Xa usiva eli gama, mhlawumbi ucinga ngeePoems, akunjalo? Kodwa eli asilobali malunga neembongo. I-POEMS syndrome sisifo segazi esingaqhelekanga esinokuchaphazela imithambo-luvo yomzimba wethu, amalungu ahlukeneyo, inkqubo yeehomoni, kunye nolusu ngaxeshanye. Kunzima kancinci ukuyixilonga, kuba iimpawu ezinxulumene nayo zinokufana neempawu zezinye izifo ezininzi. Yiyo loo nto kubaluleke kakhulu ukuqaphela oku.
Kutheni oku kubizwa ngokuba yiMIBONGO?
Igama layo livela koonobumba bokuqala beempawu ezintlanu eziphambili zesifo. Masibone ukuba ziintoni.
| Ileta | Intsingiselo kunye nenkcazo elula |
|---|---|
| P | I-Polyneuropathy: Oku kubhekisa ekonakaleni kwemithambo-luvo emzimbeni wonke, ingakumbi emalungwini. |
| O | I-Organomegaly: Ukwanda kwamalungu athile emzimbeni, njengesibindi, i-spleen, okanye ii-lymph nodes, ngaphaya kobukhulu obuqhelekileyo. |
| E | I-Endocrinopathy: Ukuphazamiseka kokusebenza kwamadlala (inkqubo ye-endocrine) emzimbeni wethu okuvelisa iihomoni. |
| M | Iproteni ye-monoclonal: Ikwabizwa ngokuba yi-M-protein. Amanqanaba aphezulu eproteni engaqhelekanga egazini. |
| S | Utshintsho lolusu:Izinto ezinje ngokutshintsha kombala wesikhumba kunye nokuqina kolusu. |
Kukho amaxesha apho kuthatha iinyanga zovavanyo ukuze kufunyanwe le meko, kuba ezi mpawu zinokubhidaniswa lula nezinye izifo ezininzi xa zithathwa ngokwahlukeneyo.
Ziziphi iimpawu ze-POEMS syndrome?
Ngoku makhe sijonge ngokweenkcukacha iimpawu zenxalenye nganye yeMIBONGO ekuthethwe ngayo apha ngasentla.
- P - Umonakalo wemithambo-luvo (iPolyneuropathy): Le yinto yokuqala abantu abaninzi abayiqaphelayo. Iinzwane kunye neenyawo zinokuziva zingabalulekanga, zihlaba, kwaye zinokuziva zitshisa okanye zibuhlungu kakhulu . Ekuhambeni kwexesha, le meko inokusasazeka ezandleni. Amandla ezandleni ayancipha, kwaye usenokungakwazi ukubamba nantoni na esandleni sakho. Kwiimeko ezinzima, kunokuba nzima ukuma, ukuhamba, kwanokuphefumla.
- O - I-Organomegaly: Isibindi sakho, i-spleen, okanye ii-lymph nodes zinokudumba. Oku kunokubangela iimpawu ezifana nokuziva ugcwele, iintlungu zesisu okanye ukudumba, kunye nokudinwa kakhulu .
- E - Utshintsho kwiHormonal (Endocrinopathy): Utshintsho kwiHormonal lunokubangela izinto ezifana nokuncipha komnqweno wesondo, ukuphuma kobisi emabeleni (kubasetyhini nakumadoda), kunye nokudumba kwezandla neenyawo .
- I-M - I-M-protein engaqhelekanga: Le protein inokuqokelelana egazini ize ibangele ukuba amathambo abe buthathaka okanye onakale , nto leyo enokubangela iintlungu zamathambo.
- S - Utshintsho lolusu: Ulusu lunokuba mnyama kunesiqhelo. Ngamanye amaxesha ulusu lunokuqina, kwaye amabala abomvu angabonakala esifubeni. Kunokubakho nokukhula kweenwele ebusweni nasemilenzeni, kwaye iinzipho zingaba mhlophe .
Kutheni kusenzeka into enje?
Isizathu esichanekileyo soku asikaziwa okwangoku, kodwa oogqirha nabaphandi bakholelwa ukuba kunxulumene nokusebenza okungaqhelekanga kohlobo lweseli emzimbeni wethu olubizwa ngokuba zii-plasma cells .
Ngamafutshane, ezi seli zeplasma ezingaqhelekanga zikhupha i-M-protein esithethe ngayo egazini. Ezi seli ezingaqhelekanga kunye ne-M-protein ziyadibana ukuze zonakalisa iindawo ezahlukeneyo zomzimba. Ukongeza, ezi zigulana zikwanamanqanaba aphezulu eproteni ebizwa ngokuba yiVascular Endothelial Growth Factor (VEGF) egazini lazo. Le protein yeVEGF ibangela ukuba ulwelo luphume kwimithambo yegazi luye kwizicubu ezingqongileyo. Olu lwelo lungaphezulu lubangela ukudumba, ukwanda kwamalungu omzimba, kunye nomonakalo wemithambo-luvo.
Ngaba le meko inokuba yinto enzulu?
Ewe, i-POEMS syndrome ngamanye amaxesha inokuba yingozi ngokukhawuleza, nto leyo ekhokelela kwiingxaki ezisongela ubomi.
Ezinye zazo zezi:
- I-Capillary Leak Syndrome: Ukungasebenzi kakuhle kwamalungu omzimba kunokwenzeka ngenxa yokuvuza kolwelo olugqithisileyo oluvela kwimithambo yegazi.
- Isifo Semiphunga Esithintelweyo: Ubunzima bokuphefumla ngenxa yokuba imiphunga ayikwazi ukwanda kakuhle.
- Uxinzelelo lwegazi oluphezulu lwemiphunga: Uxinzelelo olugqithisileyo kwimithambo yegazi ethwala igazi ukuya emiphungeni.
Ngoko ke, ukuba uneempawu, kubaluleke kakhulu ukufuna iingcebiso zonyango kwaye uqale unyango ngokukhawuleza.
Ugqirha uyifumana njani le nto?
Xa usiya kugqirha, uya kumamela ngenyameko iimpawu zakho. Emva koko uya kuhlola umzimba wakho aze akucebise ukuba wenze iimvavanyo ezininzi ukuqinisekisa ukuba unesifo.
| Uvavanyo | Ujonga ntoni? |
|---|---|
| Uvavanyo lwegazi nomchamo | Amanqanaba e-M-protein okanye e-VEGF ajongwa ukuba awanazo iiseli ze-plasma ezingaqhelekanga. |
| Uvavanyo lwemifanekiso (i-X-ray, i-CT scan) | Bajonge ukubona ukuba i-M-protein ibangele umonakalo emathanjeni na. |
| iBone Marrow Biopsy | Ujonga iiseli zeplasma ezingaqhelekanga kumongo wethambo. |
| I-Electromyogram (EMG) | Umsebenzi wakho wemithambo-luvo uyalinganiswa. |
Ukongeza, iimvavanyo zokuphefumla, ii-echocardiograms, kunye novavanyo lweehomoni zinokwenziwa kuxhomekeke kwiimpawu zakho.
Zithini iindlela zonyango zoku?
Eyona njongo yonyango kukulawula iimpawu kunye neeseli zeplasma ezingaqhelekanga. Kukho iindlela ezahlukeneyo zonyango zoku.
- I-Chemotherapy: Olu nyango lutshabalalisa iiseli ze-plasma ezingaqhelekanga okanye luyekise ukukhula kwazo.
- Unyango lwe-Immunotherapy: La mayeza anceda ekusebenziseni inkqubo yakho yomzimba yokuzikhusela ukutshabalalisa iiseli ze-plasma ezingaqhelekanga.
- Unyango ngemitha: Unyango ngemitha lutshabalalisa iiseli zeplasma ezingaqhelekanga.
- Ukufakelwa kweeseli ze-Autologous stem: Oku kuquka ukususa iiseli ze-plasma ezingaqhelekanga emzimbeni wakho uze uzifake iiseli ze-stem eziphilileyo endaweni yazo.
- Unyango lomzimba: Ukonakala kwemithambo-luvo kunokwenza kube nzima ukuhamba nokwenza imisebenzi yemihla ngemihla. Uqheliso lonyango lomzimba lunokukunceda ufumane amandla kwakhona.
- Inkxaso yengqondo: Ukuphila nesifo esingaqhelekanga nesinganyangekiyo esifana nesi kunokuba nzima kakhulu engqondweni. Oku kunokukhokelela kwiimeko ezifana nokuxhalaba nokudakumba. Ukufuna uncedo lomcebisi kunokunceda ukulawula olu xinzelelo.
Ungaphila ixesha elingakanani nale sifo?
Le yingxaki abantu abaninzi abanayo. Ukuba isifo sifunyenwe kwangethuba kwaye sinyangwa ngokufanelekileyo, abantu abaninzi banokuphila iminyaka eli-10 nangaphezulu emva kokuba befunyenwe. Amayeza amatsha ngoku ayafumaneka ukunyanga esi sifo. Nangona kunjalo, oku kunokwahluka kumntu nomntu. Umntu ofanelekileyo onokufumana ingcaciso engcono kunye nokuqonda imeko yakho ngugqirha wakho. Ngoko thetha naye ngokukhululekileyo ngayo.
Umyalezo Wokuya Ekhaya
- I-POEMS syndrome sisifo segazi esingaqhelekanga kodwa esinzulu esichaphazela iinkqubo ezahlukeneyo zomzimba.
- Eli gama livela kwiileta zokuqala zeempawu zalo eziphambili: iPolyneuropathy , i-Organomegaly , i-Endocrinopathy , i-M -protein, kunye notshintsho lwesikhumba .
- Ukuba une-nausea engapheliyo, ukudumba, okanye utshintsho lolusu kwimilenze yakho, musa ukuyityeshela.
- Ukufumanisa isifo kunokuba nzima kwaye kuthathe ixesha elide, kodwa ngoku kukho unyango olusebenzayo kakhulu lokulawula imeko.
- Ukuba uneempawu ezikrokrisayo, bona ugqirha wakho ngokukhawuleza ukuze akunike icebiso.
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