetumi ne yare yi atra ase?

Wɔ nnipa dodow no ara a wɔwɔ UCD fam no, nkɔmhyɛ, anaa nea wɔhwɛ no ye yiye. Sɛ woyi ntini a ɛka ho no fi hɔ a, ebetumi asa yare no. Sɛ wɔsa yare a, mpɛn pii no, UCD nnya nkwa nna so nkɛntɛnso.

Nanso sɛnea nnipa a wɔwɔ MCD hwɛ kwan no gyina MCD ko a wɔwɔ ne sɛnea yare no mu yɛ den so. Wɔ nnipa binom fam no, yare no ho sɛnkyerɛnne betumi aba mpofirim na ɛde wɔn nkwa ato asiane mu. Wɔ afoforo fam no, yare no ho sɛnkyerɛnne betumi atra hɔ akyɛ. Nnipa a wɔahu sɛ wɔwɔ MCD no mu 65% kosi 75% da so te ase wɔ mfe anum akyi . Sɛ wɔde ayaresa foforo di dwuma a, anidaso wɔ hɔ sɛ tebea yi bɛtu mpɔn.

Mmuae a ɛyɛ mmerɛw biara nni hɔ bere a ɛfa Castleman yare ho no. Wo suahu begyina Castleman yare ko a wowɔ so. Tebea foforo te sɛ HIV, anaa tebea horow a ɛfa ho, te sɛ TAFRO ne POEMS nso betumi aka wo suahu. Wɔ tebea horow bi mu no, ebia oprehyɛn ne hwɛ a wɔbɛhwɛ no yiye nkutoo ne nea ehia. Anaasɛ, ebia wubehia ayaresa ahorow wɔ w’asetra nyinaa mu na ama woatumi asiw nsɛnnennen ano. Bisa wo duruyɛfo sɛnea wo yare no bɛka w’ayaresa ne w’adwene.

Enti, nneɛma bɛn na ɛho hia paa a ɛsɛ sɛ yɛkae fi eyi mu?

• Castleman Yare yɛ nipadua mu ahoɔden a ɛko tia nyarewa a ɛntaa nsi nanso ebetumi ayɛ aniberesɛm a yɛn nipadua no mu tumi a ɛko tia nyarewa no yɛ adwuma boro so.
• Nneɛma atitiriw abien na ɛwɔ hɔ: UCD ne MCD. Wɔ UCD mu no, ntini a ɛma mogya tu no hohoro wɔ baabi biako, bere a wɔ MCD mu no, ɛhohoro wɔ mmeae pii.
• Ɛsono sɛnea yare no ho sɛnkyerɛnne. Ɛtɔ mmere bi a, ebia yare no ho sɛnkyerɛnne biara nni hɔ koraa, na ɛyɛ akisikuru a ahonhon ara kwa. Anaasɛ ebia sɛnkyerɛnne ahorow te sɛ atiridiinini, ɔbrɛ, ne mu duru a ɛso tew bɛba.
• Ɛnyɛ bere nyinaa na nea ɛde ba no da adi pefee, nanso wɔde HHV-8 mmoawa no abata MCD ho. Nnipa a wɔwɔ HIV no wɔ asiane kɛse mu.
• Mpɛn pii no, sɛ wohu ntɛm na wɔsa no yare yiye a, ebetumi ama nneɛma pa afi mu aba. Enti sɛ wuhu sɛ akisikuru, ɔfe, anaa sɛnkyerɛnne afoforo a ɛyɛ soronko wɔ wo nipadua so a, hwehwɛ aduruyɛfo afotu ntɛm ara. Ade a ɛho hia sen biara ne sɛ wobɛkɔ so abɔ amanneɛ na woanbɔ hu.

` Castleman Yare, Ntini a Ɛma Ntini, Ntini a Ɛko Tia Nyarewa, UCD, MCD, HHV-8, Nsɛnkyerɛnne, Ayaresa