Yintoni i-POEMS Syndrome? Ngaba nawe uyafuna ukwazi ngale nto?

Ngaba wakha weva nge-POEMS Syndrome? Le yimeko engaqhelekanga engaqhelekanga ukuviwa ngayo. Nangona kunjalo, kubalulekile ukwazi okuncinci ngayo kuba inokuchaphazela iinkqubo ezininzi emzimbeni wethu ngaxeshanye. Ngamafutshane, yimeko enzima enxulumene negazi lethu. Namhlanje, siza kuthetha ngokuba yintoni i-POEMS Syndrome, zithini iimpawu zayo, ifunyaniswa njani, kwaye ingaba ikhona na unyango.

Yintoni kanye kanye i-POEMS Syndrome?

Kulungile, masibone ukuba yintoni i-POEMS Syndrome. Esi sisifo segazi esingaqhelekanga. Sinohlobo lweeseli zegazi ezibizwa ngokuba zii-plasma cells emzimbeni wethu. Xa kuvela i-POEMS syndrome, ezi seli ze-plasma ziyahlukana kwaye zande ngendlela engaqhelekanga, ngokungalawulekiyo. Ezi seli ze-plasma ezingaqhelekanga zikhupha iproteni ekhethekileyo egazini lethu. Ibizwa ngokuba yi-monoclonal protein, okanye i-M-protein ngamafutshane. Ngoku ungacinga, akunjalo? Xa ezi seli ze-plasma ezingaqhelekanga, i-M-protein, zanda kakhulu egazini, zingonakalisa imithambo-luvo yethu kunye namalungu angaphakathi.

Igama elithi POEMS livela kwiileta zokuqala ezintlanu zeempawu eziphambili zesi sifo:

• P - I- Polyneuropathy: Oku kubhekisa kumonakalo kwimithambo-luvo emzimbeni wonke. KwiMIBONGO, lo monakalo unokubangela iintlungu okanye ezinye iimvakalelo ezingathandekiyo (ezifana nokurhawuzelela, ukutsha, njl.njl.) emilenzeni yakho, ezingalweni, ezinyaweni nasezizandleni.
• O - I-Organomegaly: Oku kuthetha ukuba amalungu angaphakathi aba makhulu ngendlela engaqhelekanga. Umntu one-POEMS unokuba namalungu amakhulu anjengesibindi, i-spleen, kunye ne-lymph nodes.
• E - I-Endocrinopathy: Ngamafutshane, le yingxaki yehomoni. Kwi-POEMS, ubungakanani beehomoni ezikhutshwa zii-endocrine glands zethu bunokuba bungaqhelekanga. Ezinye iihomoni zinokukhutshwa ngokugqithisileyo okanye ngobuninzi obuncinci.
• I-M - Iproteni ye-Monoclonal (i-M-protein): Le yiproteni ekhethekileyo eqokelelana egazini kwi-POEMS. Xa iseli enye yeplasma isenza iikopi ezininzi (ii-clones) zayo, nganye kwezo seli yongeza le protein ye-M egazini. Ekugqibeleni, ubungakanani beproteni ye-M egazini buyanda kakhulu.
• S - Utshintsho lolusu: Abantu abaneeMBONGO banokufumana utshintsho oluhlukeneyo lolusu, olufana nokuba mnyama kolusu kunye nokuqina kolusu.

I-POEMS syndrome ikwaziwa ngokuba yi -osteosclerotic myeloma , i-Crow-Fukase syndrome , i-PEP syndrome , kunye ne -Takatsuki syndrome .

Ngubani onokuthi abe nesifo se-POEMS?

I-POEMS syndrome yimeko engaqhelekanga kakhulu. Ichaphazela inani elincinci labantu kwihlabathi liphela. Ixhaphake kakhulu kubantu abaneminyaka ephakathi kwama-50 nama-60 ubudala. Ingachaphazela zombini izini, kodwa ibonakala ixhaphake kakhulu kumadoda.

Ziziphi iimpawu ze-POEMS syndrome?

Njengoko besitshilo ngaphambili, oonobumba abakwigama elithi POEMS babonisa ezinye zeempawu eziphambili. Oko kukuthi, i-Polyneuropathy, i-Organomegaly, i-Endocrinopathy, ukuphakama kwe-M-protein, kunye notshintsho lolusu zezona mpawu ziphambili. Phantse wonke umntu one-POEMS une-polyneuropathy kunye nokuphakama kwe-M-protein. Ezinye iimpawu zinokuhluka kumntu nomntu.

Iimpawu ezibangelwa yi-polyneuropathy

Iimpawu ezibangelwa yile ngxaki yemithambo-luvo zezona zixhaphakileyo kwi-POEMS. Ngokuqhelekileyo, intlungu ivakala kakhulu emilenzeni nasezinyaweni. Kodwa ngamanye amaxesha inokuchaphazela iingalo nezandla. Nantsi indlela onokuziva ngayo xa unesi sifo:

• Ubuthathaka
• Ukungqungqa
• Ukuba ndindisholo
• Imvakalelo yokuhlaba /yokuhlaba iinaliti
• Imvakalelo yokutsha

I-Organomegaly - ukwandiswa kwamalungu angaphakathi

Iinkqubo zokuthatha imifanekiso ezenziwa ngoogqirha zinokubona ukuba la malungu akhulisiwe na:

• Isibindi (Isibindi - `(Hepatomegaly)`)
• I-spleen (i-spleen)
• Ama-lymph nodes (Ama-lymph nodes - `(Lymphadenopathy)`)

I-Endocrinopathy - iingxaki zehomoni

Ingxaki ephambili yehomoni eza ne-POEMS kukwehla kwenqanaba leehomoni zesini. Ezi homoni zesini zibizwa ngokuba yi-``Testosterone``` kumadoda kunye ne-``Estrogen``` kubafazi. Ukongeza, unokubona iimpawu ezifana nezi:

• Iingxaki zokulawula amanqanaba eswekile egazini (umz. isifo seswekile)
• Iingxaki ze-thyroid (umz. i-Hypothyroidism)
• Isifo sika-Addison (Ukungasebenzi kakuhle kwe-adrenal - `(Isifo sika-Addison)`)

Amadoda anokufumana ubunzima bokuzala kunye nokukhula kwamabele (iGynecomastia).

Kwabasetyhini , ukuya exesheni kunokuyeka (`(Amenorrhea)`), amabele anokukhula. Ngexesha lokukhulelwa, ihomoni `(Prolactin)`, enceda ekuveliseni ubisi, inokuveliswa ngokugqithisileyo. Ke ngoko, kunokwenzeka ukuba ubisi luphume kwiingono nokuba awukhulelwe.

I-M-Protein eyandisiweyo

Uphawu oluphambili lwe-POEMS linqanaba eliphezulu le-M-protein egazini. Oku kugqithiswa kwe-M-protein kunokukhokelela ekwakhiweni kweethumba egazini. Kwakhona, ngenxa yenqanaba eliphezulu le-M-protein,Amathambo anokuba makhulu aze aqine (i-osteosclerosis) .

Utshintsho lolusu

Utshintsho oluhlukeneyo lolusu lunokwenzeka, olufana nolu:

• Ulusu olumnyama
• Ulusu olutyebileyo
• Iinzipho ezimhlophe
• Ukukhula kweenwele ebusweni nasemilenzeni
• Ukukhula kwemithambo yegazi efana neetsheri ezincinci eluswini lwesifuba

Ezinye iimpawu

Ukongeza koku, ezinye iimpawu zingabonakala:

• Ukudumba kwezandla neenyawo
• Ukunciphisa umzimba ngaphandle kwesizathu
• Intlungu emathanjeni nasemalungeni
• Ukukhubazeka kokubona
• Ubunzima bokuphefumla
• Ukudinwa
• Ifiva

Abanye abantu abane-POEMS syndrome banokuba nesifo esibizwa ngokuba yiCastleman Disease (CD), esichaphazela ii-lymph nodes. Ukuba uneempawu zeCastleman Disease, ugqirha wakho unokurhanela i-POEMS.

Yintoni ebangela i-POEMS syndrome?

Xa une-POEMS, ufumana iimpawu ezahlukeneyo kunye nemiphumo ngenxa yesifo se-monoclonal plasma cell . Ngamafutshane, iseli ye-plasma engaqhelekanga yenza iikopi ezininzi kakhulu. Okukhona zininzi ezi seli, kokukhona zinokubangela umonakalo omkhulu kwizicwili. Ezi seli zongeza i-M-protein egazini, nto leyo ebangela ukuba inani le-M-protein egazini linyuke. Zizi seli ezigqithisileyo kunye ne-M-protein ezonakalisa iinkqubo ezahlukeneyo emzimbeni.

Nangona kunjalo, izazinzulu azikayazi kakuhle ukuba kutheni ezi seli zeplasma ziqala ukukhula ngendlela engaqhelekanga kwasekuqaleni.

Abaphandi bafumanise ukuba iproteni ebizwa ngokuba yiVascular Endothelial Growth Factor (VEGF) isenokuba ibandakanyeka. Uninzi lwabantu abane-POEMS syndrome banamanqanaba aphezulu e-VEGF egazini labo. Uphando malunga nolwalamano phakathi kwe-VEGF kunye ne-POEMS syndrome lusaqhubeka.

Ifunyanwa njani i-POEMS syndrome? (Ukuxilongwa)

Ugqirha wakho uza kuqala akubuze ngembali yakho yezonyango kunye neempawu zakho zangoku. Emva koko, uza kwenza uvavanyo lomzimba ukujonga iimpawu ze-POEMS syndrome.

Ukongeza, ugqirha angenza iimvavanyo ezininzi, ezifana nezi:

• Uvavanyo lwegazi nomchamo: Olu vavanyo lunokunceda ukujonga amanqanaba aphezulu e-M-protein okanye i-VEGF egazini okanye kumchamo wakho. Lunokujonga nokubalwa kweeseli zegazi okungaqhelekanga okanye iiseli zegazi ezibonakala zingaqhelekanga. Ezi zinokuba ziimpawu ze-POEMS.
• Uvavanyo lokucinga: Uvavanyo olufana ne-X-ray kunye ne-CT scans lunokubonisa ukuba amathambo akho aqinile okanye aqinile. Olu tshintsho lunokwenzeka xa amanqanaba e-M-protein ephakanyisiwe.
• Uvavanyo lwe-bone marrow biopsy: Oku kunokufumanisa ukuba uneeseli ze-plasma ezibonakala zingaqhelekanga kwi-bone marrow yakho, okanye ukuba inani leeseli ze-plasma liphezulu kakhulu.
• I-Electromyogram (EMG): I-EMG luvavanyo olulinganisa ukusebenza kwemithambo-luvo. Inokunceda ugqirha wakho ukuba afumanise i-polyneuropathy.

Ngokuxhomekeke kwiimpawu zakho, ugqirha wakho unokwenza nezinye iimvavanyo, ezifana novavanyo lokuphefumla, i-echocardiograms, okanye uvavanyo lwe-endocrine.

Ukuba ufunyaniswe unesifo esibizwa ngokuba yiChronic Inflammatory Demyelinating Polyneuropathy (CIDP) , ugqirha wakho unokukuvavanya ukuze abone ukuba unayo na i-POEMS syndrome.

Iphathwa okanye inyangwa njani i-POEMS syndrome?

Akukho nyango lwe-POEMS syndrome. Nangona kunjalo, unyango lunokunceda ukulawula nokulawula iimpawu. Iindlela zonyango ziquka:

• Unyango ngemitha: Oku kuquka ukukhokela amanqanaba aphezulu emitha kwiiseli zeplasma ezingaqhelekanga ukuze zitshabalalise.
• Unyango lweKhemotherapy: Amayeza okulwa nomhlaza, akwabizwa ngokuba yi-"Chemotherapy," asetyenziselwa ukubulala okanye ukucothisa ukukhula kweeseli zeplasma ezingaqhelekanga. Unyango lweKhemotherapy lwe-POEMS lufana nonyango lwe-multiple myeloma. Olu nyango lusebenza kakhulu kwaye alusoloko lubangela iziphumo ebezingalindelekanga.
• Ukufakelwa kweeseli ze-Autologous stem: Le nkqubo ithatha indawo yeeseli zakho ze-plasma ezingaqhelekanga ngeeseli ze-stem eziphilileyo. Iiseli ze-stem ziiseli ezikhula zibe ziiseli ezivuthiweyo njengeeseli ze-plasma. Kolu kufakelwa, ugqirha wakho uqala ngokususa ezinye iiseli ze-stem eziphilileyo emzimbeni wakho. Emva koko, basebenzisa i-chemotherapy okanye unyango lwe-radiation ukutshabalalisa iiseli ze-plasma ezingaqhelekanga. Emva konyango, iiseli ze-stem ezisusiweyo zibuyiselwa emzimbeni wakho. Emva koko zikhula zibe ziiseli ze-plasma eziphilileyo.
• Unyango lomzimba: Ukuba unengxaki yokuhamba ngenxa ye-polyneuropathy, olu nyango lunokukunceda.

Ukongeza, ugqirha angakunika amayeza anjengee-steroids ukunciphisa iimpawu kunye ne-diuretics (iipilisi zamanzi) ukunciphisa ukudumba.

Ziziphi iingxaki ezinxulumene ne-POEMS syndrome?

Nge-POEMS syndrome, umonakalo wemithambo-luvo kunye nobuthathaka kunokwanda ngokuhamba kwexesha. Kungenza kube nzima nokuhambahamba. Ukuba ulwelo oluninzi luqokelelana kufutshane nemiphunga okanye ngaphakathi kwesifuba, lunokubangela intlungu esifubeni kunye nobunzima bokuphefumla.

Kodwa khumbula,Unyango oluphumelelayo lunokuphelisa okanye lunciphise umonakalo wemithambo-luvo. Lunokunceda ekunciphiseni ezinye iimpawu. Kwanomonakalo wemithambo-luvo unokuphola kancinci kancinci ngokuhamba kwexesha.

Ngaba kukho izinto ezibangela umngcipheko we-POEMS syndrome?

Okwangoku akukho zinto zibalulekileyo ezibangela umngcipheko wokuphuhlisa isifo se-POEMS.

Ngaba isifo se-POEMS singathintelwa?

Ngelishwa, isifo se-POEMS asinakuthintelwa.

Iyintoni ingqikelelo yabantu abane-POEMS syndrome?

Impilo yakho ixhomekeke kwizinto ezininzi, kuquka ukuba zeziphi iindawo zomzimba wakho ezichaphazeleka, ukuba isifo sifunyanwa ngokukhawuleza kangakanani kwaye unyango luqala njani, kunye nendlela osabela ngayo kunyango.

Kubalulekile ukufumana unyango lwe-POEMS. I-POEMS syndrome inokuba yingozi ebomini ukuba ayinyangwa. Kwelinye icala, unyango oluphumelelayo olutshabalalisa iiseli ze-plasma ezingaqhelekanga lunokukunceda uphile ixesha elide. Ukongeza, unyango lunokunciphisa iimpawu kwaye luphucule umgangatho wobomi bakho.

Izinga eliphakathi lokusinda kwabantu abaninzi abane-POEMS limalunga neminyaka eli-14. Oko kuthetha ukuba malunga nesiqingatha sabantu siphila ngaphantsi kweminyaka eli-14, ngelixa esinye isiqingatha siphila ixesha elide. Ubungakanani bobomi bakho buxhomekeke kumava akho athile esifo kunye nonyango.

Buza ugqirha wakho ngekamva lakho njengomntu ophila ne-POEMS syndrome.

Ufanele uye nini kugqirha malunga ne-POEMS syndrome?

Ukuba ufumana naziphi na iimpawu ezinxulumene ne-POEMS syndrome, ingakumbi ubuthathaka kwiingalo nasemilenzeni , bonana nogqirha ngokukhawuleza.

Yeyiphi imibuzo endifanele ndiyibuze ugqirha wam malunga ne-POEMS syndrome?

Ungabuza imibuzo efana nale:

• Ingxaki yam inzulu kangakanani?
• Ziziphi iindlela zonyango endinazo?
• Ndingenza ntoni ukunciphisa okanye ukulawula iziphumo ebezingalindelekanga?
• Zeziphi iinkonzo zenkxaso ezikhoyo kum?
• Ngaba kufuneka ndidibane nogqirha ogxile kwizifo ze-plasma cell ukuze andinike isikhokelo kunyango lwam?

Ndingabuyela nini kwimisebenzi yam yesiqhelo ukuba ndine-POEMS syndrome?

I-POEMS syndrome inokuchaphazela amandla akho okwenza imisebenzi yakho yemihla ngemihla. Ugqirha wakho uza kukuchazela indlela le meko eza kuyichaphazela ngayo ubomi bakho bemihla ngemihla.

Ngaba i-POEMS syndrome sisifo sokuzikhusela komzimba?

I-POEMS sisifo se-paraneoplastic.Asisisifo esibangelwa kukuzikhusela komzimba. I-paraneoplastic syndrome yingqokelela yeempawu ezibangelwa yimeko yezonyango engaphantsi. Kwi-POEMS, ingxaki esisiseko kukuba iiseli ze-plasma ezingaqhelekanga zivelisa i-M-protein eninzi egazini (isifo se-monoclonal plasma cell). Iimpawu ze-POEMS zibangelwa ziiseli ze-plasma ezingaqhelekanga kunye ne-M-protein ezonakalisa izicubu zomzimba.

Ngaba i-POEMS syndrome iyafana ne-multiple myeloma?

I-multiple myeloma ngumhlaza ochaphazela iiseli ze-plasma. I-POEMS syndrome yingqokelela yeempawu ezibangelwa ziiseli ze-plasma ezingaqhelekanga kunye nokwanda kwe-M-protein. Izinga lokusinda kwe-POEMS syndrome lingcono kunokulindeleka kobomi be-multiple myeloma.

Nangona kunjalo, unyango lwe-chemotherapy lwe-POEMS kunye ne-multiple myeloma ludla ngokufana. Ezinye zeempawu zisenokufana.

I-POEMS syndrome sisifo esintsonkothileyo. Kungathatha ixesha ugqirha wakho ukufumanisa ukuba iimpawu zakho zibangelwa yi-POEMS syndrome kungekhona esinye isifo. Nje ukuba ufunyaniswe ukuba unayo, sebenzisana ngokusondeleyo neqela lakho lezonyango ukuze ubone ukuba loluphi unyango oluya kunceda kakhulu kwiimpawu zakho. Buza ugqirha wakho malunga nemibutho okanye amaqela enkxaso anceda abantu kunye neentsapho ezijongene nokuxilongwa okufana ne-POEMS syndrome.

Umyalezo Wokuya Ekhaya

Kulungile, ngoko ke sithethe kakhulu nge-POEMS Syndrome namhlanje. Into ebalulekileyo kukuqonda ukuba le yimeko engaqhelekanga, kodwa inzima . Ingachaphazela iinkqubo ezininzi emzimbeni wethu.

• Igama elithi POEMS livela kwiileta zokuqala zamagama esiNgesi eempawu ezintlanu eziphambili (iingxaki zemithambo-luvo, ukwanda kwamalungu angaphakathi, iingxaki zehomoni, ukwanda kwe-M-protein, kunye notshintsho lolusu).
• Eyona nto ibangela oku ziiseli zeplasma ezingaqhelekanga kunye ne-M-protein ezizivelisayo.
• Nangona kungekho nyango lupheleleyo, kukho unyango olusebenzayo lokulawula iimpawu nokuphucula umgangatho wobomi.
• Kubaluleke kakhulu ukufumanisa isifo nokuqala unyango kwangethuba .

Ukuba unayo nayiphi na kwezi mpawu, nceda ucele ingcebiso kugqirha. Musa ukoyika, ngolwazi olufanelekileyo kunye nenkxaso kugqirha, ungamelana nale meko.

Isifo se - POEMS, izifo zegazi, umonakalo wemithambo-luvo, iingxaki zehomoni, i-M-protein, utshintsho lolusu, iiseli zeplasma