Uyafuna ukwazi ngalesi sifo segazi esingavamile? Ake sixoxe nge-Waldenström Macroglobulinemia ngamagama alula!

Wake wezwa nge-Waldenström Macroglobulinemia? Yigama elide, okunzima ukulibiza, akunjalo? Empeleni uhlobo lomdlavuza olukhula kancane egazini. Kungenzeka ukuthi awukaze uzwe ngalo, ngoba kuyisimo esingavamile. Kodwa kulungile lokho, namuhla sizokhuluma ngakho kalula, ngendlela ongayiqonda.

Kuyini i-Waldenstrom macroglobulinemia (WM)?

Kalula nje, i-Waldenstrom macroglobulinemia, noma i-WM ngamafuphi, umdlavuza wegazi . Luhlobo lomdlavuza olubizwa ngokuthi i-non-Hodgkin lymphoma, olwaziwa nangokuthi i-lymphoplasmacytic lymphoma. Empeleni aluvamile kakhulu. Cabanga nje, ngisho nasezweni elifana neMelika, abantu abathathu kuya kwabane kuphela kwabayisigidi abathola lesi sifo.

Ngakho-ke, lokhu `(WM)` kwenzeka kanjani? Sinomongo emzimbeni wethu, futhi yilapho amangqamuzana ethu egazi enziwa khona. Lesi sifo siqala lapho amanye amangqamuzana abizwa ngokuthi `(amangqamuzana e-B)` (lawa awuhlobo lweseli ohlelweni lwethu lokuzivikela komzimba, okungukuthi, amangqamuzana asivikela ezifweni) kulo mongo eba amangqamuzana anomdlavuza.

Lawa maseli omdlavuza, esikhundleni sokushiywa wodwa, aqala ukwenza amaseli amaningi afana nawo. Njengokhula ehlathini. Kwenzekani-ke? Amaseli egazi anempilo adingwa yimizimba yethu awanayo indawo, futhi aqala ukuncipha.

• Uma amangqamuzana abomvu egazi ephansi, kuvela i-anemia. Lokhu kukwenza uzizwe ukhathele futhi uphaphathekile.
• Uma amangqamuzana amhlophe egazi ephansi, kuvela isimo esibizwa ngokuthi "i-neutropenia", okwenza kube lula ukuthi uhlaselwe yizifo.
• Uma ama-platelet (amangqamuzana asiza ukujiya kwegazi) ephansi (i-thrombocytopenia), ukopha kungase kungapheli kalula.

Akukhona lokho kuphela, la maseli omdlavuza akhiqiza iphrotheni engajwayelekile ebizwa ngokuthi `(immunoglobulin M)` noma `(IgM)`. Lapho le phrotheni `(IgM)` iba phezulu kakhulu egazini, igazi lethu liba likhulu, njengoju . Lokhu kubizwa ngokuthi `(hyperviscosity syndrome).` Lapho igazi liba likhulu kanje, kuba nzima ngegazi ukugeleza emithanjeni yegazi emincane kuwo wonke umzimba. Ngemuva kwalokho, kungase kuvele uchungechunge lwezimpawu ezingathi sína.

Into ebalulekile ukuthi alikho ikhambi le-WM okwamanje. Kodwa ungakhathazeki. Kunezindlela zokwelapha ezingasiza ekulawuleni izimpawu, ngezinye izikhathi zize ziqede, futhi zikusize uhlale uphilile. I-WM ivame ukuba yisifo esikhula kancane, ngakho ungaphila kahle naso iminyaka eminingi.

Ziyini izimpawu zalesi sifo `(WM)`?

Cabanga nje, cishe umuntu oyedwa kwabane abanalesi sifo akabonisi zimpawu . Bathola ulwazi ngalesi sifo kuphela uma beya kudokotela ngesizathu esithile. Kodwa, uma kuvela izimpawu, zivame ukuza kancane kancane . Ake sibheke ukuthi ziyini lezi zimpawu:

• Ubuthakathaka nokukhathala okuqhubekayo:Kuzwakala sengathi ibhethri lifile.
• Umkhuhlane: Umzimba uzizwa ushisa ngaphandle kwesizathu.
• I-Anorexia: Ukungazizwa sengathi ufuna ukudla.
• Ukujuluka ebusuku: Ukujuluka kakhulu ngenkathi ulele.
• Ukwehlisa isisindo: Uba mncane ngaphandle kwesizathu esithile.
• Ukudideka: Ubunzima bokugxilisa ingqondo, ukudideka kancane.
• Ukuvuvukala kwesibindi, ubende, noma ama-lymph node: Udokotela kuphela ongakutshela ngokuqinisekile.
• Izimpawu ze-peripheral neuropathy, njengokungazweli eminweni nasezinzwaneni : Kuzwakala sengathi kukhona umuzwa wokuthuthumela, noma ukulahlekelwa umuzwa.
• Izimpawu zokuqhekeka kwegazi: ukuphuma kwegazi ekhaleni, ukuphuma kwegazi ezinsinini lapho uxubha amazinyo, isiyezi, ikhanda elibuhlungu, kanye nokungaboni kahle.

Ukuthi nje unesinye noma ezimbili zalezi zimpawu akusho ukuthi une-WM. Kodwa uma lezi zimpawu ziqhubeka, kungcono ukufuna iseluleko sezokwelapha.

Yiziphi izizathu zokwakheka kwe-`(WM)`?

Imbangela eyinhloko ye-macroglobulinemia kaWaldenstrom ukuguquka kwezakhi zofuzo . Abantu abangaphezu kuka-90% abanalesi sifo, noma abantu abayisishiyagalolunye kwabayishumi, banokuguquka kwezakhi zofuzo okubizwa ngokuthi i-MYD88. Abantu abangaba ngu-40% nabo banokuguquka kwezakhi zofuzo okubizwa ngokuthi i-CXCR4. Zombili lezi zinguquko zezakhi zofuzo zisiza amangqamuzana angajwayelekile ku-WM ukuba ahlukane ngokushesha.

Into ebalulekile ukuthi lezi zinguquko zofuzo azilona ufuzo . Okusho ukuthi, aziyona into oyithola kubazali bakho, futhi aziyona into oyidlulisela ezinganeni zakho. Zenzeka kukho konke ukuphila. Kodwa-ke, abacwaningi abakaqiniseki ukuthi yini ebangela lezi zinguquko zofuzo kwasekuqaleni.

Ubani osengozini enkulu yokuthola lesi sifo? (Izici eziyingozi)

Kunezici ezithile ezandisa amathuba okukhula kwe-`(WM)`. Ake sibone ukuthi ziyini:

• Ubudala: Lesi sifo sivame ukutholakala kubantu abaneminyaka engu-65 noma ngaphezulu.
• Uhlanga: Lesi sifo sivame kakhulu kubantu abamhlophe.
• Ubulili: Amadoda anamathuba amaningi okuthola lesi sifo.
• Ezinye izimo zezokwelapha: Ungase ube sengozini enkulu uma unezifo ezifana ne-`(Hepatitis C)`, `(AIDS)`, `(Sjögren's Syndrome)`, noma isimo esibizwa ngokuthi `(MGUS - Monoclonal Gammopathy of Undetermined Significance)` (lokhu kuyisandulela se-`(WM)`). Kodwa-ke, kubalulekile ukukhumbula ukuthi akuwona wonke umuntu one-`(MGUS)` ozothuthuka nge-`(WM)`.
• Umlando womndeni: Uma izihlobo zakho zegazi ziye zaba ne-WM noma ezinye izinhlobo ze-lymphoma, nawe ungase ube sengozini.

Yiziphi izinkinga ezingaba khona ngenxa yalesi sifo?

Kwezinye izimo ezinzima kakhulu, i-WM ingabangela ezinye izinkinga.

• I-Amyloidosis: Lokhu kwenzeka lapho amaprotheni anephutha efakwa ezithweni ezifana nenhliziyo, amaphaphu nezinso.
• I-Cryoglobulinemia: Kulesi simo, amaprotheni athile egazi asabela emakhazeni aqoqana emilenzeni bese ehlangana ndawonye. Lokhu kungabangela ubuhlungu kanye nokushintsha kombala okuluhlaza okwesibhakabhaka/okumhlophe (i-cyanosis).

Odokotela basithola kanjani lesi sifo? (Ukuxilongwa)

Udokotela wakho angasebenzisa izivivinyo eziningana ukuthola ukuthi une-WM yini. Ngokuyinhloko zibheka amangqamuzana omdlavuza kanye neprotheni ye-IgM esikhulume ngayo ngaphambili.

• Ukuhlolwa kwegazi nomchamo: Kuthathwa amasampula egazi nomchamo ukuze kuhlolwe izimpawu ze-WM. Abheka izinto ezifana nokubalwa kwamangqamuzana egazi aphansi kanye namaprotheni e-IgM angajwayelekile.
• Ukuhlolwa kwezithombe: I-CT scan noma i-CT-PET scan, okuyinhlanganisela ye-CT kanye ne-PET scan, isetshenziselwa ukubheka izimpawu ze-WM emzimbeni. Lezi zivivinyo zibheka izinto ezifana nezitho ezivuvukele kanye nama-lymph node.
• Ukuhlolwa kwamehlo: Ukuhlolwa kokuphuma kwegazi ngemuva kweso. Lokhu kuyisibonakaliso sokuqhekeka kwegazi.
• Ukuhlolwa komnkantsha wethambo: Lokhu kuhilela ukuthatha isampula encane yomnkantsha wethambo bese uyibheka ngaphansi kwesibonakhulu ukuze ubone ukuthi akhona yini amangqamuzana omdlavuza.

Iphathwa kanjani i-`(WM)`?

Njengoba sesishilo ngaphambili, alikho ikhambi lalesi sifo okwamanje. Ngakho-ke, ukwelashwa okungcono kakhulu ukuqeda izimpawu ngemiphumela emibi emincane . Udokotela wakho uzokhuluma nawe futhi adale uhlelo lokwelashwa olufanele wena. Nazi ezinye zezinketho zokwelapha ze-`(WM)`:

• Ukulinda ngokuqapha: Uma ungenazo izimpawu, udokotela wakho angase angaqali ukwelashwa. Abanye abantu abane-WM bangase bangadingi ukwelashwa iminyaka eminingi.
• I-Plasmapheresis / ukushintshaniswa kwe-plasma: Lokhu kuhilela ukusebenzisa umshini ukuhlunga iphrotheni ye-IgM engajwayelekile kusuka ku-plasma, ingxenye ewuketshezi yegazi lakho. I-plasma ehlanziwe ibe isibuyiselwa egazini lakho. Lokhu kwelashwa kusetshenziselwa ukunciphisa izimpawu zokujiya kwegazi.
• Ukwelashwa Ngezifo Ezingalawuleki: Lokhu kwelashwa kusebenzisa amasosha omzimba wakho ukubhubhisa noma ukubambezela ukukhula kwamaseli e-WM. Umuthi i-Rituximab (Rituxan®) uvame ukunikezwa wodwa noma nge-chemotherapy.
• Ukwelashwa ngamakhemikhali: Imithi yokubulala amangqamuzana omdlavuza inganikezwa yodwa noma ihlanganiswe ne-immunotherapy.
• Ama-Corticosteroids: Uhlobo lwe-steroid, njenge-dexamethasone, lunganikezwa kanye nokwelashwa kokucindezela amasosha omzimba kanye ne-chemotherapy. Zisiza ekulweni nomdlavuza ngenkathi futhi zinciphisa imiphumela emibi yokwelashwa.
• Ukwelashwa okuqondiwe:Lokhu kwelashwa kusebenza ngokuvimbela amaprotheni asetshenziswa amangqamuzana omdlavuza ukuze akhule. I-Ibrutinib (Imbruvica®) kanye ne-zanubrutinib (Brukinsa®) yizindlela ezimbili zokwelapha eziqondiwe ezivunyelwe yi-U.S. Food and Drug Administration (FDA) ukwelapha i-WM.
• Ukufakelwa kwamaseli e-stem: Kulokhu, kufakelwa umongo wethambo ophilile ukuze kuthathelwe indawo umongo wethambo othintekile ngamaseli angajwayelekile. Lokhu akwenziwa njalo, futhi kwenziwa kuphela ezigulini ezikhethiwe.

Kufanele ngifune nini iseluleko sezokwelapha?

Uma utholakale une-WM, khuluma nodokotela wakho ngokushesha uma unezimpawu ezintsha noma uma ukhathazekile nganoma yiziphi izimpawu ezikhona. Uma usutholakale une-WM, kubalulekile ukubuza imibuzo ukuze uthole ukuthi yini ongayilindela. Nansi eminye imibuzo ongayibuza udokotela wakho:

• Kubaluleke kangakanani `(WM)`? Lokhu kuzoyithinta kanjani impilo yami?
• Yini okufanele ngiyilindele esikhathini esifushane neside?
• Ingabe ngidinga ukuqala ukwelashwa ngokushesha?
• Hlobo luni lokwelashwa olunconywayo?
• Yimiphi imiphumela emibi engalindelwa ekwelashweni?

Yini engingayilindela uma nginalesi simo?

Akuwona wonke umuntu onesifo esiqhubeka kancane kancane njenge-Waldenstrom's macroglobulinemia ofanayo. Ucwaningo lubonisa ukuthi ama-66%, noma abantu abangaphezu kwababili kwabathathu, basaphila eminyakeni eyi-10 ngemva kokuxilongwa. Kodwa-ke, izikhathi zokusinda zingahluka ngokweminyaka . Iningi labantu abangaphezu kweminyaka engama-65 (iqembu lobudala elivame ukutholakala linalesi sifo) lifa ngenxa yezimbangela ezingahlobene ne-WM.

Ziningi izinto ezithinta ukubikezelwa kwesifo sakho. Isibonelo:

• iminyaka yakho
• Imiphumela yokuhlolwa kwegazi
• Uhlobo lokuguquka kwezakhi zofuzo (ngezinye izikhathi ukungabikho kokuguquka kwe-`(MYD88)` kungabonisa umphumela omubi)

Uma unemibuzo ngesimo sakho, khuluma nodokotela wakho . Uyakwazi kangcono kanye nezici ezingase zithinte impilo yakho.

Ingabe kukhona engingakwenza ukuze ngizizwe ngingcono?

Ukuphila nesifo se-lymphoma esifana ne-Waldenstrom's macroglobulinemia kungasho ukwenza izinguquko ezinkulu empilweni yakho. Kubalulekile ukusebenzisa zonke izinsiza onazo. Khuluma nodokotela wakho mayelana nokudla okufanele ukudle kanye nezinyathelo zokuzinakekela okufanele uzithathe .

Ukuze ugweme ukuzizwa unesizungu, xhumana nabanye abanalesi sifo esingavamile. Ngisho noma uzizwa ngaleyo ndlela ekuqaleni lapho uthola ukuthi unalesi sifo, awuwedwa kulolu hambo . Buza udokotela wakho bese ujoyina amaqembu okusekela.

Ekugcineni, khumbula lokhu.

Abacwaningi abakayitholi ikhambi le-Waldenstrom macroglobulinemia (WM). Kodwa-ke, bathole izindlela zokwelapha eziningana ezenza ukuphila nalesi sifo kube lula . Abantu abaningi baphila nalesi sifo iminyaka eminingi ngaphandle kwezimpawu. Izindlela zokwelapha ezintsha zivumela abantu abane-WM ukuba baphile isikhathi eside kunanini ngaphambili, ngaphandle kokudela ikhwalithi yabo yokuphila.

Uma uthola lokhu kutholakala, ungesabi futhi ubuze udokotela wakho ngombono wesikhathi esifushane nowesikhathi eside. Bazokusiza ukuthi unqume uhlelo lokwelashwa olungcono kakhulu kuwe. Khumbula, ngeseluleko sezokwelapha esifanele kanye nokusekelwa, ungaphila ngempumelelo nalesi simo.

I- Waldenstrom macroglobulinemia, i-WM, umdlavuza wegazi, iphrotheni ye-IgM, i-hyperviscosity syndrome, umongo wamathambo, i-lymphoma