Autoimmune lymphoproliferative syndrome yog ib hom kab mob pathological. belongs rau qeb ntawm heterogeneous. Muaj ob hom kev qub txeeg qub teg: autosomal dominant thiab recessive. Nyob rau hauv tsawg zaus, qhov ua rau yog somatic mutations. Lymphoproliferative syndrome yuav kis tau.
History and Facts
Tus thawj x-txuas lymphoproliferative syndrome nyob rau hauv cov tub tau raug lees paub thiab formalized nyob rau hauv science nyob rau hauv 1967 thawj zaug. Txij li xyoo 1976, nws tau raug cais raws li kev tiv thaiv kab mob tseem ceeb. Kev saib xyuas ntawm cov kws tshawb fawb rau cov kab mob pathological tau riveted txij li lub xyoo dhau los ntawm lub xyoo pua xeem. Txawm li ntawd los, nws tau tshwm sim tias qhov txawv txav lymphocytic apoptosis dhau los ua lub hauv paus rau kev loj hlob ntawm tus kab mob.
Qhia tawm cov yam ntxwv ntawm autoimmune lymphoproliferative syndrome, cov kws tshawb fawb tau pom tias txhua tus neeg mob muaj qhov txawv txav ntawm fasl membrane receptors, CD95. Nws yog qhov nuance uas txiav txim siab txog kev muaj peev xwm piav qhia txog caj cescell tuag. Cov kab mob pathological tshwm sim nyob rau hauv rooj plaub ntawm kev hloov pauv noob uas cuam tshuam rau apoptosis.
Biology thiab anatomy: nws ua haujlwm li cas?
Nyob rau hauv autoimmune lymphoproliferative syndrome nyob rau hauv ib tug me nyuam, CD95 qhia yog nyob rau hauv T, B hlwb. Hyperplasia yog kuaj tau nyob rau hauv daim ntawv mob ntawm cov qog nqaij hlav. Kom meej meej, cov txheej txheem pathological yog pom nyob rau hauv cov qog nqaij hlav, spleen. Lub siab mob hnyav heev. Qhov feem pua ntawm cov qauv cellular cuam tshuam vim cov txheej txheem pathological yog T, CD4, CD8 hlwb. Cov kws tshawb fawb qhia tias cov qauv no, ua ntej raug cuam tshuam los ntawm cov txheej txheem tsis raug, tej zaum yuav yog cov neeg laus CTLs, uas, vim yog vim li cas caj ces, tau poob lub peev xwm los qhia cov coreceptors. Nyob rau tib lub sijhawm, cov qauv ntawm tes tau hloov mus rau hauv cov polyclonal thiab tau txais kev qhia ntawm lwm cov ntsiab lus. Qhov no ua rau muaj kev tsim tawm ntau dhau ntawm IL-10, cov hlwb uas txhawb nqa autoimmunity.
Hauv kev kho mob propaedeutics, lymphoproliferative syndrome feem ntau muab faib ua ntau yam. Rau kev faib tawm, cov yam ntxwv ntawm qhov sib txawv ntawm cov noob caj noob ces ntawm ib rooj plaub tshwj xeeb raug coj mus rau hauv tus account. Cov noob hloov pauv tuaj yeem cuam tshuam rau qhov thib yim thiab thib kaum caspases, CD95, CD178. Nyob rau tib lub sijhawm, nws yuav tsum raug sau tseg tias tsis muaj kev lees paub dav dav ntawm cov xwm txheej hauv pawg.
Txoj kev tshwm sim
Cov tsos mob ntawm tus kab mob no muaj ntau haiv neeg. Feem ntau x-linked lymphoproliferative syndrome tau kuaj pom hauv thawj xyoo ntawm lub neej,tsawg zaus - thaum muaj hnub nyoog laus (txog kaum tsib). Cov tsos mob tseem ceeb yog kev loj hlob ntawm cov ntaub so ntswg lymphoid, uas provokes splenomegaly, lymphadenopathy. Cov phenomena yog muaj nyob rau hauv cov xwm txheej ntev ntawm chav kawm. Nyob rau tib lub sijhawm, tus neeg mob raug kev txom nyem los ntawm kev tshwm sim ntawm autoimmune imbalance. Kev ntsuam xyuas pab txheeb xyuas autoimmune cytopenia. Nws yog ua tau nyob rau hauv daim ntawv ntawm neutro-, thrombocytopenia, anemia. Tsawg zaus, cytopenia tshwm sim ua ntej kev loj hlob ntawm cov ntaub so ntswg lymphoid.
X-txuas lymphoproliferative syndrome provokes kev cuam tshuam hauv kev ua haujlwm ntawm hematopoietic, circulatory systems. Raws li txoj cai, kab mob siab ntawm tus kab mob autoimmune yog kho. Ntau tus neeg raug kev txom nyem los ntawm eczema, glomerulonephritis. Cov neeg mob muaj cov tsos mob ntawm uveitis, thyroiditis. Kwv yees li 1 ntawm 10 tus kab mob B-cell lymphoma nyob rau lub sijhawm.
Kev kho mob tshwm sim
Lymphoproliferative syndrome hauv cov menyuam yaus muaj ntau yam zoo li. Qhov tshwj xeeb tshaj plaws yog lymphoproliferation. Cov txheej txheem yog tus cwj pwm los ntawm tus cwj pwm benign, tus mob pathological yog mob ntev. Feem ntau tsim nyob rau hauv cov me nyuam thaum yau, tej zaum nws yog tsim nyob rau hauv cov me nyuam muaj ib xyoos. Tus mob tseem nyob rau rau lub hlis lossis ntau dua. Nyob rau tib lub sijhawm, tsis tu ncua proliferation ntawm lymph nodes ntawm peripheral lymphatic system yog pom. Txhawm rau kuaj mob, nws yuav tsum tau txheeb xyuas cov txheej txheem no hauv peb pawg ntawm cov nodes lossis ntau dua. Cov pob caus yog ntom, tsis soldered rau cov ntaub so ntswg nyob ze. Rau ntau, kev sim pab tshawb nrhiav hepatosplenomegaly.
X-txuas lymphoproliferative syndrome hauv cov menyuam yaus pom nws tus kheej ua cov tsos mob autoimmune. Lub classic variant yog anemia,Neutro-, thrombocytopenia. Ua tau vasculitis. Feem ntau muaj mob caj dab, kab mob siab. Cov neeg mob feem ntau ua rau uveitis, glomerulonephritis, thyroiditis. Qee yam kab mob autoimmune tuaj yeem ua tau.
Paj siab
Lymphoproliferative syndrome yog txuam nrog qhov muaj feem ntau ntawm kev tsim cov kab mob malignant. Cov txheej txheem localization cheeb tsam yog unpredictable. Kev ua tsis raug apoptosis, kev ua haujlwm uas cuam tshuam nrog kev ua haujlwm ntawm Fas receptors, ua rau txo qis hauv kev tswj hwm cov txheej txheem ntawm cov ntaub so ntswg loj hlob. Lub peev xwm ciaj sia nyob rau hauv cov hlwb uas tau dhau los ntawm kev hloov pauv pathological tau loj hlob. Feem ntau, cov noob no yog ib yam uas cuam tshuam kev loj hlob ntawm cov qog nqaij hlav.
Ntau zaus tus kab mob no nrog rau kev tsim ntawm hom B, T lymphomas. Tsis tas li ntawd, muaj qhov tshwm sim ntawm cov kab mob qog noj ntshav hauv cov qog mammary, plab hnyuv, thiab cov kab mob ua pa. Myelo-lymphoproliferative syndrome nrog rau qib siab ntawm qhov tshwm sim tuaj yeem ua rau lymphogranulomatosis.
Nyob rau hauv tus kab mob autoimmune, tus neeg mob yog nquag mus rau urticaria, vasculitis. Ib txhia muaj kev loj hlob qeeb ntawm lub cev.
Qhia meej txog kev kuaj mob
Lymphoproliferative syndrome yog kuaj tau yog tias tsis yog mob qog noj ntshav lymphadenopathy raug tsim. Ua tau splenomegaly. Kev kuaj mob yog tsim los ntawm kev sib xyaw ua ke ntawm ob qhov tshwm sim los yog muaj ib qho ntawm lawv, yog tias lub sijhawm ntawm kev loj hlob ntawm tus mob yog rau lub hlis lossis ntau dua. Yog tias xav tias muaj kev kuaj mob, tus neeg mob yuav tsum raug xa mus kuaj. Hauv vitrotsim kom muaj qhov tsis ua tiav ntawm kev kho cov lymphocyte apoptosis, qhia meej qhov concentration ntawm cellular qauv CD4, CD8 T: yog tias cov ntsiab lus ntau dua 1%, peb tuaj yeem tham txog cov kab mob pathological.
Nyob rau hauv lymphoproliferative syndrome, genetic kev tshawb fawb qhia pom muaj cov noob hloov. Hauv kab mob autoimmune, ntau tus cim tshwj xeeb tuaj yeem ua tau uas muaj nyob hauv tus kheej. Muaj ob peb paub uas tam sim no siv los ua tus pab cuam hauv kev kuaj mob sib txawv. Lymphoproliferative syndrome tuaj yeem qhia nws tus kheej li cov cim ntawm kev ua haujlwm ntawm T-hom cell qauv, qhov nce ntawm CD5 + B hlwb. Kev tsom xam histological tso cai rau koj pom cov follicular proliferation ntawm lymphoid cov ntaub so ntswg nyob rau hauv cov pob txha, dawb pulp.
Txoj kev ntawm rooj plaub
Cov hauv qab no yog ib hom kab mob uas tsis cuam tshuam nrog kev ua txhaum ntawm caj ces thaum lub sij hawm kev loj hlob ntawm lub embryo. Cov hauv qab no siv tau rau daim ntawv ntawm tus kab mob.
Lymphoproliferative syndrome yog cov tsos mob nyuaj uas tuaj yeem nrog tsis tau tsuas yog lymphocytic leukemia, uas tshwm sim raws li qhov xwm txheej, tab sis kuj muaj ntau hom kab mob tsawg. Qee lub sij hawm nws tau nruab nrog cov plaub hau cell leukemia, lymphatic, uas yog ib qho teeb meem ntawm cytolysis. Nws yog paub tias ib tug complex ntawm cov tsos mob tuaj yeem tshwm sim tawm tsam keeb kwm ntawm kev kho tshuaj, hluav taws xob, thiab kev cuam tshuam ntawm cov tshuaj lom neeg. Ntau xim nyob rau hauv niaj hnub tshuaj attractspost-transplant lymphoproliferative syndrome, uas ua rau muaj kev pheej hmoo ntawm tus neeg tau txais kev phais. Nyob rau hauv txoj kev loj hlob ntawm tus mob uas tsis tau txais txiaj ntsig, kev cuam tshuam ntawm retroviruses muaj zog tshaj plaws.
Nuances and prevalence
Cov ntaub ntawv kho mob qhia tau tias feem pua ntawm cov neeg mob uas muaj lymphoproliferative syndrome yog cov neeg muaj hnub nyoog tshaj tsib caug xyoo. Qee zaum, tus kab mob no tau kuaj pom hauv cov hnub nyoog qis dua 25, tab sis cov xwm txheej zoo li no tsis tshua muaj. Ntawm cov txiv neej, qhov zaus ntawm qhov tshwm sim yog qhov nruab nrab ob zaug siab li ntawm cov poj niam. Raws li cov chav kawm, lawv hais txog ib tug benign daim ntawv, splenomegalic, qog, nquag mus sai sai, cuam tshuam rau cov pob txha pob txha, plab kab noj hniav. Kuj tseem muaj hom prolymphocytic.
Thaum tus mob lymphoproliferative syndrome tab tom pib tsim, cov kab mob sab hauv tsis thab, tus neeg xav tias txaus siab, tsis muaj kev tsis txaus siab. Qee qhov kev ceeb toom tsis muaj zog, nyiam ua khaub thuas. Cov qog hws ua haujlwm me ntsis ntau dua li cov qauv. Tus kab mob nyob rau theem no tuaj yeem kuaj tau raws li ib feem ntawm kev kuaj xyuas kev tiv thaiv lossis kev kuaj random. Cov cim qhia tseem ceeb yog cov qog ntshav qog ntshav loj, lymphocytosis, nce ntxiv ntawm cov leukocytes hauv cov hlab ntshav.
Cov tsos mob tshwj xeeb
Thaum tus kab mob no ua rau cov qog nqaij hlav hauv caj dab, hauv caj dab. Ib me ntsis tom qab, thaum tus kab mob siv rau hauv daim ntawv tsim, kev nce hauv lwm pab pawg tau sau tseg. Qhov ntau thiab tsawg sib txawv, zoo li qhov sib xws: qee qhov zoo li xoob khob noom cookie, thaum kuaj mobtsis txhob teb, tsis txhob sib koom ua ke los yog nrog daim tawv nqaij. Rau cov cheeb tsam xws li, qhov tsim ntawm rwj lossis suppuration yog uncharacteristic.
Thaum tus kab mob siv rau hauv daim ntawv tsim, qhov tshwm sim tshwm sim, tus neeg mob xav tias tsis muaj zog, lub peev xwm ua haujlwm tau qis dua. Tus neeg mob lub hws qog ua haujlwm, nws poob phaus, raug mob kub taub hau. Lymph nodes tau loj zuj zus, uas ua rau muaj kev nyiam thaum kuaj thawj zaug.
Kev kuaj mob ntawm tus neeg mob: kev ua kom pom tseeb
Thaum kuaj tus neeg mob, lymphoproliferative syndrome tuaj yeem xav tias yog lymphadenopathy tau kuaj pom meej. Hauv ntau tus neeg mob, qhov kev hloov pauv ntawm ib tus neeg ntawm daim tawv nqaij yog pom: ib qho infiltrate tshwm sim, kuaj pom qhov txhab tsis raug. Yog hais tias ib tug neeg yav tas los raug kev txom nyem los ntawm cov kab mob ntawm daim tawv nqaij, lawv yuav hnyav heev vim hais tias tus syndrome tau piav qhia. Ntau tus txhawj xeeb txog exfoliative erythroderma. Tawm tsam keeb kwm yav dhau los ntawm tus mob, kev loj hlob ntawm herpes, urticaria, dermatitis yog ua tau.
Txhawm rau kom paub meej qhov mob, nws yuav tsum xa tus neeg mob mus rau CT, ultrasound. Lub lymphoproliferative syndrome yog qhia los ntawm kev loj hlob ntawm cov qog nqaij hlav hauv lub sternum, plab kab noj hniav, thaum tus mob tsis yog ib txwm nrog los ntawm kev tshwm sim ntawm compression. Tus neeg mob tus po thiab daim siab loj dua li qub. Kev tshawb fawb ntawm cov mucous daim nyias nyias ntawm lub plab zom mov tso cai rau koj kom pom cov leukemic infiltration. Lwm qhov tshwm sim yog rwj hauv plab, plab hnyuv, los ntshav hauv cheeb tsam no. Muaj qhov ua tau ntawm malabsorption syndrome.
xwm txheej nce qib
Thaumlymphoproliferative syndrome tuaj yeem koom nrog kev ua pa hauv cov txheej txheem pathological. Leukemic infiltration tuaj yeem cuam tshuam rau ob qho tib si sab sauv thiab sab hauv nruab nrab. Tus neeg mob hnoos, ua tsis taus pa luv yog cuam tshuam, expectoration ntawm hnoos qeev nrog ntshav inclusions yog ua tau. Qee zaum pleurisy tau tsim.
Qee qhov xwm txheej, cov tsos mob tau piav qhia ua rau muaj kev nkag mus ntawm lub raum parenchyma. Cov mob no tsis tshua muaj tshwm sim nws tus kheej raws li ib tug raug symptomatology. Nws muaj peev xwm hais tias qhov infiltrate kis mus rau hauv nruab nrab lub paj hlwb, uas ua rau mob meningitis, qee hom kab mob encephalitis thiab tuag tes tuag taw ntawm cov paj hlwb, thiab tuaj yeem ua rau coma. Thaum cov infiltrate kis mus rau lub cavernous lub cev, tus neeg mob raug kev txom nyem los ntawm ib tug ntev thiab mob-provoking erection, nyob rau hauv cov tshuaj hu ua priapism.
Lab tests
Yog tus neeg mob xav tias muaj tus mob lymphoproliferative, tus neeg mob raug xa mus kuaj ntshav. Cov mob no yog nrog los ntawm kev nce hauv cov concentration ntawm lymphocytes, leukocytes. Ua tau anemia.
Kev kuaj sim pab kuaj ntshav hemato-, proteinuria hauv tus neeg mob. Kev tshuaj xyuas biochemistry qhia meej meej hypogammaglobulinemia. Hauv ib feem me me ntawm cov neeg mob, cov neeg mob muaj hypoalbuminemia. Hepatocyte cytolysis qhia tias hyperenzymemia.
Ib qho kev tshawb fawb txog kev tiv thaiv kab mob qhia tau tias muaj kev nce ntxiv hauv cov poov xab, cov kab mob ntawm cov lymphocytes, kev ua tsis tiav ntawm kev sib npaug ntawm cov pab cuam thiab cov tshuaj tua kab mob los ntawm cov lymphocytes. Nyob rau tib lub sijhawm, qhov concentration ntawm IgG, IgA, IgM txo qis (rau ob qhov kawg, qhov kev hloov pauv tshwj xeeb tshaj tawm). Immunophenotyping - lub hauv pausxaus tias leukemic cell qauv yog CD 5, 19, 20, 23 los ntawm chav kawm ntawm B-lymphocytes. Cov txiaj ntsig ntawm kev tshuaj ntsuam cytogenetic hauv 65% ntawm cov neeg mob qhia tias chromosomal txawv txav.
Yuav ua li cas?
Nrog lymphoproliferative syndrome, tus neeg mob tau pom tias ua raws li txoj cai kho mob tsim los ntawm tus kws kho mob - qhov kev zov me nyuam raug xaiv tus kheej. Tus neeg mob tau sau tshuaj cytotoxic. Qhov no yog qhov tseeb tshwj xeeb yog tias lub xeev ntawm kev noj qab haus huv yog sai sai, lub siab thiab tus po, cov qog ntshav qab zib nce sai. Cytostatics yog indispensable rau leukemic infiltration ntawm CNS fibers, raws li zoo raws li yog hais tias tus txheej txheem cuam tshuam rau lub cev sab nraum lub hematopoietic system. Tus mob qhia nws tus kheej nrog mob hnyav thiab tsis ua haujlwm ntawm lub cev thiab lub cev.
Yog tias tus lej ntawm leukocytes hauv cov hlab ntshav nce zuj zus thiab nrawm, chlorbutin, spirobromine tau qhia. Cov tshuaj tiv thaiv zoo ntawm lub cev tso cai rau koj kom tau txais prospidin, cyclophosphamide. Qee zaum cov kws kho mob pom zoo kom nres ntawm pafencil. Yog tias muaj cov lus qhia tshwj xeeb rau qhov no, polychemotherapy yuav raug sau tseg. Nyob rau hauv lub moj khaum ntawm cov chav kawm zoo li no, cytostatic cov neeg ua haujlwm uas cuam tshuam rau lub cev ntawm ntau txoj hauv kev tau ua ke nrog ib leeg.
Kev Ua Haujlwm thiab Txoj Kev: Yuav pab tus neeg mob li cas?
Nrog kev nce hauv cov ntsiab lus ntawm leukocytes mus rau qib 20010 los ntawm 9 / l, lymphocytapheresis raug pom zoo. Yog tias ib tus neeg cov qog ntshav nce siab thiab muaj zog, cov txheej txheem zoo li no tau kuaj pom hauv tus po, yog tias lymphadenopathy dhau mus rau hauv cov txheej txheem dav dav, kev kho hluav taws xob tau muab tshuaj. Nrog kev loj hlob ntawm tus po,kho los ntawm cov tshuaj thiab hluav taws xob, tus neeg mob tau pom zoo kom splenectomy. Nws yuav tsum dhau mus yog tias lub plawv nres ntawm lub cev no nquag, nrog rau cov kab mob nrog rau qhov mob hnyav splenomegaly, qee hom leukocytosis, leukemia. Splenectomy yog qhov tseem ceeb rau granulocyto-, erythro-, thrombocytopenia, anemia ntawm hom autoimmune, thrombocytopenia, uas tsis tuaj yeem tswj hwm los ntawm glucocorticoids.
Yog tias cov tshuaj hormonal qhia tau hais tias muaj txiaj ntsig zoo hauv thrombocytopenia, yog tias hemolytic anemia raug tsim, thiab mob lymphocytic leukemia yog ib qho precursor rau cov kab mob no, glucocorticoids raug sau ua lub ntsiab ntawm kev kho. Cov tshuaj no pab nrog cov kab mob subleukemic ntev ntawm lymphocytic leukemia, nrog rau kev loj hlob ntawm daim siab, tus po, thiab cov qog ntshav. Glucocorticoids yog siv yog tias tus neeg mob tsis kam noj tshuaj cytotoxic, hluav taws xob tsis tuaj yeem ua tau, lossis tus mob no tiv taus cov kev kho mob zoo li no.
tseem ceeb nuances
Cov tshuaj Hormonal yog qhov tseem ceeb yog tias cov tshuaj cytostatic tau ua rau cytopenia, hemorrhagic syndrome. Lawv tau siv los ua ib feem ntawm kev kho mob polychemotherapy, sib txuas cov chav kawm tseem ceeb thiab prednisone.
Cov kab mob pathological tau piav qhia yog qhov tshwm sim los ntawm kev cuam tshuam ntawm tus kab mob sib kis. Nrog rau qhov kev txhim kho ntawm qhov teeb meem no, tus neeg mob tau qhia txog cov tshuaj tua kab mob. Feem ntau, cov tshuaj uas muaj ntau yam kev ua tau zoo yog siv. Zoo ua pov thawjmacrolides, aminoglycosides. Koj tuaj yeem siv cov tshuaj semi-synthetic los ntawm penicillin series, cephalosporin, immunoglobulin.
YMyeloproliferative Syndrome
Cov kab mob pathological no feem ntau suav nrog hauv kev kawm nrog rau cov lus piav qhia saum toj no. Lo lus yog siv los txheeb xyuas cov kab mob uas ua rau cov hlwb myeloid nquag ua. Qhov ua rau ntawm qhov tshwm sim yog qhov ua haujlwm tsis zoo ntawm cov qia hlwb ntawm lub cev lub luag haujlwm rau kev tsim cov ntshav. Cov kab mob sib xyaw ua ke ntau yam kab mob - leukemia, myelofibrosis, thrombocytosis, polycythemia. Myelodysplastic syndrome kuj tseem raug xa mus rau ntawm no.
