Kasabah-Merritt Syndrome yog cov qog ntshav, uas cov kws kho mob kuj hu ua hemangioma. Xws li ib tug neoplasm tuaj yeem nyob rau ntawm lub cev thiab hauv qab ntawm daim tawv nqaij. Tus kab mob no ib txwm nrog los ntawm kev txo qis ntawm cov platelets thiab ntshav ntshav, uas yog tus cwj pwm los ntawm cov hemoglobin tsawg dhau thiab cov qe ntshav liab.
Tawm tsam keeb kwm yav dhau los ntawm thrombocytopenia, cov txheej txheem ntawm cov ntshav txhaws tau cuam tshuam, uas feem ntau ua rau cov ntshav tawm sab nraud thiab sab hauv. Feem ntau, cov me nyuam yug tshiab raug rau qhov tsis txaus ntseeg.
ICD-10 hemangioma code - P83.
Ntse
Tus kab mob no suav hais tias tsawg heev. Lub raum hemangioma feem ntau, tab sis txawm hnub no nws tuaj yeem kho tau zoo.
Kev tsim cov qog thiab qhov tshwm sim ntawm cov ntshav yog feem ntau cuam tshuam nrog kev txawv txav hauv cov qauv ntawm cov ntshav. Thrombocytopenia thiab kev ua kom muaj zog ntau dhau ntawm fibronolysis ua rau cov qe ntshav nyob hauv thaj tsam ntawm neoplasm, qhov chaw uas lawv raug rhuav tshem los ntawm cov khoom ntuj tsim.
Ua rau kev loj hlob ntawm pathology
Raws liCov kws kho mob, Kasabach-Merritt Syndrome yog ib yam kab mob caj ces thiab tau txais txiaj ntsig, feem ntau nyob rau hauv tus qauv autosomal dominant.
Ntxiv rau, muaj ntau qhov kev xav txog qhov ua rau tus kab mob no:
- Squeezing tus me nyuam thaum lub sij hawm nws hla txoj kev yug me nyuam, ntau yam kev raug mob thaum yug. Nrog rau cov caj ces predisposition rau tus kab mob no, txawm me me hematomas tuaj yeem ua rau kev loj hlob ntawm hemangioma.
- Tshuaj txwv tsis pub thaum cev xeeb tub los ntawm leej niam uas xav tau.
- Ib puag ncig tsis zoo, piv txwv li, raug leej niam yav tom ntej rau hluav taws xob - cov xwm txheej no cuam tshuam ncaj qha rau DNA, ua rau muaj kev hloov pauv thiab, yog li, ntau yam tsis zoo hauv lub cev.
Cov no yog cov laj thawj tseem ceeb ntawm Kazabah-Merritt syndrome. Txawm li cas los xij, yuav tsum tau hais tias tus kab mob no tseem tsis tau kawm tiav.
daim duab kho mob
Cov tsos mob tseem ceeb ntawm tus mob yog qhov muaj hemangiomas. Lawv yog dab tsi? Cov no yog vascular hlav uas tshwm sim feem ntau tam sim tom qab yug me nyuam. Muaj tseeb tiag, hemangiomas tej zaum yuav nyob rau hauv lub cev ntawm tus menyuam hauv plab.
Maj mam, cov qog vascular loj tuaj, kis mus rau cov qauv nyob ze. Yog tias lawv nyob hauv cheeb tsam ntawm cov kab mob hauv nruab nrog cev, kev kuaj mob yog qhov nyuaj dua. Tus kab mob tsis kuaj pom nyob rau lub sijhawm yog fraught nrog thrombocytopenia, uas ua rausab hauv thiab sab nraud los ntshav.
Kev kuaj mob ntawm Kazabakh-Merritt syndrome
Kev kuaj tus kab mob no tsis yog qhov nyuaj yog tias nws cuam tshuam rau ntawm daim tawv nqaij thiab pom qhov muag pom. Yog hais tias qhov anomaly npog cov ntaub so ntswg ntawm lub hauv nruab nrog cev, kev kuaj mob yuav nyuaj. Kev tshuaj xyuas cov menyuam mos hauv qhov no yog ua los ntawm txhua txoj hauv kev:
- Kev tshuaj ntsuam ntshav peripheral. Yuav tsum tau qhia rau cov me nyuam mos nrog cov tsos ntawm cov ntshav txawv txav. Qhov kev sim no yuav qhia tau tias muaj kev txo qis hauv cov platelets thiab qib fibrinogen. Qee zaum, cov kws kho mob pom zoo kom cov niam txiv siv sijhawm kuaj prothrombin. Qhov kev soj ntsuam no tso cai rau koj los txiav txim qhov tseeb ntawm cov ntshav txhaws hauv vivo.
- Chromosomal tests. Cov no yog cov kev tshawb fawb noob caj noob ces txhawm rau txiav txim siab txog cov kab mob pathological uas ua rau muaj tus kab mob.
- Angioma biopsy. Ib txoj hauv kev zoo tshaj plaws, uas suav nrog kev siv cov khoom siv roj ntsha rau kev tshawb fawb. Ib qho loj ntawm cov platelets puas, fibrin thiab lwm yam ntshav tuaj yeem pom hauv cov kua dej.
- Magnetic resonance imaging.
- Angiography.
- Isotope kawm.
- Computed tomography.
cov yam ntxwv tshwj xeeb
Nws yuav tsum tau hais tias Kazabah-Merritt syndrome ib txwm tshwm sim tam sim tom qab yug los lossis thawj lub lis piam ntawm tus menyuam lub neej, tab sis tsis pub dhau 4-5 lub hlis. Tsis tas li ntawd, tus kab mob no ib txwm muaj qhov tshwm sim ntawm hemangioma, anemia thiab thrombocytopenia. Yog tias daim duab kho mob tsis tiav, muaj lwm yam pathology tuaj yeem xav tias muaj. Tsuas yog cov cuab yeej thiab cov kev kuaj mob hauv chav kuaj ua kom nws tuaj yeem txiav txim siab qhov kev kuaj mob thiab cov yam ntxwv ntawm cov kab mob.
Txoj Kev Kho Mob
Kev kho tus kab mob nyuaj no yuav tsum ua kom tiav. Ntxiv mus, sai dua koj pib kho, ntau qhov kev vam meej koj tuaj yeem ua tiav hauv cov txheej txheem.
Cov kws kho mob tuaj yeem pom zoo ntau yam kev kho kom zoo, nyob ntawm qhov hnyav ntawm cov kab mob thiab cov yam ntxwv ntawm nws cov chav kawm. Cov tshuaj niaj hnub muaj ntau txoj hauv kev los kho Kazabach-Merritt syndrome:
- tshuaj kho mob;
- surgery;
- xov tooj cua;
- pneumatic compression.
Cov lus qhia kho mob rau Kasabach-Merritt syndrome hauv cov menyuam yaus
Yog xaiv kev kho mob kom zoo, tus menyuam raug xaiv los kho tus qauv. Nws suav nrog ntau pawg tshuaj sib txawv:
- Pills "Prednisolone". Lawv pab dab tsi? Nws yog ib qho tshuaj steroid ib txwm ua los ntawm cov qog adrenal. Nws yog nquag siv los ntawm cov kws kho mob los tswj cov kev ua haujlwm ntawm cov kab mob hauv nruab nrog cev hauv autoimmune pathologies. Cov tshuaj no yog muab raws li kev kho mob adjuvant. Cov ntsiav tshuaj tuaj yeem ua rau muaj kev phiv los ntawm kev los ntshav, rog rog, kev tsis haum xeeb hauv plab zom mov, qhov tsos ntawm lub hump, kev ua haujlwm ntawm cov tshuaj hormones. Tus nqi ntawm cov tshuaj nyob ntawm 100-130 rubles.
- "Metipred" yog corticosteroid, yuav luag zoo ib yam li prednisone. Cov muaj pes tsawg leeg kuj muaj ib qho ntxiv radical - methyl, uas muaj kev txhawb nqa. Nws tuaj yeem raug tshuaj los hloov "Prednisolone", vim nws yooj yim dua los ntawm cov menyuam yaus thiab muaj kev phiv tsawg dua. Tus nqi yog kwv yees li 240-260 rubles.
- "Prednisone" - qhov tseeb analogue ntawm "Prednisolone" los ntawm lwm lub chaw tsim khoom. Tus nqi ntawm cov tshuaj nce mus txog 120 rubles.
- "Methotrexate" yog cov tshuaj hauv tsev, uas raws li nws cov txheej txheem ntawm kev ua haujlwm sib raug rau cov tshuaj folic acid antagonist. Cov tshuaj no tiv thaiv kev loj hlob thiab kev loj hlob ntawm cov qog. Cov kev mob tshwm sim muaj xws li: teeb meem hauv plab zom mov, cuam tshuam rau lub cev xeeb tub, daim siab, ob lub raum, cov tshuaj tiv thaiv kab mob. Tus nqi ntawm "Methotrexate" yog 160-180 rubles.
- "L-asparaginase" yog ib qho tshuaj enzymatic zoo tsim los tua cov qog ntawm ntau lub hauv paus chiv keeb. Ua rau cov amino acids xav tau los ntawm neoplasm rau kev loj hlob thiab kev loj hlob. Cov kev mob tshwm sim muaj xws li: hemostasis, cuam tshuam ntawm lub hauv paus paj hlwb, kev tsis haum tshuaj, rwj hauv plab hnyuv. Tus nqi ntawm cov tshuaj yog kwv yees li 220 rubles.
- "Daunorubicin" - ib qho tshuaj nyob rau hauv daim ntawv ntawm kev daws rau intramuscular txhaj. Pab sib ntausnrog ntau yam neoplasms, tiv thaiv cov txheej txheem ntawm cov qog cell faib. Kev loj hlob ntawm kev phiv tshuaj tsis raug cais tawm: ntshav congestion, phlebitis, kev tsis haum tshuaj, kev ua me nyuam tsis ua haujlwm, lub plawv tsis ua haujlwm. Tus nqi ntawm cov tshuaj nce mus txog 520 rubles.
- "Cytarabine" - inhibits kev ua ntawm cov qog hlwb thiab inhibits cov txheej txheem metabolic hauv lawv. Nws yog muab rau cov me nyuam nyob rau hauv daim ntawv ntawm cov ntsiav tshuaj. Cov kev mob tshwm sim ntawm cov kws kho mob muaj xws li: qaug zog, apathy, baldness, kev nyuaj siab, qhov tsos ntawm bronchospasm, tsub kom lub cev tiv thaiv kab mob, cov tsos ntawm pob txuv ntawm daim tawv nqaij. Tus nqi ntawm cov tshuaj yog tsis ntau tshaj 60 rubles.
cov lus qhia ntxiv
Ntawm lwm yam, cov kws kho mob tau sau tshuaj interferons rau cov menyuam mos - cov kev daws teeb meem tshwj xeeb rau cov menyuam yaus intramuscularly lossis intravenously los ntawm drip. Lawv tiv thaiv tus kheej ntawm kev puas tsuaj ntawm cov qe ntshav hloov thiab ntxiv dag zog rau lub cev tiv thaiv kab mob ntawm tus menyuam yug tshiab, ua rau nws yooj yim dua rau nws zoo dua. Cov tshuaj no pab tau lub cev tsis muaj zog uas raug kev txom nyem los ntawm cov txheej txheem pathological kom rov zoo.
Yog tias tus menyuam tus mob dheev hnyav zuj zus tuaj thiab nthuav tawm cov kab mob intravascular coagulation syndrome tsim, tus menyuam raug muab tshuaj antiplatelet thiab lwm yam tshuaj uas pab txo cov ntshav. Cov tshuaj no raug xaiv ib tus zuj zus rau txhua tus menyuam.
Surgery
Yog tias kev kho hormonal conservative tsis ua haujlwm lossis pom lub raum hemangioma uas tuaj yeem ua rau kev txhim kho ntawm tus mob. DIC, kev phais tshem tawm ntawm cov qog yog sau tseg. Tib yam siv tau rau cov xwm txheej uas neoplasms tso siab rau hauv cov kabmob hauv nruab nrog cev thiab cuam tshuam cov ntshav ntws hauv lawv.
Kev tshem tawm yog nqa tawm tsuas yog thaum muaj xwm txheej hnyav, thaum lub neoplasm uas tau tshwm sim raug tshem tawm tag nrho. Feem ntau, tus mob ntawm tus menyuam tom qab kev ua haujlwm sai sai rov qab mus rau qhov qub. Thaum lub sij hawm kho mob, cov kws kho mob tau sau tshuaj hormonal rau tus menyuam, ua tsaug rau nws zoo heev.
Nws tsis tsim nyog ncua qhov kev txiav txim siab kom muaj kev ua haujlwm, vim qhov no yuav ua rau lub cev puas tsuaj tsis zoo.
Physiotherapy
Pneumatic compression yog ib txoj hauv kev zoo niaj hnub los kho thrombocytopenia thiab hemangioma. Xws li kev kho yog tso cai rau cov menyuam yaus uas muaj contraindicated nyob rau hauv anticoagulants.
Pneumatic compression ua rau muaj kev nce venous tawm ntawm qhov chaw uas cov ntshav accumulates. Cov txheej txheem no feem ntau tso cai rau koj tshem tawm cov qog uas tshwm sim tag nrho.
Kev kho hluav taws xob kuj tuaj yeem qhia rau cov neeg mob hluas uas tsis muaj txiaj ntsig ntawm kev saib xyuas kev kho mob. Cov txheej txheem ntawm cov txheej txheem no yooj yim heev: nyob rau hauv kev cuam tshuam ntawm hluav taws xob, cov qog hlwb tsuas yog rhuav tshem.
Cryosurgery tuaj yeem siv yog tias hemangioma sab nraud. Txhawm rau tshem tawm cov qog nyob rau hauv rooj plaub no, qhov ntsuas kub qis tshaj plaws yog siv, uas ua rau kev puas tsuajcells.
Huab cua
Hemangioma (ICD-10 code - P83) yog ib yam kab mob uas tuaj yeem kho tau tag nrho. Qhov tsuas yog qhov txaus ntshai ntawm tus kab mob yog kev txhim kho ntawm DIC thiab tom qab hemorrhage hauv lub hlwb lossis cov kabmob tseem ceeb. Tom qab tag nrho, tawm tsam keeb kwm ntawm xws li pathology, lawv tuaj yeem tsis lees paub.
Tsis tas li ntawd, cov menyuam yaus uas raug kev txom nyem los ntawm Kasabach-Merritt syndrome feem ntau tsim cov kab mob ntev.
Yog li, nws yog ib qho tseem ceeb heev uas yuav tsum tau ua raws li kev kho mob raws sij hawm thiab, thaum tsis muaj qhov xav tau, ua rau nruab nrab hemangioma hauv txoj kev radical.
