Ngaba nawe uyafuna ukwazi ngeHairy Cell Leukemia? Masithethe!

Ngaba uziva udiniwe ngalo lonke ixesha? Okanye ngaba unesifo somkhuhlane esingapheliyo kunye nosulelo? Ngamanye amaxesha ezi zinto zithathwa njengeziqhelekileyo, kodwa azinakuba luphawu lwento eyahlukileyo. Namhlanje siza kuthetha ngomhlaza wegazi ongaqhelekanga kodwa obaluleke kakhulu ekufuneka uwuqaphele. Ubizwa ngokuba yi "Hairy Cell Leukemia".

Yintoni le "Hairy Cell Leukemia"?

Ngamafutshane, olu luhlobo lomhlaza oluqala egazini. Sinenxalenye yomzimba wethu ebizwa ngokuba yi-bone marrow. Kukuloo marrow apho kwenziwa khona iintlobo zeeseli egazini lethu. Kula marrow, ii-B-cells (ezikwabizwa ngokuba yi-'B lymphocytes') luhlobo lweeseli ezimhlophe zegazi. Umsebenzi wazo ophambili kukulwa neentsholongwane ezingena emizimbeni yethu zize zisikhusele kwizifo.

Ngoko ke, kule meko ibizwa ngokuba yi-'Hairy Cell Leukemia', okwenzekayo kukuba umongo wakho wethambo uvelisa ii-B-cells ezingaqhelekanga . Xa ujonga ezi seli ezingaqhelekanga phantsi kwe-microscope, ungabona ukuba zineenwele ezincinci eziphuma kuzo. Yiyo loo nto esi sifo sibizwa ngokuba yi-'hairy cell leukemia' ngenxa yembonakalo 'yeenwele'. Sichongwa ziingcali zezifo ezihlola iiseli phantsi kwe-microscope.

Kutheni oku kusenzeka? Kwenzeka ntoni emzimbeni?

Nangona kungaziwa ukuba kutheni oku kusenzeka, kukho izinto ezimbalwa ezenzeka emzimbeni xa le meko isenzeka. Khawuthelekelele ukuba unegadi yemifuno, kwaye kwenzeka ntoni ukuba ukhula lukhula ngokungalawulekiyo? Imifuno elungileyo ilahlekelwa yindawo kwaye ayikhuli kakuhle, akunjalo? Yiloo nto eyenzekayo nalapha.

• Iziphumo zomongo wethambo: Ezo B-cells ezingaqhelekanga ziqala ukuqokelelana kumongo wethambo, njengokhula. Oko kunciphisa amandla okuvelisa iiseli ezimhlophe zegazi ezisempilweni, iiseli ezibomvu zegazi, kunye neeplatelets (iiseli ezinceda ukujiya kwegazi).
• Ukudumba kwe-lymph nodes kunye ne-spleen: Ezi seli ezingaqhelekanga kwi-lymph nodes zethu kunye ne-spleen ziqala ukwahlukana kwaye zande, nto leyo ebangela ukuba ezo zitho zidumbe.
• Iziphumo zesibindi: Ngamanye amaxesha ezi seli zingaqhelekanga zinokusasazeka ziye esibindini ngegazi kwaye zisonakalise. Oku kunokukhokelela ekuqokeleleni kolwelo esiswini (imeko ebizwa ngokuba yi-'ascites').

Ingaba oku kuqhelekile?

I-'Hairy Cell Leukemia' sisifo esingaqhelekanga kakhulu . Kwanakwilizwe elifana neMelika, siyenzeka kumntu omnye kwikhulu lamawaka. Kuthiwa le meko ixhaphake kancinci phakathi kwabantu abamhlophe kunamanye amaqela eentlanga.

Zithini iimpawu?

Ngamanye amaxesha abantu abaneHairy Cell Leukemia basenokungabi nazo naziphi na iimpawu . Nangona kunjalo, xa kuvela iimpawu, zingabandakanya:

• Ukudinwa: Oku kudinwa kunokubakho ngenxa yokwehla kwamanqanaba eeseli ezibomvu zegazi (i-anemia).
• Umkhuhlane kunye nosulelo oluqhelekileyo:Ngenxa yamanqanaba aphantsi eeseli ezimhlophe zegazi ezisempilweni ezilwa nosulelo, umkhuhlane oqhelekileyo kunye nosulelo oluqatha lunokwenzeka kakhulu.
• Intlungu yesisu esisezantsi kwekhohlo: Xa iiseli ezingaqhelekanga kwi-spleen ziqokelelana zize zibangele ukuba i-spleen idumbe, unokufumana iintlungu kwindawo esezantsi kwesisu ekhohlo. I-spleen isenokukhula ikwenze uzive ugcwele, kwaye ungaziva ugcwele nasemva kokutya kancinci. Usenokunciphisa umzimba ngaphandle kokuzama.
• Intlungu kwisisu esisezantsi ngasekunene: Ukuba isibindi sikhulu, unokufumana iintlungu kwicala lasekunene.
• Ubunzima bokuphefumla: Olu lolunye uphawu lwe-anemia.
• Ii-lymph nodes ezidumbileyo: Amaqhuma angenabuhlungu anokuvela entanyeni, emakhwapheni nasesiswini.
• Ukukruquka okanye ukopha okungaqhelekanga: Ngenxa yokuba umongo wethambo awuvelisi iiplatelets ezaneleyo, ezinceda ukujiya kwegazi, nokuba kukruquka okuncinci nokopha kunokwenzeka.

Kubalulekile: Ukuba nenye okanye ngaphezulu kwezi mpawu akuthethi ukuba uneHairy Cell Leukemia. Nangona kunjalo, ukuba ezi mpawu ziyaqhubeka, kungcono ukufuna ingcebiso kagqirha.

Zithini izizathu? Ngaba kukho naziphi na izinto ezibangela umngcipheko?

Iingcali azikayazi eyona nto ibangela iHairy Cell Leukemia. Nangona kunjalo, uphando lufumanise ukuba malunga ne-90% yabantu abanesifo banotshintsho lwemfuza. Luchaphazela i-gene ebizwa ngokuba yi-BRAF. Le gene inceda iiseli zikhule. Ngoko ke, xa itshintsha, iiseli ezingaqhelekanga ziqala ukukhula kwaye zande ngokungalawulekiyo. Olu tshintsho lwemfuza lwenzeka emva kokuba sizelwe. Nangona kunjalo, akukacaci ukuba yintoni ebangela olo tshintsho.

Nangona kunjalo, kuye kwafunyaniswa ezinye izinto ezibangela umngcipheko wokufumana esi sifo:

• Ubudala: Le meko ixhaphake kakhulu kubantu abaneminyaka ephakathi kwama-40 nama-70. Ubudala obuqhelekileyo bokuxilongwa bumalunga neminyaka engama-58.
• Isini: Esi sifo sixhaphake kakhulu kumadoda kunabafazi.
• Ukuvezwa yi-Agent Orange: Abaphandi bakholelwa ukuba kukho unxibelelwano phakathi kwale khemikhali kunye ne-chronic B-cell leukemia kunye ne-lymphoma (kuquka ne-hairy cell leukemia).

Zeziphi iingxaki ezinokubangela oku?

I-Hairy Cell Leukemia ibangela ukwehla okukhulu kwenani leeseli ezimhlophe zegazi emzimbeni wakho ezilwa nosulelo, nto leyo enyusa umngcipheko wakho wokufumana usulelo . Ezi zinokuba zizifo zebhaktiriya, iintsholongwane, okanye iifungus. Ngamanye amaxesha, ezi zifo zinokuba yingozi ebomini.

Kukwakho nethuba elincinci lokufumana uhlobo lwesibini lomhlaza emva kweminyaka emininzi emva kokufunyaniswa kunye nonyango. Umzekelo, izifundo ezilandele abantu abaye banyangwa ngempumelelo iHairy Cell Leukemia kangangeminyaka eli-10-20 ziye zaxela iimeko zokuba abanye baye bafumana eminye imimhlaza.

Sifunyaniswa njani esi sifo?

Xa ubona ugqirha, uza kukubuza ngezinto ezifana nokuba iimpawu zakho zaqala nini kwaye ingaba ufumana usulelo rhoqo na. Uza kukubuza nangembali yakho yezonyango. Emva koko, baza kwenza uvavanyo lomzimba. Banokujonga nee-lymph nodes zakho, i-spleen, kunye nesibindi sakho ukuba azidumbanga na.

Emva koko, ungathunyelwa kwi-hematologist okanye kwi-medical oncologist ukuze ufumane olunye uvavanyo ukuqinisekisa ukuxilongwa. Olu vavanyo lungabandakanya:

• Ubalo lwegazi olupheleleyo (CBC) kunye nomahluko: Oku kulinganisa inani leeseli zegazi ezibomvu nezimhlophe, kunye neeplatelets. Umahluko ulinganisa inani leendidi ezahlukeneyo zeeseli zegazi ezimhlophe.
• I-smear yegazi engaphandle: Iingcali zezifo zijonga isampuli yegazi phantsi kwemakroskopu ukuze zijonge iiseli ezingaqhelekanga ezineembonakalo ezifana "neenwele".
• Ukufunxwa kwethambo kunye ne-bone marrow biopsy: Olu vavanyo lunokufumanisa iiseli ezingaqhelekanga kumongo wethambo.
• I-CT scan (i-computed tomography - `CT` scan): I-`CT` scan ingathatha imifanekiso eneenkcukacha zangaphakathi lomzimba. Oku kungasetyenziselwa ukubona ubungakanani bepleen, isibindi, kunye nee-lymph nodes, okanye i-ultrasound yesisu.
• I-Flow cytometry: Oku kujonga igazi kunye nomongo weeseli ezingaqhelekanga, ezifana neeseli zeenwele. Kukwajonga ubungakanani, imilo yeeseli, kunye nokuba zikhona iimpawu zethumba. Iimpawu zethumba zinokuba ziiproteni eziveliswa ziiseli zomhlaza.

Iphathwa njani?

Ukuba unentsholongwane, kuya kufuneka uyinyange kuqala. Emva koko, kuya kuqaliswa ezinye iindlela zonyango zokulawula iimpawu ze-'Hairy Cell Leukemia'. Nangona kunjalo, njengoko besitshilo ngaphambili, ayinguye wonke umntu oza kuba neempawu. Kubantu abanjalo, ingcali yakho ye-hematologist okanye ingcali yomhlaza inokucebisa indlela ebizwa ngokuba yi -active surveillance . Ukuba kukho iimpawu, banokukunika amayeza.

Uhlolo Olusebenzayo

I-Hairy Cell Leukemia sisifo esikhula kancinci kakhulu . Kungathatha iminyaka ukuba iimpawu zibonakale. Ngenxa yokuba unyango lomhlaza lunokubangela iziphumo ebezingalindelekanga kunye neengxaki, ukuba awunazo iimpawu, ugqirha wakho unokugqiba ekubeni alibazise ukuqala unyango de kuvele iimpawu.

Ngexesha lokuhlolwa okusebenzayo, uza kubona ugqirha wakho rhoqo kwiinyanga ezintathu ukuya kwezintandathu. Ngexesha lotyelelo:

• Baza kukubuza ngempilo yakho ngokubanzi, ingakumbi ukuba uzive udiniwe ngaphezu kwesiqhelo.
• Kuya kwenziwa uvavanyo lomzimba ukujonga ukudumba kwesibindi kunye nepleen.
• Uvavanyo lwegazi luyalelwe ukujonga amanqanaba eeseli zegazi kunye neeplatelets.

Kunokuba nzima ngokweemvakalelo ukulinda ukuba iimpawu zibonakale ngaphambi kokuba uqale unyango. Kodwa ugqirha wakho uya kuziqonda iimvakalelo zakho aze akuchazele ukuba kutheni kucetyiswa ukuba uhlolwe.

Amayeza

Unyango oluphambili kubantu abane-leukemia yeeseli ezinoboya yi -chemotherapy . Amayeza okulwa nomhlaza asetyenziswa kakhulu yiCladribine (Leustatin®) okanye iPentostatin (Nipent®).

Ukuba i-chemotherapy ayisebenzi, okanye ukuba isifo siphinde sibuye, kukho ezinye iindlela zonyango:

• Unyango lwe-Immunotherapy: `Rituximab (Rituxan®)` lunyango lwe-`monoclonal antibody` olusetyenziselwa `Hairy Cell Leukemia`. Inganikwa njengonyango lokuqala nge-chemotherapy okanye ukuba isifo siphinde sibuye. Olunye unyango lwe-immunotherapy yi-`Recombinant Interferon Alfa-2b (Intron A®)`.
• Unyango olujoliswe kulo: KwiHairy Cell Leukemia ephinde yabuyela kwisimo sayo, amayeza abizwa ngokuba yiRituximab kunye neMoxetumomab pasudotox (Lumoxiti®) (olu luhlobo lwe-antibody-drug conjugate) angasetyenziswa.
• I-Splenectomy: Kunqabile kakhulu, olu tyando lunokucetyiswa ukuba unyango olufana ne-chemotherapy aluncedi kwaye i-spleen iyaqhubeka nokudumba ngenxa yeeseli ezingaqhelekanga kwi-spleen.

Ngaba kukho naziphi na iziphumo ebezingalindelekanga zonyango?

Njengalo naluphi na unyango lomhlaza, lunokuba nemiphumo emibi. Ngokukodwa, amayeza e-chemotherapy kwi-hairy cell leukemia andisa umngcipheko wosulelo olusongela ubomi . Ugqirha wakho uza kukuchazela amanyathelo onokuwathatha ukuthintela usulelo kunye notshintsho emzimbeni wakho olunokuba ziimpawu zosulelo.

Yintoni onokuyilindela ngelixa uphila nesi sifo?

Okwangoku akukho nyango lweHairy Cell Leukemia. Nangona kunjalo, uphando lubonisa ukuba unyango, kuquka ne-chemotherapy, lunokugcina esi sifo siphilile iminyaka eli-10 nangaphezulu . Ukuxolelwa kuthetha ukuba awunazo iimpawu kwaye akukho zimpawu zesifo ezinokufunyanwa kwiimvavanyo. Uphononongo oluhlalutye amava abantu abaneHairy Cell Leukemia lufumanise ukuba emva konyango, banokuphila ixesha elide njengabantu abangenaso esi sifo.

Nangona kunjalo, i-hairy cell leukemia inokuphinda ivele emva kweminyaka eliqela emva konyango lokuqala. Nangona kunjalo, ukuba oko kuyenzeka, kukho unyango olunokubuyisela isifo kwimeko entle.

Abantu abaninzi bafuna unyango kunye nenkxaso ubomi babo bonke, njengoko oogqirha kufuneka bajonge ukuba isifo siphinde sibuye na. Nokuba unesifo kodwa awunazo iimpawu, kuya kufuneka wenze uvavanyo kunye nokudibana nogqirha rhoqo emva kweenyanga ezintathu ukuya kwezintandathu emva kokuba uxilongiwe.

Ingakanani izinga lokubuyela kwimeko yesiqhelo?

Ngokutsho kwe-Leukemia and Lymphoma Society, ama-90% abantu abanyangwa ngamayeza e-chemotherapy i-Cladribine bayaphila emva kweminyaka emihlanu emva konyango. Nangona kunjalo, i-prognosis yakho - okwenzekayo emva konyango - ixhomekeke kwizinto ezininzi . Ubudala bakho, impilo yakho iyonke, iimpawu, kunye nendlela umzimba wakho osabela ngayo kunyango zonke zidlala indima. Ukuba unemibuzo malunga noku, buza ugqirha wakho.

Ndingayinyamekela njani impilo yam?

I-Hairy Cell Leukemia yandisa umngcipheko wosulelo olusongela ubomi. Ungaqinisa amajoni akho omzimba kwaye uzikhusele kwiintsholongwane . Nazi ezinye iingcebiso:

• Yitya ukutya okunempilo: Izinto ezifana neziqhamo, imifuno, iiproteni ezingenamafutha, kunye neenkozo ezipheleleyo zinceda inkqubo yomzimba yokuzikhusela ekusebenzeni kakuhle.
• Ukuzilolonga imihla ngemihla: Ukuzilolonga yonke imihla kulungele iinkalo ezininzi zempilo yakho, kuquka nenkqubo yakho yomzimba yokuzikhusela.
• Lala ngokwaneleyo: Ukungalali ngokwaneleyo kunokwenza inkqubo yakho yomzimba yokuzikhusela ingasebenzi kakuhle.
• Fumana amayeza akho okugonya ngexesha: Amayeza okugonya aqeqesha umzimba wakho ukuba ulwe neentsholongwane ezibangela izifo. Thetha nogqirha wakho malunga nokuba zeziphi iimithi zokugonya ozifunayo kunye nokuba uza kuzifumana nini.

Ufanele uye nini kugqirha?

I-Hairy Cell Leukemia kunye nonyango lwayo kunokunyusa umngcipheko wakho wokufumana usulelo olunzima, ngamanye amaxesha olusongela ubomi. Bonana nogqirha ngoko nangoko ukuba unezi mpawu:

• Umkhuhlane ongaphezulu kwama-38 degrees Celsius (100.4 degrees Fahrenheit) kwaye awunciphi.
• Umphimbo obuhlungu ohlala iintsuku ezingaphezu kwezimbalwa okanye oqhubeka uba mandundu.
• Intlungu yesisu engapheliyo okanye eba mandundu.

Isishwankathelo kunye nesokugqibela (Umyalezo oya nawo ekhaya)

Mhlawumbi ubuziva udiniwe ngaphezu kwesiqhelo ngaphandle kwesizathu. Okanye mhlawumbi ubucinga ngesifo sokuphefumla esingaphelanga nangona amayeza amaninzi obuwasebenzisa. Ngoku uvavanyo lubonise ukuba unomhlaza wegazi ongaqhelekanga obizwa ngokuba yi-'Hairy Cell Leukemia', ongenakunyangeka ngokupheleleyo ngonyango.

Kodwa ungakhathazeki . Kukho unyango olunokugcina imeko ilawulwa iminyaka emininzi ngaphandle kweempawu. 'Ukuxolelwa' kwexesha elide kuthetha ukuba iqela lakho lezempilo liya kujonga impilo yakho ubomi bakho bonke. Kwaye, ukuba iHairy Cell Leukemia iyabuya, abaphandi bayaqhubeka nokukhangela iindlela zokubuyisela imeko 'kwimeko yokuxolelwa'.

Kukho izinto onokuzenza ngecala lakho. Umzekelo, unyango lomhlaza lunokunyusa umngcipheko wakho wokosuleleka. Buza ugqirha wakho malunga nokugonywa kunye namanye amanyathelo onokuwathatha ukunciphisa umngcipheko wakho wokosuleleka.Awuwedwa, kukho oogqirha kunye nabasebenzi bezempilo abanokukunceda kolu hambo.

I- Hairy Cell Leukemia, i-leukemia, umhlaza wegazi, ii-B cells, umongo wamathambo, i-spleen, iimpawu