Kab mob POEMS yog dab tsi? Koj puas xav paub txog qhov no thiab?

Koj puas tau hnov ​​txog POEMS Syndrome? Qhov no yog ib yam mob uas tsis tshua muaj neeg hnov ​​txog. Txawm li cas los xij, nws yog ib qho tseem ceeb kom paub me ntsis txog nws vim nws tuaj yeem cuam tshuam rau ntau lub cev hauv peb lub cev ib zaug. Hais yooj yim xwb, nws yog ib yam mob nyuaj uas cuam tshuam nrog peb cov ntshav. Niaj hnub no, peb yuav tham txog POEMS Syndrome yog dab tsi, nws cov tsos mob yog dab tsi, nws kuaj mob li cas, thiab seb puas muaj kev kho mob.

POEMS Syndrome yog dab tsi tiag?

Zoo, cia peb saib seb POEMS Syndrome yog dab tsi. Qhov no yog ib yam kab mob ntshav uas tsis tshua muaj. Peb muaj ib hom qe ntshav hu ua plasma cells hauv peb lub cev. Thaum POEMS syndrome tshwm sim, cov qe ntshav plasma no faib thiab loj hlob tsis zoo li qub, tsis tuaj yeem tswj tau. Cov qe ntshav plasma tsis zoo no tso tawm ib qho protein tshwj xeeb rau hauv peb cov ntshav. Nws hu ua monoclonal protein, lossis luv luv M-protein . Tam sim no koj tuaj yeem xav txog, puas yog? Thaum cov qe ntshav plasma tsis zoo no, M-protein, nce ntau dhau hauv cov ntshav, nws tuaj yeem ua rau peb cov hlab ntsha thiab cov kabmob sab hauv puas tsuaj.

Lub npe POEMS yog los ntawm cov tsiaj ntawv thawj zaug ntawm tsib cov tsos mob tseem ceeb ntawm tus kab mob no:

• P - Polyneuropathy: Qhov no hais txog kev puas tsuaj rau cov hlab ntsha thoob plaws lub cev. Hauv POEMS, qhov kev puas tsuaj no tuaj yeem ua rau mob lossis lwm yam kev tsis xis nyob (xws li tingling, kub hnyiab, thiab lwm yam) hauv koj ob txhais ceg, caj npab, taw, thiab tes.
• O - Organomegaly: Qhov no txhais tau hais tias cov kab mob sab hauv loj zuj zus. Ib tug neeg uas muaj POEMS tej zaum yuav muaj cov kab mob loj xws li lub siab, lub qog ntshav, thiab cov qog ntshav hauv lub cev.
• E - Endocrinopathy: Hais yooj yim xwb, qhov no yog teeb meem hormonal. Hauv POEMS, cov tshuaj hormones uas peb cov qog endocrine tso tawm tuaj yeem tsis zoo li qub. Qee cov tshuaj hormones yuav raug tso tawm ntau dhau lossis me me.
• M - Monoclonal protein (M-protein): Qhov no yog cov protein tshwj xeeb uas sib sau ua ke hauv cov ntshav hauv POEMS. Thaum ib lub cell plasma ua ntau daim ntawv theej (clones) ntawm nws tus kheej, txhua lub cell ntawd ntxiv cov M-protein no rau hauv cov ntshav. Thaum kawg, qhov ntau ntawm M-protein hauv cov ntshav nce ntxiv ntau heev.
• S - Kev hloov pauv ntawm daim tawv nqaij: Cov neeg uas muaj POEMS yuav muaj ntau yam kev hloov pauv ntawm daim tawv nqaij, xws li daim tawv nqaij tsaus ntuj thiab daim tawv nqaij tuab zuj zus.

POEMS syndrome kuj tseem hu ua osteosclerotic myeloma , Crow-Fukase syndrome , PEP syndrome , thiab Takatsuki syndrome .

Leej twg yuav muaj tus mob POEMS syndrome no tshaj?

Kab mob POEMS yog ib yam mob tsis tshua muaj. Nws tsuas cuam tshuam rau ob peb tug neeg thoob ntiaj teb xwb. Feem ntau pom muaj rau cov neeg hnub nyoog ntawm 50 thiab 60 xyoo. Nws tuaj yeem cuam tshuam rau ob leeg poj niam txiv neej, tab sis nws zoo li pom ntau dua rau cov txiv neej.

Cov tsos mob ntawm tus kab mob POEMS yog dab tsi?

Raws li peb tau hais ua ntej lawm, cov ntawv hauv lub npe POEMS qhia txog qee cov tsos mob tseem ceeb. Ntawd yog, Polyneuropathy, Organomegaly, Endocrinopathy, M-protein nce, thiab kev hloov pauv ntawm daim tawv nqaij yog cov tsos mob tseem ceeb. Yuav luag txhua tus neeg uas muaj POEMS muaj polyneuropathy thiab M-protein nce. Lwm cov tsos mob yuav txawv ntawm ib tus neeg mus rau ib tus neeg.

Cov tsos mob ua rau polyneuropathy

Cov tsos mob uas tshwm sim los ntawm kev puas tsuaj ntawm cov hlab ntsha no yog qhov tshwm sim ntau tshaj plaws hauv POEMS. Feem ntau, qhov mob feem ntau hnov ​​​​​​nyob rau hauv ob txhais ceg thiab taw. Tab sis qee zaum nws tuaj yeem cuam tshuam rau caj npab thiab tes. Nov yog li cas koj yuav xav thaum koj muaj tus mob no:

• Tsis muaj zog
• Kev khaus khaus
• Kev tsis muaj zog
• Kev xav tias muaj qhov txhab / qhov txhab zoo li koob
• Kev xav kub hnyiab

Organomegaly - kev loj hlob ntawm cov kab mob sab hauv

Cov txheej txheem thaij duab uas cov kws kho mob ua tuaj yeem kuaj pom seb cov kabmob no puas loj dua:

• Mob siab (Hepatomegaly)
• Tus mob Splenomegaly ( Spleen )
• Cov qog ntshav hauv lub cev (Cov qog ntshav hauv lub cev - `(Lymphadenopathy)`)

Endocrinopathy - teeb meem hormonal

Qhov teeb meem tseem ceeb ntawm cov tshuaj hormones uas los nrog POEMS yog qhov txo qis ntawm cov tshuaj hormones poj niam txiv neej. Cov tshuaj hormones poj niam txiv neej no hu ua "Testosterone" rau cov txiv neej thiab "Estrogen" rau cov poj niam. Tsis tas li ntawd, koj yuav pom cov tsos mob xws li:

• Teeb meem tswj cov ntshav qab zib (piv txwv li ntshav qab zib)
• Cov teeb meem thyroid (piv txwv li Hypothyroidism)
• Kab mob Addison (Adrenal insufficiency - `(Addison's disease)`)

Cov txiv neej yuav muaj teeb meem kev xeeb tub tsis tau zoo thiab lub mis loj (gynecomastia).

Rau cov poj niam , kev coj khaub ncaws yuav nres ('(Amenorrhea)'), lub mis yuav loj dua. Thaum cev xeeb tub, cov tshuaj hormones '(Prolactin)', uas pab tsim mis, yuav raug tsim ntau dhau. Yog li ntawd, nws muaj peev xwm ua rau mis tawm ntawm lub txiv mis txawm tias koj tsis cev xeeb tub los xij.

Nce M-Protein

Ib qho tseem ceeb ntawm POEMS yog qib siab ntawm M-protein hauv cov ntshav. Qhov ntau dhau ntawm M-protein no tuaj yeem ua rau muaj qog hauv cov ntshav. Tsis tas li ntawd, vim muaj qib siab ntawm M-protein, kojCov pob txha tuaj yeem tuab thiab tawv (osteosclerosis) .

Kev hloov pauv ntawm daim tawv nqaij

Muaj ntau yam kev hloov pauv ntawm daim tawv nqaij, xws li:

• Daim tawv nqaij tsaus dua
• Tawv nqaij tuab dua
• Cov rau tes dawb
• Cov plaub hau loj hlob ntxiv rau ntawm koj lub ntsej muag thiab ob txhais ceg
• Kev loj hlob ntawm cov hlab ntsha uas zoo li cov txiv ntoo me me ntawm daim tawv nqaij ntawm lub hauv siab

Lwm cov tsos mob

Ntxiv rau qhov no, lwm cov tsos mob yuav pom tau:

• Kev o ntawm tes thiab taw
• Kev poob phaus tsis muaj laj thawj
• Mob hauv cov pob txha thiab cov pob qij txha
• Kev tsis pom kev zoo
• Ua pa nyuaj
• Kev qaug zog
• Ua npaws

Ib txhia neeg uas muaj tus kab mob POEMS kuj tseem yuav muaj tus mob hu ua Castleman Disease (CD), uas cuam tshuam rau cov qog ntshav. Yog tias koj muaj cov tsos mob ntawm tus kab mob Castleman, koj tus kws kho mob kuj yuav xav tias muaj tus kab mob POEMS.

Dab tsi ua rau muaj tus mob POEMS syndrome?

Thaum koj muaj POEMS, koj yuav muaj ntau yam tsos mob thiab teeb meem vim yog qhov teeb meem ntawm cov hlwb monoclonal plasma . Hais yooj yim xwb, cov hlwb plasma uas tsis zoo yuav ua ntau daim qauv ntawm nws tus kheej. Yog muaj cov hlwb no ntau npaum li cas, ces yuav ua rau cov nqaij puas tsuaj ntau npaum ntawd. Cov hlwb no ntxiv M-protein rau hauv cov ntshav, ua rau cov M-protein ntau npaum li ntawd hauv cov ntshav nce siab. Yog cov hlwb ntau dhau thiab M-protein no uas ua rau ntau yam kab mob hauv lub cev puas tsuaj.

Txawm li cas los xij, cov kws tshawb fawb tseem tsis tau paub meej tias vim li cas cov hlwb plasma no thiaj pib loj hlob tsis zoo li qub.

Cov kws tshawb nrhiav tau pom tias muaj ib qho protein hu ua Vascular Endothelial Growth Factor (VEGF) uas yuav koom nrog. Feem ntau cov neeg uas muaj tus kab mob POEMS muaj cov VEGF ntau hauv lawv cov ntshav. Kev tshawb fawb txog kev sib raug zoo ntawm VEGF thiab POEMS syndrome tseem tab tom ua mus.

Yuav kuaj mob POEMS syndrome li cas? (Kev kuaj mob)

Koj tus kws kho mob yuav xub nug koj txog koj keeb kwm mob thiab cov tsos mob tam sim no. Tom qab ntawd, lawv yuav kuaj lub cev kom paub seb puas muaj cov cim qhia ntawm tus kab mob POEMS.

Tsis tas li ntawd xwb, tus kws kho mob kuj tseem yuav ua ntau yam kev kuaj mob, xws li:

• Kev kuaj ntshav thiab zis: Cov kev kuaj no tuaj yeem pab kuaj seb puas muaj cov protein M-protein lossis VEGF ntau hauv koj cov ntshav lossis zis. Lawv kuj tseem tuaj yeem kuaj xyuas seb puas muaj cov qe ntshav tsis zoo lossis cov qe ntshav zoo li tsis zoo. Cov no tuaj yeem yog cov cim ntawm POEMS.
• Kev kuaj duab: Cov kev kuaj xws li X-rays thiab CT scans tuaj yeem qhia tau yog tias koj cov pob txha tuab lossis tawv. Cov kev hloov pauv no tuaj yeem tshwm sim thaum cov theem M-protein siab.
• Kev kuaj mob pob txha: Qhov no tuaj yeem nrhiav tau seb koj puas muaj cov qe ntshav dawb uas zoo li tsis zoo hauv koj cov pob txha, lossis yog tias muaj cov qe ntshav dawb ntau dhau.
• Electromyogram (EMG): Kev kuaj EMG yog qhov kev ntsuas uas ntsuas kev ua haujlwm ntawm cov hlab ntsha. Nws tuaj yeem pab koj tus kws kho mob kuaj mob polyneuropathy.

Nyob ntawm seb koj cov tsos mob zoo li cas, koj tus kws kho mob kuj tseem yuav ua lwm yam kev kuaj mob ntxiv, xws li kev kuaj ua pa, echocardiograms, lossis kev kuaj endocrine.

Yog tias koj tau raug kuaj pom tias muaj tus mob hu ua Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) , koj tus kws kho mob kuj tseem yuav kuaj koj seb koj puas muaj tus mob POEMS syndrome.

Yuav tswj lossis kho tus mob POEMS li cas?

Tsis muaj kev kho rau tus mob POEMS syndrome. Txawm li cas los xij, kev kho mob tuaj yeem pab tswj thiab tswj cov tsos mob. Cov kev xaiv kho mob suav nrog:

• Kev kho mob hluav taws xob: Qhov no suav nrog kev coj cov hluav taws xob ntau ntawm cov hlwb plasma uas tsis zoo kom rhuav tshem lawv.
• Kev Kho Mob Qog Ntsws: Cov tshuaj tua kab mob qog noj ntshav, tseem hu ua "Kev Kho Mob Qog Ntsws," yog siv los tua lossis qeeb qeeb kev loj hlob ntawm cov hlwb plasma uas tsis zoo. Kev kho mob qog noj ntshav rau POEMS zoo ib yam li kev kho mob rau ntau yam mob myeloma. Cov kev kho mob no muaj txiaj ntsig zoo heev thiab feem ntau tsis ua rau muaj kev phiv loj heev.
• Kev hloov hlwb hlwb los ntawm koj tus kheej: Cov txheej txheem no hloov koj cov hlwb plasma uas tsis zoo nrog cov hlwb hlwb zoo. Cov hlwb hlwb yog cov hlwb uas loj hlob mus ua cov hlwb laus zoo li cov hlwb plasma. Hauv kev hloov hlwb hlwb no, koj tus kws kho mob yuav tsum tshem tawm qee cov hlwb hlwb zoo ntawm koj lub cev ua ntej. Tom qab ntawd, lawv siv tshuaj kho mob qog noj ntshav lossis kev kho mob hluav taws xob los rhuav tshem cov hlwb plasma uas tsis zoo. Tom qab kev kho mob, cov hlwb hlwb uas raug tshem tawm yuav raug muab rov qab rau hauv koj lub cev. Tom qab ntawd lawv yuav loj hlob mus ua cov hlwb plasma uas noj qab nyob zoo.
• Kev Kho Mob Lub Cev: Yog tias koj muaj teeb meem taug kev vim yog polyneuropathy, kev kho mob no yuav pab tau.

Ntxiv rau qhov ntawd, tus kws kho mob yuav sau ntawv kom noj tshuaj xws li tshuaj steroids kom txo cov tsos mob thiab tshuaj diuretics (tshuaj ntsiav dej) kom txo qhov o.

Cov teeb meem uas cuam tshuam nrog tus kab mob POEMS yog dab tsi?

Nrog rau tus mob POEMS syndrome, kev puas tsuaj ntawm cov hlab ntsha thiab qhov tsis muaj zog tuaj yeem nce ntxiv zuj zus. Nws kuj tseem tuaj yeem ua rau nws nyuaj rau txav mus los. Yog tias muaj dej ntau dhau los sib sau ua ke ze ntawm lub ntsws lossis sab hauv lub hauv siab, nws tuaj yeem ua rau mob hauv siab thiab ua pa nyuaj.

Tiam sis nco ntsoov,Kev kho mob zoo tuaj yeem tiv thaiv lossis qeeb qhov kev puas tsuaj ntawm cov hlab ntsha. Nws kuj tseem tuaj yeem pab txo cov tsos mob ntawm lwm yam. Txawm tias qhov kev puas tsuaj ntawm cov hlab ntsha tuaj yeem maj mam kho tau raws sijhawm.

Puas muaj tej yam uas ua rau muaj tus mob POEMS syndrome?

Tam sim no tsis muaj cov yam ntxwv uas qhia tau tias muaj feem yuav mob POEMS syndrome.

Puas tiv thaiv tau tus kab mob POEMS syndrome?

Tu siab kawg li, POEMS syndrome tsis yog ib yam uas yuav tiv thaiv tau.

Cov neeg uas muaj tus kab mob POEMS syndrome no yuav muaj mob li cas?

Koj txoj kev noj qab haus huv nyob ntawm ntau yam, suav nrog qhov twg ntawm koj lub cev raug kev txom nyem, qhov sai npaum li cas tus kab mob raug kuaj pom thiab kev kho mob pib, thiab koj teb li cas rau kev kho mob.

Nws yog ib qho tseem ceeb heev uas yuav tsum tau kho tus kab mob POEMS. Tus kab mob POEMS tuaj yeem ua rau tuag taus yog tias tsis kho. Ntawm qhov tod tes, kev kho mob zoo uas rhuav tshem cov qe ntshav dawb tsis zoo tuaj yeem pab koj nyob ntev dua. Tsis tas li ntawd, kev kho mob tuaj yeem txo cov tsos mob thiab txhim kho koj lub neej zoo.

Tus nqi nruab nrab ntawm cov neeg feem coob uas muaj tus kab mob POEMS muaj sia nyob yog li ntawm 14 xyoos. Qhov ntawd txhais tau tias kwv yees li ib nrab ntawm cov neeg muaj sia nyob tsawg dua 14 xyoos, thaum ib nrab ntxiv muaj sia nyob ntev dua. Koj lub neej ntev npaum li cas nyob ntawm koj qhov kev mob thiab kev kho mob.

Nug koj tus kws kho mob txog koj lub neej yav tom ntej ua ib tus neeg uas muaj tus kab mob POEMS.

Thaum twg koj yuav tsum mus ntsib kws kho mob txog tus mob POEMS?

Yog tias koj muaj cov tsos mob ntsig txog POEMS syndrome, tshwj xeeb tshaj yog qhov tsis muaj zog ntawm caj npab thiab ceg , mus ntsib kws kho mob tam sim ntawd.

Kuv yuav tsum nug kuv tus kws kho mob li cas txog tus mob POEMS?

Koj tuaj yeem nug cov lus nug zoo li no:

• Kuv tus mob hnyav npaum li cas?
• Kuv muaj cov kev kho mob twg?
• Kuv yuav tsum ua li cas kom txo lossis tswj cov kev mob tshwm sim?
• Muaj cov kev pabcuam txhawb nqa dab tsi rau kuv?
• Kuv puas yuav tsum tau mus ntsib kws kho mob uas tshwj xeeb hauv kev kho mob plasma cell disorders los coj kuv txoj kev kho mob?

Yog tias kuv muaj tus mob POEMS syndrome, kuv mam li rov qab mus ua kuv tej dej num li qub tau thaum twg?

Kab mob POEMS tuaj yeem cuam tshuam rau koj lub peev xwm ua koj cov dej num txhua hnub. Koj tus kws kho mob yuav piav qhia rau koj tias qhov mob no yuav cuam tshuam li cas rau koj lub neej txhua hnub.

Puas yog POEMS syndrome yog ib qho kab mob autoimmune?

POEMS yog ib qho mob paraneoplastic syndrome.Nws tsis yog ib yam kab mob autoimmune. Ib qho paraneoplastic syndrome yog ib pawg ntawm cov tsos mob uas tshwm sim los ntawm ib qho mob hauv qab. Hauv POEMS, qhov teeb meem hauv qab yog tias cov hlwb plasma tsis zoo tsim cov protein M-protein ntau dhau hauv cov ntshav (ib qho teeb meem monoclonal plasma cell). Cov tsos mob ntawm POEMS yog tshwm sim los ntawm cov hlwb plasma tsis zoo thiab M-protein ua rau lub cev cov nqaij puas tsuaj.

Puas yog POEMS syndrome zoo ib yam li multiple myeloma?

Mob qog nqaij hlav ntau yam (multiple myeloma) yog ib hom mob qog nqaij hlav uas cuam tshuam rau cov hlwb ntshav (plasma cells). Mob qog nqaij hlav POEMS yog ib pawg ntawm cov tsos mob uas tshwm sim los ntawm cov hlwb ntshav tsis zoo thiab muaj cov protein M ntau ntxiv. Tus nqi ntawm tus mob qog nqaij hlav POEMS muaj sia nyob zoo dua li lub neej ntawm tus mob qog nqaij hlav ntau yam (multiple myeloma).

Txawm li cas los xij, kev kho mob chemotherapy rau POEMS thiab multiple myeloma feem ntau zoo sib xws. Qee cov tsos mob kuj yuav zoo sib xws.

Kab mob POEMS yog ib qho kev kuaj mob uas nyuaj heev. Tej zaum koj tus kws kho mob yuav siv sijhawm me ntsis los txiav txim siab tias koj cov tsos mob yog los ntawm kab mob POEMS thiab tsis yog los ntawm lwm yam mob. Thaum koj tau kuaj pom tias muaj tus kab mob no lawm, ua haujlwm nrog koj pab neeg kho mob kom paub seb cov kev kho mob twg yuav pab tau koj cov tsos mob zoo tshaj plaws. Nug koj tus kws kho mob txog cov koom haum lossis cov pab pawg txhawb nqa uas pab cov tib neeg thiab tsev neeg uas muaj cov tsos mob zoo li kab mob POEMS.

Cov Lus Qhia Txog Kev Coj Mus Tsev

Zoo, yog li peb tau tham ntau txog POEMS Syndrome hnub no. Qhov tseem ceeb yog kom nkag siab tias qhov no yog ib qho mob tsis tshua muaj, tab sis loj heev . Nws tuaj yeem cuam tshuam rau ntau yam kab mob hauv peb lub cev.

• Lub npe POEMS yog los ntawm cov tsiaj ntawv thawj zaug ntawm cov npe lus Askiv ntawm tsib yam tsos mob tseem ceeb (kev puas siab puas ntsws, kev loj hlob ntawm cov kab mob sab hauv, teeb meem hormonal, kev nce M-protein, thiab kev hloov pauv ntawm daim tawv nqaij).
• Qhov ua rau qhov no yog cov qe ntshav dawb tsis zoo thiab cov protein M uas lawv tsim tawm.
• Txawm hais tias tsis muaj kev kho mob tag nrho los xij, muaj cov kev kho mob zoo los tswj cov tsos mob thiab txhim kho lub neej zoo.
• Nws yog ib qho tseem ceeb heev uas yuav tsum kuaj mob thiab pib kho thaum ntxov .

Yog koj muaj ib qho ntawm cov tsos mob no, thov mus nrhiav kev pab kho mob. Tsis txhob ntshai, nrog cov ntaub ntawv raug thiab kev pab kho mob, koj tuaj yeem daws qhov teeb meem no.

POEMS syndrome, cov kab mob ntshav, kev puas tsuaj rau cov hlab ntsha, teeb meem hormonal, M-protein, kev hloov pauv ntawm daim tawv nqaij, cov hlwb plasma