Ngaba nawe uyafuna ukwazi ngesi sifo segazi esingaqhelekanga? Masithethe ngeWaldenström Macroglobulinemia ngokulula!

Ngaba wakha weva ngeWaldenström Macroglobulinemia? Ligama elide, elinzima ukulibiza, akunjalo? Ngokwenene luhlobo lomhlaza olukhula kancinci egazini. Usenokuba awuzange uve ngalo, kuba yimeko engaqhelekanga. Kodwa kulungile oko, namhlanje siza kuthetha ngalo ngokulula, ngendlela onokuyiqonda.

Yintoni iWaldenstrom macroglobulinemia (WM)?

Ngamafutshane, iWaldenstrom macroglobulinemia, okanye iWM ngamafutshane, ngumhlaza wegazi . Luhlobo lomhlaza olubizwa ngokuba yi-non-Hodgkin lymphoma, ekwaziwa ngokuba yi-lymphoplasmacytic lymphoma. Enyanisweni aluqhelekanga kakhulu. Khawucinge nje, nakwilizwe elifana neMelika, ngabantu abathathu ukuya kwabane kuphela kwisigidi abafumana esi sifo.

Ngoko ke, le `(WM)` ikhula njani? Sinomongo wamathambo emizimbeni yethu, kwaye kulapho iiseli zethu zegazi zenziwa khona. Esi sifo siqala xa ezinye zeeseli ezibizwa ngokuba yi`(iiseli ze-B)` (ezi luhlobo lweeseli kwinkqubo yethu yokuzikhusela, oko kukuthi, iiseli ezisikhusela kwizifo) kule mongo wamathambo ziba ziiseli zomhlaza.

Ezi seli zomhlaza, endaweni yokuba zishiywe zodwa, ziqala ukwenza ezinye iiseli ezifana nazo. Njengokhula ehlathini. Kwenzeka ntoni ke ngoko? Iiseli zegazi ezisempilweni ezifunekayo emzimbeni wethu azinandawo, kwaye ziqala ukuncipha.

• Xa iiseli ezibomvu zegazi ziphantsi, kubakho i-anemia. Oku kukwenza uzive udiniwe kwaye uphaphathekile.
• Xa iiseli ezimhlophe zegazi ziphantsi, kuvela imeko ebizwa ngokuba yi-"neutropenia", nto leyo ekwenza ube sesichengeni sokufumana izifo.
• Xa iiplatelets (iiseli ezinceda ukujiya kwegazi) ziphantsi (thrombocytopenia), ukopha kusenokungayeki lula.

Ayipheleli apho, ezi seli zomhlaza zivelisa iproteni engaqhelekanga ebizwa ngokuba yi-`(immunoglobulin M)` okanye `(IgM)`. Xa le protein ye-`(IgM)` iphezulu kakhulu egazini, igazi lethu liba likhulu, njengobusi . Oku kubizwa ngokuba yi-`(hyperviscosity syndrome).` Xa igazi likhula ngolu hlobo, kuba nzima ukuba igazi lihambe ngemithambo yegazi emincinci emzimbeni wonke. Emva koko, kukho uthotho lweempawu ezinzulu ezinokuvela.

Into ebalulekileyo kukuba akukho nyango lwe-WM okwangoku. Kodwa ungakhathazeki. Kukho unyango olunokunceda ukulawula iimpawu, ngamanye amaxesha lude luziphelise, kwaye lukuncede uhlale usempilweni. I-WM idla ngokuba sisifo esikhula kancinci, ngoko unokuphila kakuhle naso iminyaka emininzi.

Zithini iimpawu zesi sifo `(WM)`?

Khawucinge nje, malunga nomntu omnye kwabane abanesi sifo akabonisi zimpawu . Bafumanisa ngesi sifo kuphela xa besiya kugqirha ngesizathu esithile. Kodwa, xa iimpawu zibonakala, zihlala ziqala kancinci . Makhe sijonge ukuba zeziphi ezi mpawu:

• Ubuthathaka kunye nokudinwa rhoqo:Kuvakala ngathi ibhetri ifile.
• Umkhuhlane: Umzimba uziva ushushu ngaphandle kwesizathu.
• I-Anorexia: Ukungaziva ngathi ufuna ukutya.
• Ukubila ebusuku: Ukubila kakhulu xa ulele.
• Ukunciphisa umzimba: Uba mncinci ngaphandle kwesizathu esithile.
• Ukudideka: Ubunzima bokugxila, ukudideka kancinci.
• Ukudumba kwesibindi, i-spleen, okanye ii-lymph nodes: Ngugqirha kuphela onokukuxelela ngokuqinisekileyo.
• Iimpawu ze-peripheral neuropathy, ezinje ngokuba ndindisholo kwiminwe nakwiinzwane : Kuvakala ngathi kukurhawuzelela, okanye ukulahlekelwa yimvakalelo.
• Iimpawu zokuqhekeka kwegazi: ukopha empumlweni, ukopha kweentsini xa uhlamba amazinyo, isiyezi, intloko ebuhlungu, kunye nokungaboni kakuhle.

Ukuba unempawu enye okanye ezimbini kwezi akuthethi ukuba une-WM. Kodwa ukuba ezi mpawu ziyaqhubeka, kungcono ukufuna ingcebiso kagqirha.

Zithini izizathu zokwakheka kwe-`(WM)`?

Eyona nto ibangela i-Waldenstrom's macroglobulinemia kukuguquka kwezakhi zofuzo . Abantu abangaphezu kwama-90% abanesifo, okanye abantu abalithoba kwabalishumi, banokuguquka kwezakhi zofuzo okubizwa ngokuba yi-MYD88. Malunga nama-40% abantu nabo banokuguquka kwezakhi zofuzo okubizwa ngokuba yi-CXCR4. Zombini ezi nguqu zezakhi zofuzo zinceda iiseli ezingaqhelekanga kwi-WM ukuba zahlukane ngokukhawuleza.

Into ebalulekileyo kukuba olu tshintsho lwemfuza alulofuzo . Oko kukuthi, aluyonto uyifumana kubazali bakho, kwaye aluyonto oyidlulisela kubantwana bakho. Luyenzeka ubomi bonke. Nangona kunjalo, abaphandi abakaqiniseki ukuba yintoni ebangela olu tshintsho lwemfuza kwasekuqaleni.

Ngubani osengozini enkulu yokufumana esi sifo? (Izinto ezibangela umngcipheko)

Kukho izinto ezithile ezinyusa amathuba okukhula kwe-`(WM)`. Makhe sibone ukuba zeziphi:

• Ubudala: Esi sifo sidla ngokufunyaniswa kubantu abaneminyaka engama-65 nangaphezulu.
• Uhlanga: Esi sifo sixhaphake kakhulu kubantu abamhlophe.
• Isini: Amadoda anamathuba amaninzi okufumana esi sifo.
• Ezinye iimeko zonyango: Usenokuba sengozini enkulu ukuba unezifo ezifana ne-`(Hepatitis C)`, `(AIDS)`, `(Sjögren's Syndrome)`, okanye imeko ebizwa ngokuba yi-`(MGUS - Monoclonal Gammopathy of Undetermined Significance)` (le yindlela yokuqala ye-`(WM)`). Nangona kunjalo, kubalulekile ukukhumbula ukuba ayinguye wonke umntu one-`(MGUS)` oya kuba ne-`(WM)`.
• Imbali yosapho: Ukuba izalamane zakho zegazi ziye zaba ne-WM okanye ezinye iintlobo ze-lymphoma, nawe usenokuba sengozini.

Ziziphi iingxaki ezinokubakho zesi sifo?

Kwezinye iimeko ezinzima, i-WM inokubangela ezinye iingxaki.

• I-Amyloidosis: Oku kwenzeka xa iiproteni ezineengxaki zigcinwa kwizitho ezifana nentliziyo, imiphunga, kunye nezintso.
• I-Cryoglobulinemia: Kule meko, iiproteni ezithile zegazi ezisabela kwingqele ziyaqokelelana emalungwini zize zihlangane. Oku kunokubangela iintlungu kunye nokutshintsha kombala oluhlaza okwesibhakabhaka/omhlophe (i-cyanosis).

Oogqirha bayixilonga njani le sifo? (Ukuxilongwa)

Ugqirha wakho angasebenzisa iimvavanyo ezininzi ukuze afumanise ukuba unayo na i-WM. Ngokuyintloko zikhangela iiseli zomhlaza kunye neproteni ye-IgM esithethe ngayo ngaphambili.

• Uvavanyo lwegazi nomchamo: Kuthathwa iisampulu zegazi nomchamo ukujonga iimpawu ze-WM. Zikhangela izinto ezifana nokubalwa kweeseli zegazi eziphantsi kunye neeproteni ze-IgM ezingaqhelekanga.
• Uvavanyo lokufota: I-CT scan okanye i-CT-PET scan, edityaniswe ne-CT kunye ne-PET scan, isetyenziselwa ukukhangela iimpawu ze-WM emzimbeni. Ezi mvavanyo zijonga izinto ezifana namalungu avuvukileyo kunye nee-lymph nodes.
• Uvavanyo lwamehlo: Ukuhlola ukuba akukho gazi na ngaphakathi ngasemva kweliso. Olu luphawu lokuqhekeka kwegazi.
• I-Bone marrow biopsy: Oku kuquka ukuthatha isampuli encinci yomongo wethambo uze uyijonge phantsi kwe-microscope ukuze ubone ukuba kukho iiseli zomhlaza na.

Iphathwa njani `(WM)`?

Njengoko besitshilo ngaphambili, akukho nyango lwesi sifo okwangoku. Ke ngoko, unyango olungcono kukunciphisa iimpawu ngaphandle kwemiphumo emibi kakhulu . Ugqirha wakho uza kuthetha nawe aze enze isicwangciso sonyango esifanelekileyo kuwe. Nazi ezinye zeendlela zonyango lwe-`(WM)`:

• Ukulinda ngononophelo: Ukuba awunazo iimpawu, ugqirha wakho usenokungaqali unyango. Abanye abantu abane-WM basenokungaludingi unyango iminyaka emininzi.
• I-Plasmapheresis / i-plasma exchange: Oku kuquka ukusebenzisa umatshini ukucoca iproteni ye-IgM engaqhelekanga kwi-plasma, inxalenye engamanzi egazini lakho. I-plasma ecociweyo emva koko ibuyiselwa egazini lakho. Olu nyango lusetyenziselwa ukunciphisa iimpawu zokuxinana kwegazi.
• Unyango lwe-Immunotherapy: Olu nyango lusebenzisa inkqubo yakho yomzimba yokuzikhusela ukutshabalalisa okanye ukucothisa ukukhula kweeseli ze-WM. Iyeza iRituximab (Rituxan®) lidla ngokunikwa lodwa okanye kunye ne-chemotherapy.
• Unyango lweKhemotherapy: Amayeza okubulala iiseli zomhlaza anokunikwa eyedwa okanye edibene ne-immunotherapy.
• Ii-Corticosteroids: Uhlobo lwe-steroid, olufana ne-dexamethasone, lunokunikwa kunye nonyango lokunciphisa umngcipheko womhlaza kunye ne-chemotherapy. Zinceda ukulwa nomhlaza ngelixa zinciphisa iziphumo ebezingalindelekanga zonyango.
• Unyango olujoliswe kulo:Olu nyango lusebenza ngokuthintela iiproteni ezisetyenziswa ziiseli zomhlaza ukukhula. I-Ibrutinib (Imbruvica®) kunye ne-zanubrutinib (Brukinsa®) ziindlela ezimbini zonyango ezijoliswe kuzo ezivunyiweyo yi-U.S. Food and Drug Administration (FDA) ukunyanga i-WM.
• Ukufakelwa kweeseli zesiqu: Kule meko, kufakelwa umongo wethambo ophilileyo ukuze kuthathelwe indawo umongo wethambo ochaphazeleke ziiseli ezingaqhelekanga. Oku akwenziwa rhoqo, kwaye kwenziwa kuphela kwizigulana ezikhethiweyo.

Ndingalufuna nini uncedo lwezonyango?

Ukuba ufunyaniswe une-WM, thetha nogqirha wakho ngoko nangoko ukuba uneempawu ezintsha okanye ukuba ukhathazekile ngazo naziphi na iimpawu ezikhoyo. Wakuba ufunyaniswe une-WM, kubalulekile ukubuza imibuzo ukuze ufumane into onokuyilindela. Nazi ezinye zemibuzo onokuzibuza ugqirha wakho:

• Ingaba ibaluleke kangakanani le nto `(WM)`? Oku kuza kuyichaphazela njani ubomi bam?
• Yintoni endimele ndiyilindele kwixesha elifutshane nelide?
• Ngaba kufuneka ndiqale unyango ngoko nangoko?
• Luhlobo luni lonyango olucetyiswayo?
• Zeziphi iziphumo ebezingalindelekanga ezinokulindelwa kunyango?

Ndingalindela ntoni ukuba ndinayo le meko?

Ayingabo bonke abantu abanesifo esiqhubeka kancinci kancinci njengeWaldenstrom's macroglobulinemia abafanayo. Uphando lubonisa ukuba ama-66%, okanye ngaphezulu kwabantu ababini kwabathathu, basaphila emva kweminyaka eli-10 emva kokufunyaniswa kwesi sifo. Nangona kunjalo, amaxesha okusinda anokwahluka ngokweminyaka . Uninzi lwabantu abangaphezu kwama-65 (iqela leminyaka elihlala lifunyaniswa linesifo) bafa ngenxa yezizathu ezingadibaniyo ne-WM.

Zininzi izinto ezichaphazela ukubikezela kwangaphambili kwesifo sakho. Umzekelo:

• iminyaka yakho
• Iziphumo zovavanyo lwegazi
• Uhlobo lotshintsho lwemfuza (ngamanye amaxesha ukungabikho kotshintsho lwe-`(MYD88)` kunokubonisa isiphumo esibi)

Ukuba unemibuzo malunga nemeko yakho, thetha nogqirha wakho . Uyakwazi kakuhle kunye nezinto ezinokuchaphazela impilo yakho.

Ngaba kukho into endinokuyenza ukuze ndizive bhetele?

Ukuphila ne-lymphoma efana ne-Waldenstrom's macroglobulinemia kunokuthetha ukwenza utshintsho olukhulu ebomini bakho. Kubalulekile ukusebenzisa zonke izixhobo onazo. Thetha nogqirha wakho malunga nokutya omawukutye kunye namanyathelo okunyamekela umzimba wakho .

Ukuze ungaziva unesizungu, qhagamshelana nabanye abantu abanale sifo singaqhelekanga. Nokuba uziva ngaloo ndlela ekuqaleni xa ufumanisa ukuba unesi sifo, awuwedwa kolu hambo . Buza ugqirha wakho kwaye ujoyine amaqela enkxaso.

Okokugqibela, khumbula oku.

Abaphandi abakafumani unyango lweWaldenstrom macroglobulinemia (WM). Nangona kunjalo, bafumene unyango oluninzi olwenza kube lula ukuphila nesi sifo . Abantu abaninzi baphila nesi sifo iminyaka emininzi ngaphandle kweempawu. Unyango olutsha luvumela abantu abane-WM ukuba baphile ixesha elide kunangaphambili, ngaphandle kokubeka ubomi babo esichengeni.

Ukuba ufumana olu xilongo, ungoyiki kwaye ubuze ugqirha wakho ngembono yexesha elifutshane nelide. Baza kukunceda ukhethe isicwangciso sonyango esifanelekileyo kuwe. Khumbula, ngeengcebiso zonyango ezifanelekileyo kunye nenkxaso, ungaphila ngempumelelo nale meko.

I- Waldenstrom macroglobulinemia, i-WM, umhlaza wegazi, iproteni ye-IgM, isifo se-hyperviscosity, umongo wamathambo, i-lymphoma