Iyini i-POEMS Syndrome? Uyafuna ukwazi ngalokhu futhi?

Wake wezwa nge-POEMS Syndrome? Lesi yisimo esingavamile esingavamile ukuzwakala ngaso. Kodwa-ke, kubalulekile ukwazi okuncane ngaso ngoba singathinta izinhlelo eziningi emzimbeni wethu ngesikhathi esisodwa. Kalula nje, yisimo esiyinkimbinkimbi esihlobene negazi lethu. Namuhla, sizoxoxa ngokuthi iyini i-POEMS Syndrome, yiziphi izimpawu zayo, itholakala kanjani, nokuthi ikhona yini indlela yokwelapha.

Iyini ngempela i-POEMS Syndrome?

Kulungile, ake sibone ukuthi iyini i-POEMS Syndrome. Lesi yisifo segazi esingavamile ngempela. Sinohlobo lweseli legazi elibizwa ngokuthi amaseli e-plasma emzimbeni wethu. Lapho kuvela i-POEMS syndrome, la maseli e-plasma ayahlukana futhi aphindaphindeke ngendlela engavamile, engalawuleki. La maseli e-plasma angavamile akhipha iphrotheni ekhethekile egazini lethu. Ibizwa ngokuthi i-monoclonal protein, noma i-M-protein ngamafuphi. Manje ungacabanga, akunjalo? Lapho la maseli e-plasma angavamile, i-M-protein, anda kakhulu egazini, angalimaza imizwa yethu kanye nezitho zangaphakathi.

Igama elithi POEMS lisuselwa ezincwadini zokuqala zezimpawu ezinhlanu eziyinhloko zalesi sifo:

• I-P - I- Polyneuropathy: Lokhu kubhekisela ekulimaleni kwemizwa kuwo wonke umzimba. Ku-POEMS, lo monakalo ungabangela ubuhlungu noma eminye imizwa engakhululekile (njengokuqaqamba, ukusha, njll.) emilenzeni yakho, ezingalweni, ematheni nasezizandleni.
• O - I-Organomegaly: Lokhu kusho ukuthi izitho zangaphakathi ziba zikhulu ngendlela engavamile. Umuntu one-POEMS angase abe nezitho ezikhuliswe njengesibindi, udende, kanye nama-lymph node.
• E - I-Endocrinopathy: Kalula nje, lokhu kuyinkinga yamahomoni. Ku-POEMS, inani lamahomoni akhishwa yizindlala zethu ze-endocrine lingaba elingavamile. Amanye amahomoni angase akhishwe ngokweqile noma ngamanani amancane.
• I-M - Iphrotheni ye-Monoclonal (i-M-protein): Lena yiphrotheni ekhethekile eqongelela egazini ku-POEMS. Lapho iseli elilodwa le-plasma lenza amakhophi amaningi (ama-clone) alo, ngalinye lalawo maseli lengeza le-M-protein egazini. Ekugcineni, inani le-M-protein egazini landa kakhulu.
• S - Izinguquko zesikhumba: Abantu abanezimbongi bangase babe nezinguquko ezahlukene zesikhumba, njengokufiphala kwesikhumba kanye nokuqina kwesikhumba.

I-POEMS syndrome yaziwa nangokuthi i-osteosclerotic myeloma , i-Crow-Fukase syndrome , i-PEP syndrome , kanye ne-Takatsuki syndrome .

Ubani okungenzeka kakhulu ukuthi athole i-POEMS syndrome?

I-POEMS syndrome iyisimo esingavamile kakhulu. Sithinta inani elincane labantu emhlabeni jikelele. Sivame ukubonakala kubantu abaneminyaka ephakathi kuka-50 no-60. Singathinta ubulili bobabili, kodwa kubonakala sengathi sivame kakhulu kwabesilisa.

Ziyini izimpawu ze-POEMS syndrome?

Njengoba sishilo ngaphambili, izinhlamvu ezibizwa ngokuthi i-POEMS zibonisa ezinye zezimpawu eziyinhloko. Okusho ukuthi, i-Polyneuropathy, i-Organomegaly, i-Endocrinopathy, ukuphakama kwe-M-protein, kanye nokushintsha kwesikhumba yizimpawu eziyinhloko. Cishe wonke umuntu one-POEMS une-polyneuropathy kanye nokuphakama kwe-M-protein. Ezinye izimpawu zingahluka kumuntu nomuntu.

Izimpawu ezibangelwa yi-polyneuropathy

Izimpawu ezibangelwa yilo monakalo wemizwa zivame kakhulu ku-POEMS. Ngokuvamile, ubuhlungu buzwakala kakhulu emilenzeni nasezinyaweni. Kodwa ngezinye izikhathi bungathinta izingalo nezandla. Nansi indlela ongase uzizwe ngayo uma unalesi simo:

• Ubuthakathaka
• Ukudlidliza
• Ukuba ndikindiki
• Ukuzwa kokuhlaba /izikhonkwane nezinaliti
• Umuzwa wokushisa

I-Organomegaly - ukwandiswa kwezitho zangaphakathi

Izinqubo zokuthatha izithombe ezenziwa odokotela zingabona ukuthi lezi zitho zikhulisiwe yini:

• Isibindi (Isibindi - `(Hepatomegaly)`)
• Ubende (i-Splenomegaly)
• Ama-lymph node (Ama-lymph node - `(Lymphadenopathy)`)

I-Endocrinopathy - izinkinga ze-hormone

Inkinga enkulu yama-hormone eza ne-POEMS ukwehla kwezinga lama-hormone ocansi. Lawa ma-hormone ocansi abizwa ngokuthi ``Testosterone``` kwabesilisa kanye ``Estrogen``` kwabesifazane. Ngaphezu kwalokho, ungase ubone izimpawu ezifana nalezi:

• Izinkinga zokulawula amazinga kashukela egazini (isib. isifo sikashukela)
• Izinkinga ze-thyroid (isb. i-Hypothyroidism)
• Isifo sika-Addison (Ukungasebenzi kahle kwe-adrenal - `(Isifo sika-Addison)`)

Amadoda angase abhekane nokuncipha kokuzala kanye nokukhula kwamabele (i-Gynecomastia).

Kwabesifazane , ukuya esikhathini kungase kuphele (`(Amenorrhea)`), amabele angase akhule. Ngesikhathi sokukhulelwa, i-hormone `(Prolactin)`, esiza ekukhiqizeni ubisi, ingakhiqizwa ngokweqile. Ngakho-ke, kungenzeka ukuthi ubisi luphume emabeleni ngisho noma ungakhulelwe.

I-M-Protein Eyandisiwe

Isici esibalulekile se-POEMS izinga eliphakeme le-M-protein egazini. Lokhu kudlula kwe-M-protein kungaholela ekwakhekeni kwezimila egazini. Futhi, ngenxa yezinga eliphezulu le-M-protein,Amathambo angaba makhulu futhi aqine (i-osteosclerosis) .

Izinguquko zesikhumba

Izinguquko ezahlukene zesikhumba zingavela, njenge:

• Isikhumba esimnyama
• Isikhumba esikhudlwana
• Izinzipho ezimhlophe
• Ukukhula kwezinwele ebusweni nasemilenzeni
• Ukukhula kwemithambo yegazi ebukeka njengama-cherry amancane esikhumbeni sesifuba

Ezinye izimpawu

Ngaphezu kwalokhu, ezinye izimpawu zingabonakala:

• Ukuvuvukala kwezandla nezinyawo
• Ukwehlisa isisindo ngaphandle kwesizathu
• Ubuhlungu emathanjeni nasemalungeni
• Ukukhubazeka kokubona
• Ubunzima bokuphefumula
• Ukukhathala
• Imfiva

Abanye abantu abane-POEMS syndrome bangase babe nesimo esibizwa ngokuthi i-Castleman Disease (CD), esithinta ama-lymph node. Uma unezimpawu ze-Castleman Disease, udokotela wakho angase asole i-POEMS.

Yini ebangela i-POEMS syndrome?

Uma une-POEMS, uhlangabezana nezimpawu nemiphumela ehlukahlukene ngenxa yokuphazamiseka kweseli le-plasma le-monoclonal . Kalula nje, iseli le-plasma elingavamile lenza amakhophi amaningi kakhulu alo. Uma la maseli eba maningi, kulapho angadala khona umonakalo omkhulu ezicutshini. La maseli engeza i-M-protein egazini, okwenza inani le-M-protein egazini likhuphuke. Yila maseli angaphezulu kanye ne-M-protein elimaza izinhlelo ezahlukene emzimbeni.

Kodwa-ke, ososayensi abakakazi kahle ukuthi kungani la maseli e-plasma eqala ukukhula ngendlela engavamile kwasekuqaleni.

Abacwaningi bathole ukuthi iphrotheni ebizwa ngokuthi i-Vascular Endothelial Growth Factor (VEGF) ingase ihileleke. Iningi labantu abane-POEMS syndrome linamazinga aphezulu e-VEGF egazini labo. Ucwaningo ngobudlelwano phakathi kwe-VEGF kanye ne-POEMS syndrome lusaqhubeka.

I-POEMS syndrome itholakala kanjani? (Ukuxilongwa)

Udokotela wakho uzoqala ngokukubuza ngomlando wakho wezokwelapha kanye nezimpawu zamanje. Ngemuva kwalokho, uzokwenza ukuhlolwa ngokomzimba ukuhlola izimpawu ze-POEMS syndrome.

Ngaphezu kwalokho, udokotela angenza nezivivinyo eziningana, njenge:

• Ukuhlolwa kwegazi nomchamo: Lokhu kuhlolwa kungasiza ekuhloleni amazinga aphezulu e-M-protein noma i-VEGF egazini lakho noma emchameni. Kungahlola futhi inani lamaseli egazi elingajwayelekile noma amaseli egazi abukeka engajwayelekile. Lokhu kungaba izimpawu ze-POEMS.
• Ukuhlolwa kwezithombe: Ukuhlolwa okufana ne-X-ray kanye ne-CT scan kungabonisa ukuthi amathambo akho aqinile noma aqinile. Lezi zinguquko zingenzeka uma amazinga e-M-protein ephakanyisiwe.
• Ukuhlolwa komnkantsha wethambo: Lokhu kungathola ukuthi unamaseli e-plasma abukeka engajwayelekile emnkantsha wakho wethambo, noma ukuthi inani lamaseli e-plasma liphezulu kakhulu.
• I-Electromyogram (EMG): I-EMG ukuhlolwa okulinganisa ukusebenza kwezinzwa. Kungasiza udokotela wakho ukuthola i-polyneuropathy.

Kuye ngezimpawu zakho, udokotela wakho angase enze nokuhlolwa okwengeziwe, njengokuhlolwa kokuphefumula, ukuhlolwa kwe-echocardiograms, noma ukuhlolwa kwe-endocrine.

Uma utholakale unesifo esibizwa ngokuthi i-Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) , udokotela wakho angase akuhlole ukuze abone ukuthi une-POEMS syndrome.

Iphathwa noma yelashwa kanjani i-POEMS syndrome?

Akukho ukwelashwa kwe-POEMS syndrome. Kodwa-ke, ukwelashwa kungasiza ekulawuleni nasekuphatheni izimpawu. Izinketho zokwelapha zifaka:

• Ukwelashwa ngemisebe: Lokhu kuhilela ukuqondisa amazinga aphezulu emisebe kumaseli e-plasma angajwayelekile ukuze awabhubhise.
• Ukwelashwa ngamakhemikhali: Imithi yokulwa nomdlavuza, eyaziwa nangokuthi "i-Chemotherapy," isetshenziselwa ukubulala noma ukubambezela ukukhula kwamaseli e-plasma angajwayelekile. Ukwelashwa ngamakhemikhali nge-POEMS kufana nokwelashwa kwe-multiple myeloma. Lokhu kwelashwa kusebenza kahle kakhulu futhi ngokuvamile akubangeli imiphumela emibi kakhulu.
• Ukufakelwa kweseli le-Autologous stem: Le nqubo ithatha indawo yamaseli akho e-plasma angajwayelekile ngamaseli e-stem anempilo. Amaseli e-stem angamaseli akhula abe amaseli avuthiwe njengamaseli e-plasma. Kulokhu kufakelwa, udokotela wakho uqala ngokususa amanye amaseli e-stem anempilo emzimbeni wakho. Bese, basebenzisa i-chemotherapy noma ukwelashwa ngemisebe ukubhubhisa amaseli e-plasma angajwayelekile. Ngemva kokwelashwa, amaseli e-stem asusiwe abuyiselwa emzimbeni wakho. Abese ekhula abe amaseli e-plasma anempilo.
• Ukwelashwa ngokomzimba: Uma unenkinga yokuhamba ngenxa ye-polyneuropathy, lokhu kwelashwa kungasiza.

Ngaphezu kwalokho, udokotela angase anikeze imithi efana nama-steroid ukuze anciphise izimpawu kanye nama-diuretics (amaphilisi amanzi) ukuze kuncishiswe ukuvuvukala.

Yiziphi izinkinga ezihlobene ne-POEMS syndrome?

Nge-POEMS syndrome, ukulimala kwemizwa kanye nobuthakathaka kunganda ngokuhamba kwesikhathi. Kungenza kube nzima nokuhambahamba. Uma uketshezi oluningi luqoqana eduze kwamaphaphu noma ngaphakathi kwesifuba, kungabangela ubuhlungu besifuba kanye nobunzima bokuphefumula.

Kodwa khumbula,Ukwelashwa okuphumelelayo kungamisa noma kunciphise ukulimala kwezinzwa. Kungasiza futhi ekunciphiseni ezinye izimpawu. Ngisho nokulimala kwezinzwa kungaphola kancane kancane ngokuhamba kwesikhathi.

Ingabe zikhona izici eziyingozi ze-POEMS syndrome?

Okwamanje azikho izici ezitholakele zengozi yokuthuthukisa i-POEMS syndrome.

Ingabe i-POEMS syndrome ingavinjelwa?

Ngeshwa, i-POEMS syndrome ayinakuvinjelwa.

Iyini i-prognosis yabantu abane-POEMS syndrome?

Impilo yakho incike ezintweni eziningi, okuhlanganisa ukuthi yiziphi izingxenye zomzimba wakho ezithintekayo, ukuthi lesi sifo sisheshe sitholakale kanjani nokuthi siqala kanjani ukwelashwa, kanye nendlela osabela ngayo ekwelashweni.

Kubalulekile ukuthola ukwelashwa kwe-POEMS. I-POEMS syndrome ingaba yingozi empilweni uma ingelashwa. Ngakolunye uhlangothi, ukwelashwa okuphumelelayo okubhubhisa amaseli e-plasma angajwayelekile kungakusiza uphile isikhathi eside. Ngaphezu kwalokho, ukwelashwa kunganciphisa izimpawu futhi kuthuthukise ikhwalithi yokuphila kwakho.

Izinga lokusinda elimaphakathi labantu abaningi abane-POEMS licishe libe yiminyaka eyi-14. Lokho kusho ukuthi cishe ingxenye yabantu iphila iminyaka engaphansi kweyi-14, kanti enye ingxenye iphila isikhathi eside. Isikhathi sakho sokuphila sincike kokuhlangenwe nakho kwakho kwesifo kanye nokwelashwa.

Buza udokotela wakho ngekusasa lakho njengomuntu ophila ne-POEMS syndrome.

Kufanele ubonane nini nodokotela mayelana ne-POEMS syndrome?

Uma uhlangabezana nanoma yiziphi izimpawu ezihlobene ne-POEMS syndrome, ikakhulukazi ubuthakathaka ezingalweni nasemilenzeni , bona udokotela ngokushesha.

Yimiphi imibuzo okufanele ngiyibuze udokotela wami mayelana ne-POEMS syndrome?

Ungabuza imibuzo efana nale:

• Isimo sami sibi kangakanani?
• Yiziphi izindlela zokwelapha enginazo?
• Yini okufanele ngiyenze ukuze nginciphise noma ngilawule imiphumela emibi?
• Yiziphi izinsizakalo zokusekela ezitholakalayo kimi?
• Ingabe ngidinga ukubona udokotela ochwepheshe bezinkinga zamaseli e-plasma ukuze angiqondise ekwelashweni kwami?

Ngingabuyela nini emisebenzini yami evamile uma ngine-POEMS syndrome?

I-POEMS syndrome ingathinta ikhono lakho lokwenza imisebenzi yakho yansuku zonke. Udokotela wakho uzochaza ukuthi lesi simo sizothinta kanjani impilo yakho yansuku zonke.

Ingabe i-POEMS syndrome iyisifo sokuzimela?

I-POEMS iyi -paraneoplastic syndrome.Akusona isifo sokuzivikela komzimba. I-paraneoplastic syndrome iqoqo lezimpawu ezibangelwa yisimo sezokwelapha esingaphansi. Ku-POEMS, inkinga eyisisekelo ukuthi amangqamuzana e-plasma angajwayelekile akhiqiza i-M-protein eningi egazini (isifo se-monoclonal plasma cell). Izimpawu ze-POEMS zibangelwa amangqamuzana e-plasma angajwayelekile kanye ne-M-protein elimaza izicubu zomzimba.

Ingabe i-POEMS syndrome ifana ne-multiple myeloma?

I-multiple myeloma ingumdlavuza othinta amangqamuzana e-plasma. I-POEMS syndrome iqoqo lezimpawu ezibangelwa amangqamuzana e-plasma angajwayelekile kanye nokwanda kwe-M-protein. Izinga lokusinda kwe-POEMS syndrome lingcono kunesikhathi sokuphila se-multiple myeloma.

Kodwa-ke, ukwelashwa ngamakhemikhali kwe-POEMS kanye ne-multiple myeloma kuvame ukufana. Ezinye zezimpawu zingase zifane.

I-POEMS syndrome iyisifo esiyinkimbinkimbi. Kungathatha udokotela wakho isikhathi esithile ukuthola ukuthi izimpawu zakho zibangelwa yi-POEMS syndrome hhayi esinye isimo. Uma usuxilongiwe, sebenzisana eduze nethimba lakho lezokwelapha ukuze uthole ukuthi yiziphi izindlela zokwelapha ezizokunikeza impumuzo enkulu ezimpawini zakho. Buza udokotela wakho ngezinhlangano noma amaqembu okusekelana asiza abantu ngabanye kanye nemindeni ebhekene nokuxilongwa njenge-POEMS syndrome.

Umlayezo Wokuya Nawe Ekhaya

Kulungile, ngakho-ke sesikhulume kakhulu nge-POEMS Syndrome namuhla. Into ebalulekile ukuqonda ukuthi lesi yisimo esingavamile, kodwa esibucayi . Singathinta izinhlelo eziningi emzimbeni wethu.

• Igama elithi POEMS lisuselwa ezinhlamvini zokuqala zamagama esiNgisi ezimpawu ezinhlanu eziyinhloko (ukuphazamiseka kwemizwa, ukwandiswa kwezitho zangaphakathi, izinkinga zamahomoni, ukwanda kwe-M-protein, kanye nokushintsha kwesikhumba).
• Imbangela eyinhloko yalokhu amaseli e-plasma angajwayelekile kanye ne-M-protein ayikhiqizayo.
• Nakuba kungekho ikhambi eliphelele, kunezindlela zokwelapha ezisebenzayo zokulawula izimpawu nokuthuthukisa ikhwalithi yokuphila.
• Kubaluleke kakhulu ukuxilonga lesi sifo bese uqala ukwelashwa kusenesikhathi .

Uma unezinye zalezi zimpawu, sicela ufune iseluleko sezokwelapha. Ungesabi, ngolwazi olufanele kanye nokusekelwa kwezokwelapha, ungabhekana nalesi simo.

I- POEMS syndrome, izifo zegazi, ukulimala kwemizwa, izinkinga zamahomoni, i-M-protein, izinguquko zesikhumba, amaseli e-plasma