Hypereosinophilic Syndrome (ICD 10 - D72.1) yog ib qho mob uas qhov kev kuaj mob tseem ceeb yog qhov nce ntawm cov qe ntshav uas koom nrog pawg leukocyte, kuaj pom hauv cov hlab ntsha thiab ua rau, dhau mus, lub cev. kev ua haujlwm tsis zoo. Tam sim no, hauv zej zog kev tshawb fawb, pathology tsis suav tias yog ib qho kev ywj pheej nosological. Tab sis txawm li cas los xij, kev kuaj mob, cov tsos mob thiab kev kho mob ntawm hypereosinophilic syndrome yog qhov txaus siab rau ntau tus.
Kab mob kis
Txawm hais tias qhov mob no feem ntau pom hauv cov neeg laus, cov menyuam yaus tsis muaj qhov tshwj xeeb thiab, raws li kev txheeb cais qhia, ntawm lawv cov tub muaj mob ntau dua li cov ntxhais hauv qhov piv ntawm 4: 1.
Rau cov ncauj lus kom ntxaws ntxaws, nws yog ib qho tsim nyog yuav tsum nco qab lub luag haujlwm tseem ceeb ntawm eosinophils:
- Eosinophilic granulocytes yog ib qho ntawm cov tshuaj tiv thaiv kab mob hauv lub cev, raws li kev tshawb fawb soj ntsuam tsis ntev los no.
- Cov granulocytes tso tawm los ntawm granulocyte tswj cov peev xwm microbicidalcuam tshuam rau ob qho tib si txawv teb chaws thiab cov ntaub so ntswg ib puag ncig.
- Eosinophils ua lub luag haujlwm tseem ceeb hauv kev ua xua thiab tsim kev tiv thaiv kab mob anthelmintic.
- Koom nrog tswj cov ntaub so ntswg thiab kev tiv thaiv kab mob hauv tsev.
Hypereosinophilic syndrome hauv menyuam yaus feem ntau tshwm sim los ntawm kev ua xua, tab sis kuj tuaj yeem tshwm sim los ntawm cov txheej txheem autoimmune, hemato- thiab oncopathology. Hauv kev loj hlob ntawm cov kab mob no, cov noob caj noob ces kuj txawv - hauv cov menyuam yaus, qhov teeb meem no yuav cuam tshuam nrog trisomy ntawm 8th lossis 21st chromosome.
Kev faib tawm ntawm hypereosinophilic syndrome
By etiological factor:
- Reactive eosinophilia.
- YIdiopathic hypereosinophilic syndrome.
Rau qhov kuaj pom ntawm immunoglobulins hauv cov ntshav:
- Immunoglobulin-dependent eosinophilia yog tshwm sim los ntawm IgE.
- Immunoglobulin-independent.
Los ntawm qhov tseem ceeb hauv ib yam kab mob:
- YMyeloproliferative.
- YLymphoproliferative.
Myeloproliferative variant nthuav tawm nrog cov tsos mob hauv qab no hauv cov neeg mob:
- nce vitamin B12;
- myelofibrosis;
- spelenomegaly;
- teb rau imatinib (tyrosine kinase inhibitor);
- ntshav qab zib;
- thrombocytopenia.
Lymphoproliferative variant yog tshwm sim los ntawm clonal rearrangement ntawm T-cell receptor noob thiab yog tus yam ntxwv los ntawm cov tsos mob hauv qab no:
- CEC (kev tiv thaiv kab mobcomplexes);
- hypergammaglobulinemia (IgE);
- teb rau corticosteroid pab pawg kho;
- angioedema;
- Daim tawv nqaij anomalies.
Dab tsi yog qib ib txwm ntawm eosinophils hauv cov ntshav?
Cov ntsiab lus ib txwm ntawm eosinophilic granulocytes hauv cov ntshav peripheral yog li ntawm 1 txog 5 feem pua ntawm tag nrho cov leukocytes. Hauv cov ntsiab lus tseeb, tus nqi no yog los ntawm 120 txog 350 hlwb hauv 1 microliter.
Cov menyuam mos yug tshiab tuaj yeem muaj lub cev eosinophilia ntau dua 700 hlwb ntawm 1 µl, uas tseem suav tias yog cov ntshav eosinophil suav.
Yuav ua li cas yog cov txheej txheem tseem ceeb rau kev teeb tsa kab mob?
Ua ntej, nws tseem ceeb heev kom nkag siab tias eosinophilia yog tsim los ntawm kev tau txais qhov tseem ceeb ntawm eosinophilic granulocytes hauv cov ntshav peripheral, thiab raws li tus lej no, peb qib ntawm eosinophilia yog qhov txawv:
- I degree: me ntsis eosinophilia (ntawm 500 txog 1500 hlwb hauv 1 microliter).
- II degree: nruab nrab eosinophilia (ntawm 1500 txog 5000 hlwb hauv 1 microliter).
- III degree: hnyav eosinophilia (ntau dua 5000 hlwb hauv 1 microliter).
Peripheral ntshav eosinophilia >1500/microliter uas kav 6 lub hlis lossis ntau dua (!) yog qhov tseem ceeb rau kev kuaj mob.
Clinic
Cov tsos mob tsis tshwj xeeb suav nrog malaise, anorexia, poob phaus, mob plab, myalgia, kub taub hau, qaug zog hauvlub cev, uas yog, ashenization tshwm sim.
txhawm rau txheeb xyuas qhov tseem ceeb ntawm etiological, nws yog qhov tsim nyog los tsim kom muaj kev kho mob ua ntej, raws li cov tsos mob tseem ceeb ntawm tus kab mob:
- Cov kab mob hematological yog tus thawj coj thiab muaj tus yam ntxwv los ntawm: anemia, lymphadenopathy, splenomegaly, thrombocytopenia, thromboemboli.
- Intoxication syndrome yuav raug qhia nyob rau hauv cov kab mob xws li: myeloproliferative pathologies, lymphogranulomatosis, lymphocytic leukemia.
- Bronchopulmonary (bronchial hawb pob, periarthritis nodosa, bronchopulmonary aspergillosis).
- Cardiopulmonary syndrome yog tus cwj pwm los ntawm kev tsim ntawm parietal thrombi nrog emboli.
- Cov kab mob plab hnyuv muaj cov tsos mob xws li mob plab, quav quav, thiab ntuav.
- Cov tawv nqaij tawv nqaij tuaj yeem tshwm sim nrog atopic dermatitis, angioedema, pruritus, urticaria, dermatographism.
Kev puas tsuaj rau cov kabmob hauv cov kabmob no yog vim lawv qhov nkag mus los ntawm eosinophils, uas tuaj yeem ua rau ntau lub cev tsis ua haujlwm. Cov kabmob xws li lub plawv, tawv nqaij, tus po, lub paj hlwb, thiab lub ntsws tuaj yeem koom nrog.
Pathogenesis
Cov kws tshaj lij txheeb xyuas cov txheej txheem tseem ceeb. Nov yog:
- Antibody-dependent chemotaxis uas tsim thaum lub sij hawm helminthic invasions (qhov no yog pov thawj los ntawm cov tsos ntawm IgE thiab IgG).
- Cov txheej txheem qog nqaij hlav, qee qhov tuaj yeem tso tawm eosinophilic chemotactic factor.
- Tumor eosinophilia (leukemia).
Howpaub?
Kev kuaj mob ntawm hypereosinophilic syndrome yog raws li kev cais tawm ntawm lwm yam ua rau eosinophilia. Piv txwv li, kis kab mob parasitic. Ntawd yog, nws yog ib qho kev kuaj mob ntawm kev cais tawm thiab tsim yog tias qhov etiology ntawm qhov tshwm sim tsis tuaj yeem tsim.
Lub chaw kuaj tseem ceeb thiab cov cuab yeej siv los kuaj xyuas tus mob no yog raws li hauv qab no:
- Leukogram qhia txog tus lej ntawm eosinophilic granulocytes.
- Ntshav biochemistry (lub siab enzymes, creatine kinase, GFR, urea, troponin, mob theem proteins).
- Immunology ntawm hypereosinophilic syndrome. Cov ntsuas xws li cov tshuaj tiv thaiv kab mob antinuclear, cationic proteins, IgE, lymphogram.
- tshuaj ntsuam quav quav rau cysts, qe.
- Electrocardiography.
- Echocardiography.
- ntsuas ntsuas ntawm cov kab mob ua pa (radiography).
- Computed tomography ntawm lub hauv siab thiab plab.
- Nyob rau hauv kev tshuaj xyuas xws li cov pob txha pob txha, ob leeg paub tab eosinophils thiab progenitor cells yuav raug kuaj pom.
- Kuj ua ib qho kev kuaj mob hlwb, uas suav nrog: kev kuaj mob ntawm tus menyuam, kuaj xyuas qhov cuam tshuam, electroencephalography, kev kuaj mob ntawm fundus.
Huab cua
Kev tsis pom zoo rau cov mob hypereosinophilic hauv cov menyuam feem ntau yog vim muaj teeb meem uas feem ntau tshwm sim los ntawm kev ua haujlwm tsis zoo ntawm qee yam kabmob - feem ntau, qhov no yog lub plawv. Lub plawv tsis ua haujlwm tuaj yeem ua rau muaj kev tsis taus thiab txawm tuag.
Kev kho mob ntawm pathology
Kev kho mobpib nrog kev teem caij ntawm glucocorticosteroid prednisolone, ua raws li imatinib, cov tshuaj uas tswj cov ntsiab lus ntawm eosinophils, piv txwv li, Interferon-alpha thiab Etoposide.
"Imatinib" yog cov tshuaj tiv thaiv kab mob, ib qho inhibitor ntawm tyrosine kinase, ib qho enzyme. Synthesized nyob rau hauv chronic myeloid leukemia.
"Etoposide" yog ib qho tshuaj tiv thaiv kab mob uas muaj cov nyhuv cytotoxic. Nws yuav tsum nco ntsoov tias cov tshuaj no muaj kev txwv rau kev siv: nws yog contraindicated rau cov menyuam yaus hnub nyoog qis dua ob xyoos vim qhov tseeb tias hauv kev tshawb fawb soj ntsuam nws txoj kev nyab xeeb rau cov menyuam yaus hnub nyoog qis dua ob xyoos thiab, hauv cov ntsiab lus, nws tsis tau muaj pov thawj.).
Cov txiaj ntsig ntawm glucocorticoids yog inhibit qhov kev loj hlob ntawm kev loj hlob ntawm cov kab mob eosinophilic ntawm granulocytes, lawv qhov ua kom muaj zog. Leukotriene inhibitors, phosphodiesterase inhibitors, tshuaj myelosuppressive kuj siv tau rau lub hom phiaj no.
Kev txhawb nqa kev kho mob yog siv rau cov tsos mob uas qhia tias lub plawv tseem koom nrog hauv cov txheej txheem - qhov no tuaj yeem tshwm sim nws tus kheej li infiltrative cardiomyopathy, kab mob valvular, plawv tsis ua haujlwm). Anticoagulants, tshuaj antiplatelet ("Aspirin", "Clopidogrel") tuaj yeem siv.
Yuav tsum muaj kev sab laj ntawm cov kws tshaj lij txhawm rau xaiv txoj kev kho mob. Nrhiav kev pab los ntawm cov hauv qab noCov kws kho mob: tus kws kho mob hematologist (xaiv kev saib xyuas mob hnyav rau tus neeg mob), kws kho mob dermatologist (nws txoj kev kho mob yog qhov tsim nyog rau cov tawv nqaij tshwm sim ntawm tus mob), tus kws kho mob hlwb (koom nrog cov txheej txheem thaum muaj kev puas siab puas ntsws tshwm sim), kws kho plawv, pulmonologist.
Zoo kawg
Nws yuav tsum nco ntsoov tias hypereosinophilic syndrome xav tau kev kho mob tsim nyog. Tsis muaj qhov xwm txheej zoo li no yuav tsum tsis quav ntsej txog, txij li thaum muaj teeb meem nws feem ntau ua rau tuag.
Nws tsis tsim nyog cia siab tias txhua yam yuav ploj mus ntawm nws tus kheej - tsuas yog mus ntsib kws kho mob raws sijhawm thiab kev kho kom raug tuaj yeem lav qhov ua tiav ntawm kev ntsuas kev kho mob.
