Koj puas feem ntau muaj mob lub cev txawv txawv? Cia peb kawm ntxiv txog qhov '(Systemic Mastocytosis)' no!

Koj puas muaj tej qhov liab, khaus ntawm koj daim tawv nqaij? Los yog koj puas mob plab thiab mob taub hau uas tsuas yog tuaj thiab ploj mus xwb? Yog tias tej yam no tshwm sim tas li, nws yuav yog ib qho mob tsis tshua muaj hu ua "Systemic Mastocytosis" uas peb yuav tham txog hnub no. Tsis txhob txhawj, qhov no tsis yog ib yam kab mob uas cuam tshuam rau ntau tus neeg. Tab sis nws yog ib qho tseem ceeb uas yuav tsum paub txog qhov no.

Yog li ntawd, qhov "Systemic Mastocytosis" no yog dab tsi?

Hais yooj yim xwb, '(Systemic Mastocytosis)' yog ib yam kab mob tsis tshua muaj feem cuam tshuam nrog peb cov ntshav. Qhov tshwm sim hauv qhov no yog tias ib hom cell tshwj xeeb hu ua '(Mast cells)' hauv peb lub cev tsim tawm tsis zoo. Xav txog nws, cov mast cell no zoo li cov tub rog me me hauv peb lub cev. Lawv yog cov uas, ua ib feem ntawm peb lub cev tiv thaiv kab mob, paub thiab tawm tsam cov neeg txawv teb chaws (xws li cov kab mob, cov khoom ua xua, thiab lwm yam). Thaum tus neeg txawv teb chaws tuaj txog, cov mast cell no tso cov tshuaj lom neeg zoo li '(Histamine)'. Qhov '(Histamine)' no yog qhov ua rau cov tsos mob ua xua (xws li liab, khaus, o). Feem ntau, cov mast cell tsis tso tawm '(Histamine)' no thaum ua tiav txoj haujlwm. Txawm li cas los xij, hauv lub cev ntawm ib tus neeg uas muaj '(Systemic Mastocytosis)', cov mast cell tsis zoo no tsim tawm ntau dhau thiab tso tawm '(Histamine)' tas li. Nws zoo li ib tug neeg tso lub qhov dej rau thiab tom qab ntawd tsis nco qab tua nws. Qhov no yog vim li cas peb thiaj li muaj cov tsos mob zoo li ua xua. Nws tuaj yeem cuam tshuam tsis yog peb daim tawv nqaij xwb, tab sis kuj rau peb cov kab mob sab hauv xws li peb lub siab, lub zais zis, thiab txoj hnyuv. Tus mob no, hu ua "Systemic Mastocytosis," lawv hais tias tsuas yog cuam tshuam rau 13 ntawm txhua 100,000 tus neeg hauv ntiaj teb. Qhov ntawd txhais tau tias nws tsis tshua muaj. Txawm li cas los xij, cov neeg uas muaj tus mob no muaj feem yuav mob anaphylaxis ( kev ua xua hnyav, tam sim ntawd, thiab ua rau tuag taus). Tsis tshua muaj, "Systemic Mastocytosis" kuj tuaj yeem hloov mus ua mob qog noj ntshav. Cov kws kho mob tsis tuaj yeem kho tus mob no tag nrho. Txawm li cas los xij, muaj kev kho mob uas tuaj yeem pab koj tswj koj cov tsos mob thiab ua neej nyob li qub.

Cov hom mob tseem ceeb ntawm '(Systemic Mastocytosis)' yog dab tsi?

Muaj rau hom mob "Systemic Mastocytosis" tseem ceeb. Lawv cuam tshuam rau peb ntau txoj kev sib txawv. Feem ntau, cov hlwb mast cell uas tsis zoo hauv koj lub cev ntau npaum li cas, koj yuav muaj feem ntau dua los tsim teeb meem. Cia peb saib cov hom no yog dab tsi: 1. Hom uas loj hlob qeeb ("Indolent systemic mastocytosis"):

• Qhov no yog hom uas feem ntau pom. Ib yam li tsob ntoo uas loj qeeb qeeb, nws yuav siv sijhawm ntau xyoo kom cov tsos mob tshwm sim. Tus naj npawb ntawm cov hlwb mast tsis zoo yuav nce ntxiv raws sijhawm. Qhov no tuaj yeem ua rau muaj cov tsos mob hauv daim tawv nqaij, daim siab , tus po , thiab lub plab zom mov (piv txwv li, cov hnyuv).

2. Hom mob mastocytosis uas nyuaj dua me ntsis ('Systemic smoldering mastocytosis'):

• Hauv hom no, cov hlwb mast cell txawv txav yuav sib sau ua ke ntau heev hauv lub siab thiab lub po. Raws sijhawm mus, qhov no tuaj yeem ua rau lub siab lossis lub po o. Xav txog nws zoo li qhov tshwm sim thaum lub tsev me me puv nkaus.

3. Hom uas los nrog lwm yam kab mob ntshav ('Systemic mastocytosis with associated hematologic neoplasm'):

• Cov neeg uas muaj hom no yuav muaj lwm yam mob ntsig txog ntshav, xws li myeloproliferative neoplasms thiab myelodysplastic syndrome . Hom no cuam tshuam rau kwv yees li ib ntawm tsib tus neeg uas muaj Systemic Mastocytosis.

4. Hom mob hnyav ('Aggressive systemic mastocytosis'):

• Qhov no loj dua me ntsis vim nws tuaj yeem cuam tshuam rau cov pob txha (qhov chaw uas cov qe ntshav tsim) thiab cov pob txha. Cov qe mast cell txawv txav tuaj yeem loj hlob hauv cov pob txha, ua rau lawv qaug zog thiab ua rau lawv tawg yooj yim.

5. Hom uas hloov mus ua mob leukemia ('Mast cell leukemia'):

• Tsis tshua muaj, Systemic Mastocytosis tuaj yeem tsim mus rau hauv ib qho mob hu ua Mast Cell Leukemia . Qhov no yog ib hom Acute Myeloid Leukemia (AML) .

6. Hom qog ('Mast cell sarcoma'):

• Qhov no yog thaum cov qog nqaij hlav (qog nqaij hlav) uas tsim los ntawm cov hlwb mast cells uas tsis zoo li qub ua rau ntau yam nqaij hauv lub cev puas tsuaj. Muaj cov neeg tsawg heev uas muaj tus kab mob "Systemic Mastocytosis" no hu ua "Mast cell sarcoma".

Cov tsos mob ntawm tus kab mob no (Systemic Mastocytosis) yog dab tsi?

Cov tsos mob yuav txawv ntawm ib tus neeg mus rau ib tus neeg. Nws nyob ntawm seb qhov twg hauv lub cev cov hlwb mast cell txawv txav nyob ntau tshaj plaws. Piv txwv li, yog tias koj muaj ntau cov hlwb mast hauv koj lub plab, koj yuav mob qog thiab mob plab. Tsis tas li ntawd, yog tias cov hlwb mast sib sau ua ke hauv koj cov pob txha, nws tuaj yeem cuam tshuam rau kev tsim cov qe ntshav. Nov yog qee cov tsos mob uas feem ntau tshwm sim:

• Anemia ( tsis muaj ntshav txaus )
• Mob pob txha
• Los ntshav ntau dhau (piv txwv li, ntshav ntau los ntawm qhov txhab me me)
• Xav tias nkees txhua lub sijhawm (` Qaug zog` )
• Daim tawv nqaij liab
• Lub plawv dhia ceev
• Mob pob khaus (khaus, ua pob loj rau ntawm daim tawv nqaij)
• Khaus tawv nqaij
• Kev hloov pauv ntawm lub siab lossis kev nyuaj siabkev nyuaj siab` )
• Cov pob khaus dub thiab khaus ntawm daim tawv nqaij ('Urticaria pigmentosa')

Qee zaum, cov neeg uas muaj Systemic Mastocytosis yuav muaj ntau yam tsos mob no ib zaug. Cov kws kho mob hu qhov no ua "mastocytosis attack" lossis "flare."

Tseem Ceeb: Tsis yog txhua tus neeg uas muaj cov tsos mob no muaj '(Systemic Mastocytosis).' Txawm li cas los xij, yog tias koj muaj ntau yam ntawm cov tsos mob no ib zaug, thiab lawv tseem nyob, koj yuav tsum mus ntsib kws kho mob kom tau txais kev qhia.

Vim li cas qhov '(Systemic Mastocytosis)' no thiaj tshwm sim?

Kev tshawb fawb pom tau tias kwv yees li 80% ntawm cov neeg uas muaj '(Systemic Mastocytosis)' muaj 'KIT gene variant '. Cov 'KIT' gene no pab qee hom cell hauv peb lub cev, tshwj xeeb tshaj yog cov cell ntshav thiab mast cell, tsim. Cov gene variant no tshwm sim tom qab peb xeeb tub hauv plab. Qhov no txhais tau tias nws tsis yog los ntawm noob caj noob ces.

Cov yam dab tsi ua rau tus kab mob hnyav dua lossis ua rau muaj teeb meem ('Triggers')?

Muaj ntau yam ua rau muaj cov tsos mob ntawm "Systemic Mastocytosis." Tsis yog txhua tus neeg raug cuam tshuam los ntawm tib yam. Txawm li cas los xij, muaj ob peb yam uas feem ntau tshwm sim:

• Cawv
• Cov khoom noj ntsim
• Qee cov tshuaj: Piv txwv li , NSAIDs ( tshuaj tua mob), cov tshuaj so cov leeg, thiab tshuaj loog.
• Kev tawm dag zog thiab kev ua ub ua no ( tsuas yog rau qee tus)
• Kab tom (tshwj xeeb tshaj yog ntsaum, ntsaum, thiab muv)
• Kev ntxhov siab ntawm lub cev lossis kev xav
• Kev txhuam los yog kev sib txhuam rau ntawm koj daim tawv nqaij
• Kev hloov pauv sai sai ntawm qhov kub thiab txias (piv txwv li, dhia mus rau hauv dej txias)

Yog koj muaj tus kab mob no, nws yog ib qho tseem ceeb heev uas koj yuav tsum nug koj tus kws kho mob seb koj qhov tsis muaj zog yog dab tsi thiab koj yuav tsum zam dab tsi.

Koj kuaj mob no li cas kom meej? (Kev kuaj mob)

Thaum koj mus ntsib kws kho mob, thawj qhov uas lawv yuav ua yog kuaj koj lub cev. Lawv yuav nug txog koj cov tsos mob thiab tej yam mob uas koj tau muaj yav dhau los. Tom qab ntawd, lawv yuav ua ib lossis ntau qhov kev kuaj mob hauv qab no kom paub seb koj puas muaj Systemic Mastocytosis:

• Kev kuaj ntshav: Qhov no xyuas seb koj cov ntshav muaj pes tsawg tryptase . Tryptase yog ib qho enzyme tshwj xeeb uas cov hlwb mast tso tawm thaum lawv teb rau cov khoom txawv teb chaws.
• Kev soj ntsuam pob txha: Xyuas seb pob txha puas puas tsuaj.
• Kev kuaj mob pob txha: Qhov no suav nrog kev coj me me ntawm koj cov pob txha thiab kuaj nws kom pom tias puas muaj cov hlwb mast tsis zoo hauv nws, thiab muaj pes tsawg.
• Kev kuaj noob caj noob ces: Tshawb xyuas seb puas muaj kev hloov pauv hauv cov noob 'KIT' uas tau hais ua ntej.

Yuav kho li cas?

Cov kws kho mob kho Systemic Mastocytosis los ntawm kev tswj cov tsos mob thiab teeb meem. Piv txwv li, yog tias koj muaj kua qaub ntau hauv plab, lawv yuav sau ntawv rau H2 blockers (ib hom tshuaj antacid). Yog tias anemia yog qhov tshwm sim ntawm tus kab mob, nws yuav raug kho. Nov yog qee qhov kev kho mob tseem ceeb:

• Cov Tshuaj Antihistamines: Txo cov tsos mob ntawm daim tawv nqaij xws li khaus thiab liab.
• Corticosteroids: Txo qhov o thiab o hauv lub cev.
• Bisphosphonates: Lawv pab txhawb cov pob txha yog tias lawv tsis muaj zog.
• Kev kho mob tsom mus rau ib qho protein tshwj xeeb: Qhov no suav nrog kev tsom mus rau ib qho protein tshwj xeeb uas pom muaj nyob rau hauv cov hlwb mast uas tsis zoo.
• Kev kho mob qog noj ntshav: Qhov no tsuas yog siv yog tias Systemic Mastocytosis tau hloov mus ua mob qog noj ntshav.
• Kev phais tshem tawm lub qog ntshav: Yog tias lub qog ntshav o heev, nws tuaj yeem raug phais tshem tawm.
• Kev hloov pob txha: Qhov no yog ua rau cov neeg uas muaj kab mob hnyav heev, lossis cov neeg uas nyob rau theem kawg.

Nco ntsoov: Yog tias koj muaj mob Systemic Mastocytosis, koj yuav tsum nqa EpiPen tas li. EpiPen yog ib yam tshuaj uas siv tau tam sim ntawd yog tias muaj kev tsis haum tshuaj hnyav, xws li Anaphylaxis uas tau hais los saum toj no.

Puas tsis tuaj yeem tiv thaiv qhov "Systemic Mastocytosis" no tau?

Raws li peb tau tham ua ntej lawm, "Systemic Mastocytosis" yog tshwm sim los ntawm kev hloov pauv caj ces. Yog li ntawd, nws tsis tuaj yeem tiv thaiv nws kom tsis txhob loj hlob. Txawm li cas los xij, koj tuaj yeem tswj tus kab mob los ntawm kev zam "cov yam ua rau" uas ua rau cov tsos mob nce ntxiv.

Ib tug neeg uas muaj '(Systemic Mastocytosis)' muaj lub neej yav tom ntej zoo li cas?

Qhov no yeej nyob ntawm seb koj muaj hom kab mob "(Systemic Mastocytosis)" twg. Cov neeg uas muaj hom kab mob loj hlob qeeb ("Indolent systemic mastocytosis") feem ntau tuaj yeem tswj tau tus kab mob zoo nrog kev kho mob. Lawv tuaj yeem ua neej nyob tau ib txwm. Txawm li cas los xij, cov neeg uas muaj lwm hom kab mob hnyav yuav muaj lub neej luv dua me ntsis. Txawm li cas los xij, "(Systemic Mastocytosis)" tuaj yeem kho tau tag nrho nrog kev kho mob hu ua "Bone marrow transplant". Txawm li cas los xij, cov kws kho mob tsuas yog muab kev kho mob ntawd rau cov neeg uas nyob rau theem hnyav tshaj plaws ntawm tus kab mob.

Kuv yuav ua li cas thiaj saib xyuas kuv tus kheej thaum kuv tseem muaj tus kab mob hu ua '(Systemic Mastocytosis)'?

Kev paub txog yam ua rau koj cov tsos mob thiab zam lawv yog qhov tseem ceeb tshaj plaws uas koj tuaj yeem ua thaum nyob nrog Systemic Mastocytosis. Yog tias koj muaj tus mob no, ceev faj txog cov no:

• Nqa EpiPen nrog koj ib txwm. Kho qhov kev ua xua hnyav sai sai.
• Paub meej tias koj cov teeb meem yog dab tsi thiab zam lawv ntau li ntau tau.
• Tswj kev ntxhov siab. Xyaum ua kev xav lossis lwm yam kev ua kom lub siab tus.
• Hnav ib txoj hlua tes ceeb toom kho mob uas teev cov tshuaj uas koj tsis tuaj yeem noj. Qhov no tseem ceeb heev vim nws pab cov kws kho mob paub koj tus mob yog tias koj hais tsis tau lus thaum muaj xwm txheej ceev.

Kuv yuav tsum mus ntsib kws kho mob thaum twg?

Yog koj muaj mob Systemic Mastocytosis, koj yuav tsum mus ntsib koj tus kws kho mob kom kuaj xyuas koj tus kheej tas li. Tsis tas li ntawd, nco ntsoov qhia rau koj tus kws kho mob yog tias koj muaj cov tsos mob tshiab, lossis yog tias koj cov tsos mob zoo li hnyav zuj zus tuaj.

Kuv yuav tsum nug tus kws kho mob li cas?

Vim tias Systemic Mastocytosis yog ib yam kab mob tsis tshua muaj, tej zaum koj yuav muaj ntau cov lus nug. Nov yog qee cov lus nug uas yuav nug koj tus kws kho mob:

• Kuv muaj hom "Systemic Mastocytosis" twg?
• Cov tsos mob twg ntxiv uas yuav qhia tau tias kuv tus mob hnyav zuj zus tuaj?
• Muaj kev kho mob dab tsi rau kuv hom?
• Kev kho mob puas yuav tshem tawm kuv cov tsos mob tag nrho?
• Puas muaj kev sim tshuaj uas kuv tuaj yeem koom nrog?

Kev kuaj pom tias muaj tus kab mob Systemic Mastocytosis yog ib qho kev paub uas hloov pauv lub neej. Koj yuav tsum tau mus ntsib koj tus kws kho mob tsis tu ncua, kuaj ntshav, thiab kawm paub txog thiab zam cov teeb meem uas ua rau muaj tus kab mob no. Kev nyob nrog tus kab mob no yog ib qho kev hloov pauv loj. Yog li, khaws kev sib raug zoo nrog koj tus kws kho mob. Lawv nyob ntawd los pab koj. Lawv yuav muab cov ntaub ntawv thiab cov pab pawg txhawb nqa rau koj uas tuaj yeem pab koj. Cov no yuav ua rau koj muaj peev xwm txiav txim siab txog koj txoj kev noj qab haus huv.

Thaum kawg, tej yam yuav tsum nco ntsoov (Cov Lus Coj Mus Tsev)

Zoo, yog li peb tau tham ntau txog '(Systemic Mastocytosis)', puas yog? Nco ntsoov, txawm tias qhov no yog ib qho kab mob tsis tshua muaj, nws tseem ceeb heev uas yuav tsum paub txog nws.

Qhov tseem ceeb tshaj plaws yog mus ntsib kws kho mob yog tias koj muaj cov tsos mob no. Yog tias pom tus kab mob thaum ntxov, kev kho mob tuaj yeem pib thiab cov tsos mob tuaj yeem tswj tau.

Koj tsis nyob ib leeg xwb. Koj cov kws kho mob, tsev neeg, thiab cov phooj ywg yeej npaj siab pab koj. Thaum koj paub cov ntaub ntawv zoo thiab ua raws li koj tus kws kho mob cov lus qhia, koj yuav ua neej tau zoo nrog tus mob no. Nyob twj ywm thiab xav zoo!