Ntau xyoo dhau los, kev kawm tseem ceeb tau ua tiav hauv kev kawm txog cov yam ntxwv ntawm cov kab mob macrophage, uas yog suav tias yog cov lim roj ntsha ntawm cov ntshav thiab cov qog ntshav, pab tshem tawm cov kab mob, cov hlwb cuam tshuam los ntawm cov qog thiab cov kab mob, co toxins, metabolites, tshuaj residues.
Hemophagocytic syndrome raws li daim ntawv nosological suav nrog ib pab pawg ntawm cov kab mob kis tau thiab lub cev xeeb tub, uas yog raws li kev tswj hwm ntawm lub cev tiv thaiv kab mob rau qhov tsis txaus ntseeg hauv kev ua kom cytotoxic T-lymphocytes thiab macrophages thiab tshwm sim ntawm kev puas tsuaj rau cov cuam tshuam. inflammatory organ mediated by them.
Basic concept
Lub tswv yim ntawm "hemophagocytosis" txhais tau hais tias pathomorphological tshwm sim ntawm phagocytosis ntawm cov ntshav zoo li qub los ntawm cov ntaub so ntswg macrophages. Qhov tshwm sim no muaj ntau heev thiab tsis suav hais tias yog ib qho lus hais rau lymphohistiocytosis ntawm hom hemophagocytic raws li daim ntawv nosological.
Kev mob siab rau cov kab mob no hauv kev kho mob ntawm cov kws kho mob tshwj xeeb yog vim qhov tseeb tias nyob rau theem kev kuaj mob, cov neeg mob uas muaj cov kab mob ntawm cov kab mob hemophagocytic lymphohistiocytosis, vim qhov peculiarities ntawm lawv qhov kev nthuav qhia kho mob, feem ntau tau sau tseg hauv Cov kab mob sib kis hauv tsev kho mob nrog kev kuaj mob zoo ib yam, xws li, piv txwv li, kab mob hauv plab lossis cov txheej txheem cuam tshuam nrog sepsis thiab feem ntau ua rau muaj kev phom sij ntev rau qhov kuaj pom tseeb.
Ntawm qhov tod tes, cov kev kawm ntawm ntau yam kab mob banal tuaj yeem cuam tshuam los ntawm kev txhim kho lub neej-kev phom sij thib ob hemophagocytic syndrome. Nws yuav tsum tau, ntxiv rau cov txheej txheem etiotropic kev kho mob, immunosuppressive thiab immunomodulatory txoj kev kho, uas yuav tsum tau tsom rau kev tswj cov kab mob ua kom lub cev tiv thaiv kab mob. Raws li kev faib tawm uas tau txais los ntawm Lub Koom Haum rau Kev Kawm ntawm Histiocytosis, hemophagocytic lymphohistiocytosis yog cais raws li kab mob uas muaj cov duab kho mob sib txawv.
Pib, uas yog, tau txiav txim siab hemophagocytic syndromes suav nrog tsev neeg lymphohistiocytosis thiab qee qhov tsis tshua muaj kev tiv thaiv kab mob. Qhov thib ob suav nrog cov ntaub ntawv uas tsim nyob rau hauv cov ntsiab lus ntawm qog, kis thiab autoimmune pathological phenomena.
Nws yuav tsum tau hais meej tias raws li cov txheej txheem molecular ntawm qhov tshwm sim ntawm pathology tau kawm, kab ntawm caj ces txiav txim siab thiabSecondary lymphohistiocytosis ho poob clarity.
Hemphagocytic syndrome tshwm sim li cas hauv cov neeg laus thiab menyuam yaus?
Symptomatics
Daim duab kho mob ntawm tus kab mob yog tus cwj pwm los ntawm kev ua npaws ntev, rov ua rau cov tshuaj tua kab mob, splenomegaly, hemorrhagic syndrome, edematous syndrome, hepatomegaly, cov tsos mob ntawm CNS puas. Cov txheej txheem pathological feem ntau pib mob hnyav heev. Qhov tshwm sim rau kev kho mob tshwm sim ntawm pathology yog kab mob banal.
Lub psychomotor thiab kev loj hlob ntawm lub cev ntawm tus neeg mob kom txog thaum pib ntawm hemophagocytic syndrome feem ntau tsis raug kev txom nyem. Thaum thawj theem ntawm kev kuaj mob, kab mob intrauterine feem ntau kuaj pom, los yog (nyob rau theem tom qab ntawm kev loj hlob ntawm tus kab mob) meningoencephalitis lossis sepsis. Intractable kub taub hau thiab tshwm sim ntawm intoxication yog lub ntsiab yog vim li cas rau lub tsev kho mob nyob rau hauv lub tsev kho mob ntawm cov kab mob kis kab mob nyob rau hauv lub tsev kho mob, qhov twg cov kev kuaj mob feem ntau tuaj yeem paub meej tias muaj kab mob nrog cov kab mob uas feem ntau siv hauv kev kuaj mob. Kev teem caij kho etiotropic tsis ua rau muaj kev txhim kho tseem ceeb hauv tus neeg mob tus mob.
Kev ua haujlwm ib ntus ntawm tus mob tuaj yeem raug sau tseg nrog kev teem sijhawm ntawm corticosteroids raws li qhov mob hnyav. Txawm li cas los xij, ua npaws feem ntau tshwm sim, hepatosplenomegaly zuj zus, hemorrhagic syndrome pib tshwm sim, cov tsos mob ntawm lub paj hlwb nce zuj zus thiab suav nrog kev chim siab heev, ntuav, tsis kam ua.noj, qaug dab peg, mob meningeal.
Qee qhov xwm txheej, qhov pib ntawm cov txheej txheem pathological dhau los ntawm kev sib cais ntawm cov kab mob encephalomyelitis, encephalitis, thiab rov tshwm sim tuaj yeem ua rau muaj qhov tsis txaus ntawm cov paj hlwb. Raws li txoj cai, cov kev kho mob tshwm sim ntawm hemophagocytic syndrome nyob rau hauv cov me nyuam thiab cov neeg laus yog complemented los ntawm cov yam ntxwv kev hloov nyob rau hauv lub chaw soj nstuam tsis, uas yuav tsum muaj xws li peripheral ntshav cytopenia, uas muaj ob los yog ntau tshaj ceg ntawm hematopoiesis..
Anemia thiab thrombocytopenia
Feem ntau, ntshav liab thiab thrombocytopenia raug kuaj pom nyob rau theem pib ntawm tus kab mob, thiab thaum nws loj tuaj, neutropenia thiab leukopenia tsim. Qhov tshwm sim ntawm coagulopathy kuj yog yam ntxwv, uas feem ntau tshwm sim los ntawm hypofibrinogenemia, me ntsis tsawg dua - nyob rau hauv daim ntawv ntawm tag nrho cov hypocoagulation.
Ntawm cov txiaj ntsig ntawm kev kuaj ntshav biochemical, hypoalbuminemia, hypertriglyceridemia, hyponatremia, nce qib ntawm lactate dehydrogenase thiab bilirubin yog cov yam ntxwv tshaj plaws.
Npaj ferritin
Nws kuj yog tus yam ntxwv tseem ceeb kom nce cov ntsiab lus ntawm ferritin hauv cov ntshav, qee zaum mus rau qib siab (ntau dua 10,000 mcg / l). Kev kuaj xyuas ntawm cov kua cerebrospinal qhia tau hais tias nruab nrab monocytic thiab lymphocytic pleocytosis, nce qib protein ntau.
Thaum ua tus myelogram siv tus qauv staining, daim duab polymorphic tau pom nyob rau hauv cov pob txha pob txha nrog qhib.macrophages los yog monocytes, nrog rau cov phenomena ntawm phagocytosis nyob rau hauv cellular ntsiab (erythrocytes, tsawg dua platelets thiab leukocytes). Qhov tshwj xeeb tshaj plaws tshwm sim hauv kev tshawb fawb hauv chav kuaj ntawm hemophagocytic syndrome yog qhov txo qis hauv NK cell cytotoxicity.
Kev faib tawm
Hemophagocytic lymphohistiocytosis yog categorized li "H" nyob rau hauv niaj hnub kev faib tawm ntawm histiocytosis. Cov ntaub ntawv tseem ceeb ntawm tus kab mob no yog qhov txawv, vim paub txog caj ces tsis xws luag, thiab theem nrab, uas tsis cuam tshuam nrog kev hloov pauv caj ces tsis zoo.
Kev faib tawm tag nrho ntawm cov kab mob no:
- primary syndrome: hereditary pathologies ua rau kev loj hlob ntawm HLH;
- HLH cuam tshuam nrog qhov tsis xws luag hauv lymphocyte cytotoxicity;
- HLH cuam tshuam nrog cov kab mob ua kom tsis zoo;
- HLH cuam tshuam nrog cov kab mob caj ces uas cuam tshuam rau kev txhim kho cov txheej txheem inflammatory;
- tsev neeg cov ntaub ntawv ntawm HF uas tsis paub txog caj ces tsis xws luag;
- thib ob hemophagocytic syndrome cuam tshuam nrog kev kis mob;
- HLH-associated virus;
- HLH cuam tshuam nrog kab mob;
- HFH cuam tshuam nrog cov kab mob fungal;
- HLH cuam tshuam nrog tus kab mob cab;
- HLH, uas cuam tshuam nrog kev ua phem;
- CHLH cuam tshuam nrog kev coj tus cwj pwm khomob (tsis cuam tshuam nrog thawj qhov pib ntawm malignancy);
- HLH cuam tshuam nrog kev txhim kho cov kab mob rheumatic;
- GLG,cuam tshuam nrog kev hloov pauv;
- HLH, uas yog txuam nrog iatrogenic ua kom lub cev tsis muaj zog;
- iatrogenic immunosuppression syndrome;
- hemophagocytic syndrome txuam nrog sepsis.
Procalcitonin
Procalcitonin yog ib qho chaw sib xyaw ua ke ntawm glycoproteins ntawm lawv cov protein-carbohydrate complex, uas muaj 116 amino acids. Nws yog ib tug precursor rau calcitonin, ib tug hormonal compound tsim los ntawm C-cells nyob rau hauv cov thyroid caj pas.
Procalcitonin thaum lub sijhawm cov txheej txheem metabolic decomposes rau hauv calcitonin, katacalcin thiab terminal peptide. Kev nce qib ntawm cov tshuaj no feem ntau pom nyob rau hauv cov qog nqaij hlav qog nqaij hlav me me, uas qhia tias nws tsim tsis yog hauv cov thyroid caj pas xwb. Tsis tas li ntawd, qhov txaus nyiam tshaj plaws, qhov kev hloov pauv ntawm qhov concentration ntawm procalcitonin hauv hemophagocytic syndrome hauv cov menyuam yaus yog qhov tshwj xeeb tshaj tawm.
Qhov no yog qhov ua rau muaj kev phom sij txaus ntshai, uas yog raws li kev tswj hwm ntawm lub cev tiv thaiv kab mob vim yog kev hloov pauv hauv cov noob uas tswj cov haujlwm cytolytic, ua rau muaj kev ua haujlwm tsis zoo ntawm cytotoxic monocytes, T-lymphocytes, macrophages thiab tshwm sim. ib tug systemic inflammatory teb nyob rau hauv tus me nyuam. Tus kab mob no belongs rau qeb ntawm histiocytic, uas tsim los ntawm cov hlwb ntawm macrophage series. Nws yog ib qho tseem ceeb heev uas yuav tsum tau ua qhov kev kuaj mob hauv chav kuaj kom pom cov qib ntawm procalcitonin thiabLwm cov tshuaj tshwj xeeb hauv cov ntshav.
Secondary Syndrome nws loj hlob li cas?
Secondary hemophagocytic syndrome nyob rau hauv cov menyuam yaus tshwm sim, raws li txoj cai, nrog kev kis kab mob, rheumatic pathologies, qog malignant thiab kab mob oncohematological. Kev sib koom ua ke ntawm HPS ntawm qhov xwm txheej thib ob nrog ntau tus kab mob tau raug pov thawj, uas suav nrog cov kab mob (herpesvirus, HIV, influenza A, parvovirus B19, adenovirus), kab mob (mycobacteria, salmonella, mycoplasma, rickettsia, pneumococcus), fungi thiab protozoa.
Herperviruses
Ntawm cov kab mob feem ntau cuam tshuam nrog cov kab mob hemophagocytic theem nrab, herpesviruses yog thawj qhov chaw. Nyob rau tib lub sijhawm, tus kab mob no tuaj yeem ua rau muaj ntau yam kab mob sib kis, xws li visceral leishmaniasis, leptospirosis thiab sepsis.
HFS complex suav nrog splenomegaly, ua npaws, CNS puas, kab mob siab, coagulation disorders, cytopenias, ntau cov cim biochemical. Cov tsos mob saum toj no tsis yog qhov tshwj xeeb, uas ua rau muaj teeb meem loj rau kev kuaj mob ntawm cov kab mob pathological, tshwj xeeb tshaj yog nyob rau theem pib, ua rau muaj qhov tsis meej pem txog kev ua neej, tshwj xeeb tshaj yog nyob rau hauv cov neeg mob immunocompromised.
Yuav kuaj mob ntshav qab zib theem nrab thiab thawj hemophagocytic li cas?
Diagnosis
Kev kuaj mob thawj zaug ntawm tus kab mob suav nrog kev sau ntawm anamnesis thiab kev tsis txaus siab ntawm tus neeg mob, nrog rau kev kuaj lub cev. Tsis tas li ntawd, qhov tsim nyog kuaj kev kuaj mob yog nqa tawm, uas yuav tsum tau qhia meejcov ntshav ntawm cov tshuaj hauv qab no:
- bilirubin ncaj qha thiab tag nrho;
- ALT/ACT;
- LDG;
- albumin;
- sodium;
- triglycerides;
- ferritin;
- urea;
Ntxiv rau, xav tau ib coagulogram, kev tshawb fawb cerebrospinal kua, cytometry, thiab lwm yam.
Instrumental diagnostics
ntsuas ntsuas ntsuas suav nrog:
- MRI ntawm lub hlwb nrog gadolinium sib piv;
- CT ntsws;
- Ultrasound ntawm lub plab kab noj hniav;
- x-ray.
Nrhiav seb qhov kev cia siab rau hemophagocytic syndrome hauv cov menyuam yaus thiab cov neeg laus yog dab tsi?
Kev kho mob
Lub hom phiaj ntawm kev saib xyuas kev noj qab haus huv yog txhawm rau txo qis cov lus teb inflammatory, rov ua haujlwm hauv nruab nrog cev, thiab ua cov hematopoietic qia cell transplantation. HSCT yog tib txoj kev kho mob xwb. Tom ntej no, cov tshuaj tiv thaiv kab mob tiv thaiv kab mob tau ua tiav, ua ke nrog kev siv tshuaj: Dexamethasone, Methotrexate, Prednisolone, Cyclospogrin A, thiab lwm yam.
Lub sijhawm ntawm kev kho mob hemophagocytic syndrome thiab kev tsim cov koob tshuaj yog ua los ntawm kws kho mob.
Kev hloov pauv
Cov tswv yim niaj hnub rau kev kho mob ntawm cov kab mob qog nqaij hlav lymphohistiocytosis suav nrog tsis yog cov hau kev saum toj no xwb. Cov kev mob tshwm sim ntawm tus kab mob no tau txhim kho zoo nrog rau lub sijhawm ua haujlwm ntawm kev hloov pauv los ntawm qhov sib xwsntsig txog tus pub. Nrog cov kab mob, kab mob thiab kab mob parasitic uas provoked cov tsos mob ntawm tus kab mob no, etiotropic antimicrobial therapy, high-dose immunoglobulin infusions, thiab kev kho immunosuppressive yog siv. Qee zaum qhia txog kev hloov pob txha pob txha.
Kev Ntsuas rau hemophagocytic syndrome
Qhov no ncaj qha ntsig txog kev tsis saib xyuas ntawm cov txheej txheem pathological thiab nws daim ntawv. Cov tsos mob tseem ceeb yog qhov nyuaj rau kev kho mob, thiab qhov kev pheej hmoo ntawm kev rov qab los kuj muaj tsawg. Ib qho kev pom tsis zoo heev hauv cov menyuam yaus uas muaj cov kab mob hauv lub cev - nrog kev kho tsis raws sijhawm thiab cov ntaub ntawv nyuaj ntawm cov kab mob, cov neeg tuag tau siab heev.
