Cov kab mob ntshav ua rau muaj ntau pawg neeg mob uas ua rau ua txhaum ntawm cov ntshav muaj txiaj ntsig thiab muaj pes tsawg leeg. Tag nrho cov ntawm lawv yog systematized raws li kev puas tsuaj rau ib feem ntawm cov ntshav. Cov kab mob hematological tau muab faib ua peb pawg loj:
- kab mob tshwm sim los ntawm kev txo qis hauv qib hemoglobin;
- hemoblastosis;
- hemostasiopathies.
Refractory anemia yog ib hom kab mob ntshav qab zib, uas tshwm sim los ntawm kev ua txhaum ntawm kev loj hlob ntawm cov kab mob liab.
Myelodysplastic syndrome - yog dab tsi?
Ib pawg ntawm cov kab mob thiab cov xwm txheej nrog kev ua txhaum ntawm kev tsim cov erythrocytes, monocytes, platelets, granulocytes hauv cov ntaub so ntswg myeloid (myeloid hematopoiesis) nrog qhov muaj feem ntau ntawm kev tsim cov qog. Cov kab mob ntawm cov kab mob hematopoietic hauv cov tshuaj hu ua myelodysplastic syndrome (MDS).
Cov tsos mob hauv qhov no yog tib yam li hauv kev mob ntshav qab zib, ib qho mob tshwm sim los ntawm kev txo qis ntawm cov neutrophil granulocytes hauv cov ntshav thiab thrombocytopenia. Tus kab mob no muaj ntau dua rau cov neeg muaj hnub nyoog tshaj 60 xyoo, thiab cov txiv neej muaj kev cuam tshuam ntau dua li cov poj niam. Txawm li cas los xij, tsis ntev los no tau muaj "rejuvenation" ntawm tus kab mob. Hematologists ntseeg hais tias yog vim li cas nyob rau hauv lub deterioration ntawm ib puag ncig qhov teeb meem.
Ib qho ntawm cov kab mob ntawm pab pawg ntawm myelodysplastic syndrome yog refractory anemia. Cov kab mob no, zoo li tag nrho lwm cov kev sib xyaw ua ke MDS, hais txog cov kab mob hematological yam ntxwv los ntawm cytopenia (ib qho kev txo qis ntawm qee yam ntawm tes hauv cov ntshav) thiab kev hloov pauv hauv cov pob txha pob txha.
Kev faib tawm ntawm myelodysplastic syndrome
Qee qhov kev hloov pauv tau ua rau kev faib tawm thoob ntiaj teb ntawm cov kab mob, uas tseem cuam tshuam rau cov kab mob hematological. Qhov systematization zoo ib yam li qhov tau hais ua ntej los ntawm WHO, tab sis txawv ntawm FAB ntawm cov kev xaiv rau kev txhais cov kab mob:
- D46.0 - refractory anemia yam tsis muaj sideroblasts.
- D46.1 - RA nrog ringed sideroblasts. Cov qe ntshav tsis paub tab tsis pom hauv cov ntshav.
- D46.2 - refractory anemia nrog ntau dhau blasts 1. Hauv kev kuaj ntshav, cytopenia, ntau dua monocytes, Auer lub cev tsis tuaj.
- D46.3 - refractory anemia (RA) nrog ntau qhov tawg nrog kev hloov pauv.
- D46.4 - RA unspecified.
- D46.5 - refractory cytopenia nrog ntau kabdysplasia.
- D46.6 - myelodysplastic syndrome txuam nrog 5q tshem tawm.
- D46.7 - lwm yam myelodysplastic syndromes.
- D46.9 - myelodysplastic syndrome, tsis qhia meej.
Tus yam ntxwv ntawm refractory anemia
Refractorius hauv Latin txhais tau tias "tsis tau txais", "intractable". Tus kab mob no belongs rau hematological thiab yog yus muaj los ntawm ib tug ua txhaum ntawm kev loj hlob ntawm ib tug erythrocyte kab mob nyob rau hauv cov ntshav uas tsim los ntawm cov pob txha pob txha. Hauv cov tsos mob ntawm refractory anemia, cov ntsiab lus ntawm erythrocytes hauv cov ntshav ua ntej txo qis, thiab tom qab ntawd tag nrho cov hlwb, lub cev ntawm cov kab mob hematopoietic lub luag haujlwm rau hematopoiesis raug cuam tshuam.
Qhov phom sij tseem ceeb ntawm cov kab mob yog tias nws tsis tuaj yeem kho tau raws li kev siv tshuaj muaj hlau. Hom ntshav qab zib no feem ntau yuav luag asymptomatic thiab kuaj pom hauv kev kuaj mob ntawm lwm yam pathologies. Tus kab mob no loj hlob sai heev thiab ua rau muaj mob leukemia.
Refractory anemia nrog ntau dhau blasts
Raws li kev txheeb cais, muaj txog 1,015,000 tus neeg uas muaj tus kabmob zoo sib xws. Tus kabmob no feem ntau tshwm sim hauv cov txiv neej hnub nyoog 75-80 xyoo, thiab cov poj niam hnub nyoog 73-79 xyoo. Pathology muab faib ua ob hom:
- RAIB-1 - tus cwj pwm los ntawm cytopenia, nce ntawm cov monocytes hauv cov ntshav. Qws-zoo li liab protein inclusions nyob rau hauv lub cytoplasm ntawm monocytes (Auer lub cev) tsis kuaj. Pob txha pob txha qhia dysplasia ntawm ib kab ntawm tes, blasts (cov qe ntshav tsis paub qab hau)txog 5-19%.
- RAIB nrog kev hloov pauv - kuaj ntshav qhia pom cov ntsiab lus ntxiv ntawm monocytes, kuaj pom lub cev Auer. Cov ntsiab lus ntawm blasts yog li 30%. Hauv cov pob txha pob txha, dysplasia ntawm ob peb kab ntawm tes tau pom ib zaug. Cov qauv no pab txhawb txoj kev loj hlob ntawm tus mob myeloid leukemia.
Tus neeg tuag los ntawm cov kab mob no yog kwv yees li 11%.
ua rau ntawm pathology
Qhov laj thawj ntawm kev txhim kho tus kabmob tseem tsis tau txheeb pom. Muaj ob hom kev mob, thiab muaj feem cuam tshuam tau tsim rau txhua tus.
Primary (ideopathic) kuaj pom nyob rau hauv yuav luag 90% ntawm cov neeg mob. Yam Tseem Ceeb:
- Smoking.
- Ntau tshaj qhov hluav taws xob.
- Residering in a unfavorable ecological zone.
- Kev nqus pa roj av tsis tu ncua, cov kuab tshuaj organic, tshuaj tua kab.
- Recklinghausen's congenital neurofibromatosis (kev loj hlob ntawm cov qog los ntawm cov hlab ntsha paj hlwb).
- Fanconi hereditary anemia.
- Down Syndrome.
Thib ob hom refractory anemia (MDS) tshwm sim hauv 20% ntawm cov neeg mob, tuaj yeem txhim kho tau txhua lub hnub nyoog. Cov laj thawj tseem ceeb rau kev txhim kho:
- Tshuaj khomob lossis xov tooj cua.
- siv sijhawm ntev ntawm qee pawg tshuaj: cyclophosphates, anthracyclines, topoisomerase inhibitors, podophyllotoxins.
Qhov kev hloov pauv thib ob yog tus yam ntxwv los ntawm kev tiv thaiv kev kho mob siab thiab muaj peev xwm ua rau mob qog noj ntshav thiab, vim li ntawd, qhov kev tshwm sim tsis zoo.
Yuav ua li cas thiaj paub tus kab mob?
Ntau zaus, cov kab mob tshwm sim yam tsis muaj tsos mob, uas ua rau nws nyuaj rau pib kho thaum ntxov thiab hauv cov ntawv me. Raws li txoj cai, kev mob ntshav qab zib hnyav tau kuaj pom thaum lub sijhawm kuaj tus neeg mob niaj hnub.
Tab sis tseem tuaj yeem kuaj pom tus kab mob nyob rau theem pib. Nws thawj cov cim qhia muaj ntau yam nrog rau qhov tshwm sim uas tshwm sim nrog rau daim siab pathologies, kab mob autoimmune thiab lwm yam ntshav qab zib. Cov no suav nrog:
- Pleness, pob tshab ntawm daim tawv nqaij, tshwj xeeb tshaj yog ntawm lub ntsej muag.
- Ntau dhau ntawm cov stratum corneum ntawm epidermis ntawm lub luj tshib thiab hauv caug.
- Kev tsim ntawm hematomas uas siv sijhawm ntev dhau.
- Ntev tawg nyob rau hauv qhov ncauj mucosa.
- Ntxawm nails.
- Hloov cov ntxhiab tsw thiab saj: ntxiv rau cov ntxhiab tsw muaj zog, xav noj lub ntiaj teb, chalk.
- Nyob.
- Ntau SARS nrog kev kawm hnyav.
Yog koj ntsib cov tsos mob no, koj yuav tsum sab laj nrog kws kho mob.
Kev kho mob tshwm sim ntawm mob hnyav
Daim duab kho mob ntawm anemia muaj ob theem: mob hemosuppression thiab hnyav (kawg) theem.
Cov kev tshwm sim hauv qab no tau pom hauv hemodepression:
- Qhov kub nce siab, ua npaws.
- Ntxhais tawg.
- Zoo siab tsis zoo.
- poob phaus.
- Ntau cov kabmob sab hauv: spleen, siab.
- o thiab mob qog nqaij hlav.
Cov xwm txheej zoo li no tau ncua, nrog lub sijhawmkev zam txim.
Cov tsos mob ntawm qhov mob ntshav qab zib hnyav heev (myelodysplastic syndrome) yog tib yam li cov mob leukemia. Tus kab mob no sai heev. Ib qho ntawm nws cov yam ntxwv yog kev tshawb pom ntawm blasts hauv kev kuaj ntshav. Tus kab mob no nrog rau cov tsos mob hauv qab no:
- Hematomas, txawm tias me me.
- Cov pos hniav.
- YGingivitis.
- Binocular displacement ntawm lub qhov muag.
- Kev rov ua dua cov txheej txheem inflammatory hauv nasopharynx.
- Ntsuab khawb, qhov txhab me me.
- Nkauj kho siab.
Hauv cov neeg laus, cov kab mob thib ob tshwm sim, cov kab mob plawv feem ntau cuam tshuam. Cov neeg mob muaj mob thaum taug kev hauv qis qis.
ntsuas ntsuas
Thaum thawj cov tsos mob tsis txaus ntseeg, koj yuav tsum sab laj nrog kws kho mob. Kev kuaj mob ntawm "myelodysplastic syndrome" lossis "refractory anemia nrog cov blasts ntau dhau" tsuas yog ua tom qab dhau los ntawm kev kuaj sim. Lawv suav nrog:
- tshuaj ntsuam xyuas ntawm cov ntshav peripheral.
- Kev tshawb fawb txog kev tshawb fawb ntawm cov pob txha pob txha aspirate.
- Trepanobiopsy yog kev noj cov khoom siv biomaterial los ntawm cov pob txha pob txha los ntawm kev xuas tibia.
- Cytogenetic test. Kev kuaj rau chromosomal abnormalities yog ib qho yuav tsum tau.
Nyob ntawm daim duab kho mob ntawm tus kab mob, kev kuaj mob ntxiv tuaj yeem raug sau:
- Ultrasound ntawm plab.
- Esophagogastroduodenoscopy ntawm lub plab zom mov.
- Kev tshuaj xyuas rau cov qog qog tshwj xeeb.
Txoj Kev Kho Mob
Kev xaiv kev kho mob rau cov ntshav qab zib refractory yog txiav txim siab los ntawm qhov mob hnyav ntawm kev kho mob thiab cov txiaj ntsig ntawm kev sim kuaj. Txoj kev kho mob yog qhov nyuaj heev thiab raug txiav txim los ntawm tus kws kho mob. Cov neeg mob uas muaj ob peb lub blasts muaj kev pheej hmoo tsawg. Lawv tau txais kev hloov pauv thiab kev kho mob thiab tuaj yeem ua lub neej zoo li qub. Cov kev kho mob kwv yees yog raws li hauv qab no:
- Nyob rau hauv qhov chaw nyob ruaj khov, kev soj ntsuam tau ua tiav. Lub luag haujlwm tseem ceeb: txhawm rau tswj hwm qib ib txwm ntawm hemoglobin thiab tiv thaiv kev txhim kho ntawm tus kab mob anemic. Tus neeg mob tau txais cov ntshav liab pub dawb.
- Nyob rau hauv cov ntshav tsis txaus, cov tshuaj tau sau tseg uas pab kho cov protein uas muaj hlau: Exjade, Desferol.
- Txhawm rau tiv thaiv kev tiv thaiv kab mob tiv thaiv kab mob rau cov pob txha, Lenalidomide yog siv nrog antimonocytic globulin thiab Cyclosporine.
- Thaum kuaj pom cov kab mob sib kis, cov tshuaj tua kab mob tau muab tshuaj nyob ntawm tus kab mob thiab nws qhov tsis kam.
- Yog tias tsim nyog, ua kev hloov pob txha pob txha.
Huab cua
Qhov kev tshwm sim ntawm refractory anemia (myelodysplastic syndrome) nyob ntawm daim duab kho mob, lub cev teb rau kev kho mob, thiab lub sijhawm nrhiav kev pab kho mob. Qhov tshwm sim ntawm tus kab mob yog txiav txim siab los ntawm qhov hnyavTsis muaj peev xwm ntawm qee lub hlwb, muaj thiab qhov profile ntawm chromosomal abnormalities, tus naj npawb ntawm blasts thiab lawv nyiam tsim nyob rau hauv cov ntshav thiab cov pob txha pob txha.
Kev Tiv Thaiv
Raws li qhov tseeb tias lub ntsiab lus, qhov xwm txheej thiab cov txheej txheem ntawm kev txhim kho pathology tseem tsis tau tshawb xyuas tag nrho, nws yuav luag tsis tuaj yeem hu ua txoj kev tiv thaiv zoo. Cov kws kho mob qhia kom ua raws li cov lus pom zoo hauv qab no:
- Nyob noj qab nyob zoo.
- Nrog cov zaub mov muaj hlau hauv koj cov zaub mov.
- Nyob sab nraum zoov ntau zaus.
- Muab tus cwj pwm phem, tshwj xeeb yog haus luam yeeb.
- Siv cov cuab yeej tiv thaiv thaum ua haujlwm nrog tshuaj tua kab lossis cov kuab tshuaj organic.
Refractory anemia yog ib yam kab mob txaus ntshai hematological uas hloov mus ua oncology. Txhawm rau kom tsis txhob muaj cov kab mob loj heev thiab qhov tshwm sim loj, nws yog ib qho tsim nyog yuav tsum tau ua qhov kev ntsuam xyuas niaj hnub. Yog tias kuaj pom pathology, nws yog ib qho tsim nyog yuav tsum ua kom tiav cov kev kho mob, rau ntau tus neeg mob qhov no pab tsis tau tsuas yog txhim kho, tab sis kuj ua rau lub neej ntev.
