Cov tshuaj teb nrog eosinophilia thiab cov tsos mob ntawm cov kab mob (DRESS), tseem hu ua tshuaj-induced hypersensitivity syndrome, yog ib qho mob hnyav T-cell-mediated cutaneous phiv tshuaj tshwm sim los ntawm cov pob liab liab, kub taub hau, kev koom tes ntawm cov kab mob hauv nruab nrog cev, thiab cov tsos mob tshwm sim tom qab siv qee yam tshuaj ntev.
DRESS tshwm sim nyob rau hauv kwv yees li 1 ntawm 1,000 mus rau 1 ntawm 10,000 tus neeg mob tau txais tshuaj, nyob ntawm seb hom tshuaj inducing. Feem ntau ntawm cov mob DRESS yog tshwm sim los ntawm tsib yam tshuaj, nyob rau hauv nqe lus nug ntawm qhov tshwm sim: allopurinol, vancomycin, lamotrigine, carbamazepine, thiab trimethopridine-sulfamethoxazole. Txawm hais tias DRESS tsis tshua muaj, nws suav txog li 23% ntawm cov tshuaj tiv thaiv ntawm daim tawv nqaij hauv cov neeg mob hauv tsev kho mob. Cov tsos mob tshwm sim ntawm DRESS (cov tshuaj teb nrog eosinophilia thiab cov tsos mob ntawm lub cev) muaj xws li kub taub hau, mob plab, mob caj pas, nqos nyuaj, khaus, kub hnyiab ntawm daim tawv nqaij, los yog ua ke ntawm cov saum toj no. Tom qab theem no, cov neeg mob feem ntau tsim cov pob liab liab zoo li pob liab liab uas pib ntawm lub cev thiab lub ntsej muag thiab maj mam kis mus, thaum kawg npog ntau dua 50% ntawm daim tawv nqaij ntawm lub cev. Lub ntsej muag edema yog ib qho ntawm cov yam ntxwv ntawm DRESS thiab tuaj yeem ua rau hnyav dua lossis ua rau lub pob ntseg pob ntseg tshiab, uas yuav pab kom paub qhov txawv ntawm DRESS los ntawm qhov tsis sib xws xws li mob qhua pias zoo li tshuaj ua pob khaus.
Cov neeg mob DRESS tuaj yeem tshwm sim nrog ntau yam kab mob, suav nrog urticaria, eczema, lichenoid hloov, exfoliative dermatitis, erythema, lub hom phiaj zoo li qhov txhab, purpura, hlwv, pustules, los yog ua ke ntawm cov no. Ntau qhov txhab ntawm daim tawv nqaij tuaj yeem tshwm sim hauv tib tus neeg mob tib lub sijhawm lossis hloov pauv thaum tus kab mob mus ntxiv. Hauv cov neeg mob uas muaj tawv nqaij tawv, erythema thaum ntxov yuav tsis pom, yog li nws yuav tsum tau kuaj xyuas kom zoo nyob rau hauv cov teeb pom kev zoo. Pustules muaj nyob rau ntawm lub ntsej muag, caj dab thiab hauv siab.
Nyob rau hauv ib tug yav tom ntej, validated European Registry ntawm mob hnyav Cutaneous Adverse Reactions (RegiSCAR) txoj kev tshawb no, 56% ntawm DRESS cov neeg mob tsim mob me me mucosal o thiab yaig, nrog rau 15% ntawm cov neeg mob muaj mucosal o nrog rau ntau qhov chaw, feem ntau cov oropharynx.Nyob rau hauv txoj kev tshawb no RegiSCAR, feem ntau ntawm cov neeg mob tsis muaj cov qog nqaij hlav thiab cov qog nqaij hlav. lymph node loj txawm predates cov tsos mob ntawm daim tawv nqaij. Cov pob khaus feem ntau kav ntev tshaj li ob lub lis piam thiab muaj lub sijhawm rov qab los ntev dua, thaum desquamation superficial yog qhov tseem ceeb. Tsis tas li ntawd, txawm tias tsis tshua muaj neeg, muaj tsawg tus neeg mob uas muaj DRESS uas yuav tsis tuaj nrog pob liab liab lossis eosinophilia.
Cov kab mob ntawm cov kab mob DRESS feem ntau cuam tshuam nrog cov ntshav, daim siab, lub raum, lub ntsws, thiab lub plawv, tab sis yuav luag txhua lub cev hauv nruab nrog cev (xws li endocrine, plab hnyuv, neurological, ocular, thiab rheumatic systems) tuaj yeem koom nrog. Hauv txoj kev tshawb fawb RegiSCAR, 36 feem pua ntawm cov neeg mob muaj tsawg kawg yog ib lub cev nqaij daim tawv ntxiv, thiab 56 feem pua muaj ob lossis ntau lub cev koom nrog. Atypical lymphocytosis yog qhov tshwm sim ntau tshaj plaws thiab ntxov tshaj plaws hematological abnormality, whereas eosinophilia feem ntau tshwm sim nyob rau theem tom qab ntawm tus kab mob thiab tej zaum yuav pheej.
Tom qab ntawm daim tawv nqaij, daim siab yog qhov feem ntau cuam tshuam rau lub cev. Kev nce siab ntawm daim siab enzyme tuaj yeem tshwm sim ua ntej cov pob liab tshwm, feem ntau mus rau qib me me, tab sis qee zaus tuaj yeem ncav cuag li 10 npaug ntawm qhov kev txwv ntawm ib txwm muaj. Hom mob siab tshaj plaws yog cholestasis, ua raws li kev sib xyaw cholestasis thiab hepatocellular raug mob. Nyob rau hauv tsawg zaus, mob siab tsis ua hauj lwm tej zaum yuav hnyav txaus yuav tsum tau hloov daim siab. Nyob rau hauv cov ntaub ntawv ntawm DRESS nrog rau daim siab ua tsis taus pa, cov tshuaj pathogenic feem ntau yog cov tshuaj tua kab mob. Kev tshuaj xyuas kev soj ntsuam tau soj ntsuam 71 tus neeg mob (67 tus neeg laus thiab 4 tus menyuam yaus) nrog DRES ntsig txog lub raum sequelae. Txawm hais tias feem ntau cov neeg mob muaj kev puas siab puas ntsws, 1 ntawm 5 tus neeg mob tam sim no tsuas muaj kev cuam tshuam rau lub raum. Cov tshuaj tua kab mob yog cov tshuaj feem ntau cuam tshuam nrog rau lub raum puas hauv cov neeg mob DRESS, nrog vancomycin ua rau 13 feem pua ntawm lub raum puas, ua raws li allopurinol thiab cov tshuaj tiv thaiv kab mob. Mob raum raug mob yog tus cwj pwm los ntawm kev nce qib creatinine los yog txo qis glomerular pom tus nqi, thiab qee kis tau nrog cov proteinuria, oliguria, hematuria lossis tag nrho peb. Tsis tas li ntawd, tej zaum yuav muaj cov hematuria cais los yog proteinuria, lossis txawm tias tsis muaj zis. 30% ntawm cov neeg mob cuam tshuam (21/71) tau txais kev kho lub raum, thiab thaum muaj ntau tus neeg mob rov qab ua haujlwm rau lub raum, nws tsis paub meej tias puas muaj qhov tshwm sim ntev. Kev koom tes hauv lub ntsws, uas ua rau ua pa luv, hnoos qhuav, lossis ob qho tib si, tau tshaj tawm hauv 32% ntawm cov neeg mob DRESS. Feem ntau cov pulmonary abnormalities nyob rau hauv kev ntsuam xyuas muaj xws li interstitial infiltration, mob ua pa nyuaj siab syndrome thiab pleural effusion. Cov teeb meem muaj xws li mob ntsws interstitial pneumonia, lymphocytic interstitial pneumonia, thiab pleurisy. Txij li thaum pulmonary DRESS yog feem ntau misdiagnosed li pneumonia, kev kuaj mob yuav tsum tau ceev faj heev. Yuav luag tag nrho cov mob ntsws muaj kev cuam tshuam nrog lwm cov khoom hauv nruab nrog cev. Hauv lwm qhov kev tshuaj xyuas, txog li 21% ntawm cov neeg mob DRESS muaj myocarditis. Myocarditis tej zaum yuav ncua sij hawm mus rau lub hlis tom qab lwm cov tsos mob ntawm DRESS down, los yog tseem nyob mus li. Cov hom muaj xws li mob eosinophilic myocarditis (kev tso cai nrog kev kho mob luv luv) mus rau mob necrotizing eosinophilic myocarditis (kev tuag ntau dua 50% thiab kev ciaj sia nyob nruab nrab ntawm 3 mus rau 4 hnub). Cov neeg mob uas muaj myocarditis feem ntau tshwm sim nrog ua tsis taus pa, mob hauv siab, tachycardia, thiab hypotension, nrog rau cov qib siab myocardial enzyme, hloov electrocardiogram, thiab echocardiographic abnormalities (xws li pericardial effusion, systolic dysfunction, ventricular hyperemia, ventricular septal physiotherapy). Cardiac magnetic resonance imaging tuaj yeem pom cov kab mob endometrial, tab sis qhov kev kuaj pom tseeb feem ntau yuav tsum muaj kev kuaj mob endometrial. Lub ntsws thiab myocardial kev koom tes tsis tshua muaj nyob rau hauv DRESS, thiab minocycline yog ib qho ntawm feem ntau inducing agents.
European RegiSCAR cov qhab nia tau raug lees paub thiab tau siv dav rau kev kuaj mob DRESS (Table 2). Cov qhab-nees yog raws li xya yam ntxwv: lub cev kub siab tshaj 38.5 ° C; Loj lymph nodes nyob rau hauv tsawg kawg yog ob qhov chaw; Eosinophilia; Atypical lymphocytosis; Cov pob khaus (npog ntau dua 50% ntawm thaj chaw ntawm lub cev, cov yam ntxwv morphological tshwm sim, lossis kev tshawb pom histological zoo ib yam nrog cov tshuaj tsis haum tshuaj); Kev koom tes ntawm cov kab mob ntxiv-cutaneous; Thiab ncua kev tso cai ntev (ntau tshaj 15 hnub).
Cov qhab nia nyob nruab nrab ntawm −4 txog 9, thiab kev kuaj xyuas qhov tseeb tuaj yeem muab faib ua plaub theem: tus qhab nia qis dua 2 qhia tias tsis muaj kab mob, 2 txog 3 qhia tias muaj kab mob tshwm sim, 4 txog 5 qhia tias muaj kab mob txaus ntshai, thiab ntau dua 5 qhia kev kuaj mob DRESS. Cov qhab-nees RegiSCAR yog qhov tshwj xeeb tshaj yog muaj txiaj ntsig zoo rau kev rov qab siv tau ntawm cov xwm txheej tshwm sim vim tias cov neeg mob yuav tsis tau ua tiav tag nrho cov kev kuaj mob ntxov ntxov ntawm tus kab mob lossis tsis tau txais kev ntsuam xyuas ua tiav nrog rau cov qhab nia.
DRESS yuav tsum muaj qhov txawv ntawm lwm yam kev tsis zoo ntawm daim tawv nqaij, suav nrog SJS thiab cov kab mob cuam tshuam, tshuaj lom epidermal necrolysis (TEN), thiab mob hnyav heev exfoliating impetigo (AGEP) (Daim duab 1B). Lub sij hawm incubation rau DRESS feem ntau yog ntev dua rau lwm yam kev tsis zoo ntawm daim tawv nqaij. SJS thiab TEN txhim kho sai thiab feem ntau daws lawv tus kheej li ntawm 3 mus rau 4 lub lis piam, thaum DRESS cov tsos mob zoo li mob ntxiv. Txawm hais tias mucosal kev koom tes hauv DRESS cov neeg mob yuav xav tau qhov txawv ntawm SJS lossis TEN, qhov ncauj qhov ntswg hauv DRESS feem ntau yog me me thiab los ntshav tsawg. Cov cim ntawm daim tawv nqaij edema tus yam ntxwv ntawm DRESS yuav ua rau catatonic thib ob hlwv thiab yaig, thaum SJS thiab TEN yog tus cwj pwm los ntawm tag nrho cov txheej txheej epidermal exfoliation nrog rau sab nraub qaum, feem ntau qhia Nikolsky lub cim zoo. Hauv qhov sib piv, AGEP feem ntau tshwm sim ntau teev rau hnub tom qab tau txais cov tshuaj thiab daws tau sai hauv 1 mus rau 2 lub lis piam. Cov pob liab liab ntawm AGEP yog nkhaus thiab tsim los ntawm cov pustules dav dav uas tsis nyob rau hauv cov hauv paus plaub hau, uas txawv me ntsis ntawm cov yam ntxwv ntawm DRESS.
Ib txoj kev tshawb fawb yav tom ntej tau pom tias 6.8% ntawm cov neeg mob DRESS muaj cov yam ntxwv ntawm SJS, TEN lossis AGEP, ntawm 2.5% tau txiav txim siab tias muaj kev cuam tshuam loj heev ntawm daim tawv nqaij. Kev siv RegiSCAR cov txheej txheem kev lees paub yuav pab txheeb xyuas cov xwm txheej no kom raug.
Tsis tas li ntawd, cov kab mob qhua pias zoo li cov kab mob tshwm sim feem ntau tshwm sim li ntawm 1 mus rau 2 lub lis piam tom qab kis tau cov tshuaj (rov kis tau sai dua), tab sis tsis zoo li DRESS, cov pob liab liab no feem ntau tsis tuaj nrog kev nce siab, nce eosinophilia, lossis lub sij hawm rov qab los ntawm cov tsos mob. DRESS kuj yuav tsum tau muab cais los ntawm lwm qhov chaw muaj kab mob, suav nrog hemophagocytic lymphohistiocytosis, vascular immunoblastic T-cell lymphoma, thiab mob graft-tiv thaiv tus tswv tsev.
Cov kws tshaj lij pom zoo lossis cov lus qhia txog kev kho DRESS tsis tau tsim; Cov lus pom zoo kho mob uas twb muaj lawm yog nyob ntawm cov ntaub ntawv soj ntsuam thiab cov kws tshaj lij kev xav. Cov kev tshawb fawb sib piv los qhia kev kho mob kuj tseem tsis muaj, yog li cov kev kho mob tsis zoo ib yam.
Clear kab mob-ua tshuaj kho mob
Thawj kauj ruam tseem ceeb tshaj plaws hauv DRESS yog txhawm rau txheeb xyuas thiab txiav tawm cov tshuaj uas yuav ua rau muaj kev phom sij tshaj plaws. Kev tsim cov kab kos qhia ntxaws ntxaws rau cov neeg mob tuaj yeem pab nrog cov txheej txheem no. Nrog rau daim ntawv qhia tshuaj, cov kws kho mob tuaj yeem sau tag nrho cov tshuaj uas ua rau muaj kab mob thiab txheeb xyuas qhov kev sib raug zoo ntawm lub sijhawm ntawm kev siv tshuaj thiab cov pob liab liab, eosinophilia, thiab kev koom tes hauv lub cev. Siv cov ntaub ntawv no, cov kws kho mob tuaj yeem tshuaj xyuas cov tshuaj feem ntau yuav ua rau DRESS thiab tsis txhob siv cov tshuaj ntawd raws sijhawm. Tsis tas li ntawd, cov kws kho mob tuaj yeem xa mus rau algorithms siv los txiav txim siab txog kev siv tshuaj rau lwm yam kev tsis zoo ntawm daim tawv nqaij.
Cov tshuaj - glucocorticoids
Systemic glucocorticoids yog lub hauv paus ntsiab lus ntawm inducing remission of DRESS thiab kho recurrence. Txawm hais tias qhov pib koob tshuaj yog 0.5 mus rau 1 mg / d / kg ib hnub ( ntsuas hauv qhov sib npaug ntawm prednisone), tsis muaj kev sim tshuaj ntsuam xyuas qhov ua tau zoo ntawm corticosteroids rau DRESS, nrog rau kev tshawb fawb txog ntau npaum li cas thiab kev kho mob. Cov koob tshuaj ntawm glucocorticoids yuav tsum tsis txhob txo qis kom txog thaum pom tseeb kev kho mob pom zoo, xws li txo cov pob liab liab, eosinophil penia, thiab rov ua haujlwm ntawm lub cev. Txhawm rau txo qhov kev pheej hmoo ntawm kev rov tshwm sim, nws raug nquahu kom maj mam txo cov koob tshuaj glucocorticoids tshaj 6 mus rau 12 lub lis piam. Yog tias tus qauv koob tshuaj tsis ua haujlwm, ces "kev poob siab" glucocorticoid kho, 250 mg txhua hnub (lossis sib npaug) rau 3 hnub, yuav raug txiav txim siab, ua raws li kev txo qis.
Rau cov neeg mob uas muaj DRESS me me, cov tshuaj corticosteroids zoo heev yuav yog ib qho kev kho mob zoo. Piv txwv li, Uhara et al. tshaj tawm tias 10 tus neeg mob DRESS tau zoo zoo yam tsis muaj cov kab mob glucocorticoids. Txawm li cas los xij, vim nws tsis paub meej tias cov neeg mob twg tuaj yeem zam kev kho mob zoo, kev siv tshuaj kho mob ntau yam tsis pom zoo ua lwm txoj hauv kev.
Tsis txhob siv tshuaj kho glucocorticoid thiab kev kho mob
Rau cov neeg mob DRESS, tshwj xeeb tshaj yog cov uas muaj kev pheej hmoo siab rau cov teeb meem (xws li kev kis kab mob) los ntawm kev siv cov tshuaj corticosteroids siab, kev kho corticosteroid zam yuav raug txiav txim siab. Txawm hais tias muaj cov ntaub ntawv tshaj tawm tias cov tshuaj txhaj tshuaj immunoglobulin (IVIG) tuaj yeem ua haujlwm tau zoo hauv qee kis, kev tshawb fawb qhib tau pom tias qhov kev kho mob muaj kev pheej hmoo siab ntawm kev tsis zoo, tshwj xeeb tshaj yog thromboembolism, ua rau ntau tus neeg mob hloov mus rau kev kho mob glucocorticoid. Lub peev xwm ua tau zoo ntawm IVIG tuaj yeem cuam tshuam nrog nws cov tshuaj tiv thaiv kab mob tshem tawm, uas yuav pab tiv thaiv kab mob los yog rov ua kom tus kab mob. Txawm li cas los xij, vim yog cov koob tshuaj loj ntawm IVIG, nws yuav tsis haum rau cov neeg mob uas muaj lub plawv tsis ua haujlwm, lub raum tsis ua haujlwm, lossis lub siab tsis ua haujlwm.
Lwm txoj kev kho mob muaj xws li mycophenolate, cyclosporin thiab cyclophosphamide. Los ntawm inhibiting T cell activation, cyclosporine blocks gene transcription ntawm cytokines xws li interleukin-5, yog li txo eosinophilic recruitment thiab tshuaj tshwj xeeb T cell activation. Ib txoj kev tshawb fawb nrog tsib tus neeg mob kho nrog cyclosporine thiab 21 cov neeg mob kho nrog cov kab mob glucocorticoids tau pom tias kev siv cyclosporine cuam tshuam nrog kev txo qis ntawm cov kab mob, txhim kho kev soj ntsuam thiab kuaj ntsuas, thiab nyob hauv tsev kho mob luv dua. Txawm li cas los xij, cyclosporine tsis yog tam sim no suav tias yog thawj kab kev kho mob rau DRESS. Azathioprine thiab mycophenolate feem ntau yog siv rau kev kho mob ntau dua li kev kho induction.
Monoclonal antibodies tau siv los kho DRESS. Cov no suav nrog Mepolizumab, Ralizumab, thiab benazumab uas thaiv interleukin-5 thiab nws cov receptor axis, Janus kinase inhibitors (xws li tofacitinib), thiab anti-CD20 monoclonal antibodies (xws li rituximab). Ntawm cov kev kho mob no, tshuaj tiv thaiv interleukin-5 yog suav tias yog kev siv tau ntau dua, muaj txiaj ntsig zoo thiab muaj kev nyab xeeb induction therapy. Cov txheej txheem ntawm kev ua tau zoo tuaj yeem cuam tshuam txog qhov nce siab ntawm interleukin-5 qib hauv DRESS, uas feem ntau yog tshwm sim los ntawm cov tshuaj tshwj xeeb T hlwb. Interleukin-5 yog lub luag haujlwm tseem ceeb ntawm eosinophils thiab yog lub luag haujlwm rau lawv txoj kev loj hlob, kev sib txawv, kev nrhiav neeg ua haujlwm, ua kom muaj sia nyob thiab muaj sia nyob. Cov tshuaj tiv thaiv interleukin-5 feem ntau yog siv los kho cov neeg mob uas tseem muaj eosinophilia lossis lub cev tsis ua haujlwm tom qab siv cov kab mob glucocorticoids.
Ntev ntawm kev kho mob
Kev kho mob ntawm DRESS yuav tsum ua kom tus kheej zoo heev thiab hloov kho raws li tus kab mob kev loj hlob thiab kev kho mob. Cov neeg mob DRESS feem ntau xav tau mus pw hauv tsev kho mob, thiab kwv yees li ib feem peb ntawm cov xwm txheej no xav tau kev saib xyuas hnyav. Thaum mus pw hauv tsev kho mob, tus neeg mob cov tsos mob raug soj ntsuam txhua hnub, kev kuaj xyuas lub cev tag nrho, thiab cov ntsuas ntsuas tau raug saib xyuas tsis tu ncua los ntsuas kev koom tes hauv nruab nrog cev thiab kev hloov pauv hauv eosinophils.
Tom qab tso tawm, kev soj ntsuam xyuas txhua lub lim tiam tseem yuav tsum tau saib xyuas cov kev hloov pauv ntawm tus mob thiab kho cov phiaj xwm kho raws sijhawm. Kev rov tshwm sim tuaj yeem tshwm sim tsis tu ncua thaum lub sijhawm koob tshuaj glucocorticoid poob qis lossis tom qab tshem tawm, thiab tuaj yeem tshwm sim raws li ib qho tsos mob lossis qhov mob hauv lub cev, yog li cov neeg mob yuav tsum tau saib xyuas mus sij hawm ntev thiab ua tiav.
Post lub sij hawm: Dec-14-2024