Cov kab mob hauv ntshav muaj tsawg tsawg, tab sis lawv ua rau muaj kev phom sij loj rau tib neeg. Ib tus kab mob no yog Vandelström macroglobulinemia.
nws tus yam ntxwv yog dab tsi?
Tus kab mob no hauv cov tshuaj niaj hnub no tau nkag siab tias yog kab mob hematological tshwm sim, nrog rau kev muaj macroglobulin hauv cov ntshav. Cov protein no yog tsim los ntawm kev ua haujlwm ntawm monoclonal B-lymphoid ntshav cov ntsiab lus (B-lymphocytes).
Nquag, cov hlwb no yog lub luag haujlwm rau kev sib txuas ntawm M-globulin hauv cov ntshav. Thaum cov pob txha ua haujlwm tsis zoo (feem ntau thaum lub sijhawm tsim cov qog nqaij hlav), kev hloov pauv tshwm sim hauv cov amino acid muaj pes tsawg leeg thiab cov noob caj noob ces, uas ua rau cov synthesis ntawm macroglobulin, i.e. Waldenström's macroglobulinemia tshwm sim.
Tus kab mob no tsawg heev, kwv yees li 3 tus neeg rau ib lab tus tib neeg. Nws tshwm sim feem ntau hauv cov txiv neej hnub nyoog tshaj 55 xyoo. Txog thaum muaj hnub nyoog 40 xyoo, cov kab mob no xyaum tsis tshwm sim.
Tus kab mob tshwm sim li cas ntawm cov cellular thiab cov ntaub so ntswg? Yam twgcov qauv raug cuam tshuam ua ntej thiab tshwm sim li cas?
Pathogenesis ntawm tus kab mob
Cov kab mob ntawm tus kab mob no yog ua raws li kev tsim tawm ntawm cov hlwb ntawm cov kab mob qog nqaij hlav ntawm cov ntshav plasma uas muaj peev xwm tsim tau cov chav kawm M immunoglobulins. zais cov kab mob pathogenic macroglobulin. Yog li, Waldenström's macroglobulinemia tshwm sim.
Kev sib sau ntawm cov kab mob pathogenic hauv cov ntshav ua rau kev loj hlob ntawm nws cov viscosity. Yog li ntawd, qhov ceev ntawm cov ntshav ntws los ntawm cov hlab ntsha txo, uas ua rau nws stagnation. Tsis tas li ntawd, plasma coagulation yam tseem ceeb yog inhibited (vim lawv lub hnab ntim thiab inactivation los ntawm macroglobulins). Vim li no, ib txwm muaj thrombosis cuam tshuam, uas thaum kawg ua rau kom ntshav ntau ntxiv.
Pom pom, yog tias koj tshuaj xyuas cov pob txha pob txha nyob rau hauv lub tshuab ntsuas, koj tuaj yeem pom cov pawg ntawm "plasmatized" lymphocytes, ntau tus neeg laus B-lymphocytes thiab secreting inflammatory mediators ntawm mast cells. Tag nrho cov hlwb no qhia tias Waldenström's macroglobulinemia tab tom tshwm sim.
Symptoms
Yuav ua li cas ib tus kws kho mob tuaj yeem xav tias kev loj hlob ntawm tus kab mob no?
Ua ntej ntawm tag nrho cov, cov neeg mob yuav yws txog cov tsos mob uas tsis yog tshwj xeeb - tsis muaj zog, subfebrile kub taub hau ntev, tawm hws, poob phaus yam tsis muaj laj thawj.
Cov tsos mob tseem ceeb raws liuas peb tuaj yeem xav tias Vandelstrom's macroglobulinemia tau tsim, yog nce los ntshav ntawm lub qhov ntswg thiab cov pos hniav. Subcutaneous bruising thiab bruising yog tsawg dua.
Hepatosplenomegaly thiab lymphadenopathy (mob qog nqaij hlav) tuaj yeem txhim kho lub sijhawm. Nws kuj tseem muaj peev xwm txhim kho cov teeb meem ntawm macroglobulinemia.
Yog tias tsis muaj kev kho mob raws sij hawm, muaj kev pheej hmoo ntawm tus neeg mob tsim mob coma thiab txawm tuag (txawm li cas los xij, cov neeg mob feem ntau tuag tsis ntau vim lawv tau tsim Waldenström's macroglobulinemia). Qhov ua rau lawv tuag yog qhov tshwm sim ntawm cov teeb meem uas tau tsim.
Kab mob sib kis
Dab tsi tuaj yeem cuam tshuam tus kab mob no?
Ua ntej ntawm tag nrho cov, cov hlab plawv system koom nrog cov txheej txheem pathological, uas yog, cov hlab ntsha me - capillaries, arterioles thiab venules. Vim tias txo cov ntshav khiav, kev txhim kho ntawm retinopathy, nephropathy tau pom. Lub raum raug cuam tshuam. Hauv qhov no, kev txhim kho ntawm occlusion ntawm glomerular hlab ntsha thiab urolithiasis nrog urate deposition yog yam ntxwv.
Waldenstrom's macroglobulinemia yog tus yam ntxwv los ntawm kev txo qis hauv kev tiv thaiv kab mob thiab ntxiv rau kev kis tus kab mob uas ua rau nyuaj rau kev kawm thiab kev kuaj mob ntawm tus kab mob.
Raws li cov txheej txheem nce ntxiv, inhibition ntawm yuav luag tag nrho cov kab mob hematopoietic thiab kev loj hlob ntawm pancytopenia tau sau tseg. Tus cwj pwm los ntawm kev txhim kho amyloidosis thiab kev puas tsuaj rau cov ntaub ntawvCov kab mob pathological ntawm cov hlab ntsha ntawm daim siab thiab tus po, uas ua rau lawv cov haujlwm tsis zoo lawm.
Diagnosis
Cov ntsuas dab tsi yuav pab kom nkag siab tias macroglobulinemia tau tsim?
Ua ntej tshaj plaws, koj yuav tsum ua tib zoo saib xyuas qhov kev kuaj ntshav dav dav. Cov cim tseem ceeb ntawm tus kab mob yuav yog qhov nce hauv ESR thiab tsim cov "npib txhua" - daig ua ke erythrocytes. Cov mis leukocyte feem ntau qhia tau tias muaj ntau cov lymphocytes thiab cov tsos ntawm cov ntaub ntawv tseem ceeb ntawm leukocytes hauv kev tshuaj ntsuam.
Immunoelectrophoresis yog qhia kom paub meej qhov kev kuaj mob. Tom qab ua qhov kev tshawb fawb no, nws tuaj yeem kuaj pom qhov nce ntawm M class immunoglobulins hauv cov ntshav.
Monoclonal immunoglobulins kuaj pom hauv cov ntshav tom qab ntxiv cov ntawv sau npe.
Ntxiv, tab sis tsis meej, cov tsos mob yog qhov loj ntawm daim siab thiab tus po, kuaj pom amyloid nyob rau hauv lub raum capillaries ntawm biopsy, thiab txo qis hauv plasma clotting yam (tshwj xeeb tshaj yog 8).
Kev kho mob
Nyob rau theem pib ntawm tus kabmob, yog tias tsis muaj kev puas tsuaj loj rau cov kabmob hauv nruab nrog cev, kev kho tshwj xeeb tsis qhia. Feem ntau cov neeg mob no nyob rau hauv lub dispensary soj ntsuam ntawm lub zos hematologist.
Thaum thawj cov tsos mob tshwm sim thiab tau lees paub tias tus kab mob no yog Waldenström's macroglobulinemia, kev kho cytostatic tshwj xeeb tau pib. Rau kev kho mob, cov tshuaj no yog siv,Zoo li Chlorbutin, Cyclophosphamide. Lawv muaj cov nyhuv cytostatic thiab inhibit qhov kev ua ntawm cov kab mob qog nqaij hlav ntawm B-lymphocytes.
Ua ntej tshaj plaws, "Chlorbutin" raug tshuaj, 6 mg ib hnub rau qhov ncauj rau 3-4 lub lis piam. Kev nce koob tshuaj yog fraught nrog kev loj hlob ntawm pob txha pob txha aplasia. Tom qab ua tiav cov chav kawm tseem ceeb ntawm kev kho mob, kev saib xyuas cov tshuaj (2-4 mg) tau muab tshuaj txhua hnub.
Plasmapheresis yog siv los txhim kho cov ntshav rheological. Cov chav kawm ntawm kev kho mob nrog plasmapheresis yog nqa tawm thaum siv cytostatics. Cov txheej txheem qhia tau hais tias tshem tawm txog li 2 litres ntawm cov ntshav nrog nws cov kev hloov pauv nrog tus neeg pub dawb, ib qho tsis ua haujlwm.
Kev Tiv Thaiv
Vim tus kab mob no yog los ntawm noob neej, nws nyuaj rau cuam tshuam nws txoj kev loj hlob, vim nws twb nyob hauv cov noob. Tib txoj kev uas yuav zam tau nws txoj kev loj hlob yog qhov kev kuaj mob raws sij hawm thiab npaj kho kom zoo.
Tus kab mob tuaj yeem cuam tshuam ncaj qha los ntawm kev tswj hwm txoj kev noj qab haus huv, txiav luam yeeb thiab cawv, txwv tsis pub haus luam yeeb thiab ntsim zaub mov.
Vim tus kab mob yog caj ces, qee qhov teeb meem kuj tuaj yeem ua rau nws txoj kev loj hlob - ua haujlwm nrog aniline dyes, pleev xim, kua roj vanish.
Kev kis kab mob tsis tu ncua kuj tseem muaj peev xwm hloov pauv tib neeg genome. Kev kho mob raws sij hawm ntawm cov kab mob no yuav pab tiv thaiv kev hloov pauv ntawm cov noob thiab tiv thaiv kev loj hlobmacroglobulinemia.
Kev tiv thaiv suav nrog kev paub cov neeg mob paub txog tus yam ntxwv ntawm tus kab mob thiab ua rau lawv ua rau lawv ua raws li kev noj qab haus huv.
kab mob prognosis
Dab tsi tos cov neeg mob uas tau tsim Waldenström's macroglobulinemia? Qhov kev cia siab ntawm tus kab mob yog nyob ntawm qhov mob hnyav, cov teeb meem tshwm sim thiab lub sijhawm ntawm kev kho mob pib.
Yog tias tus kab mob nyob rau theem pib ntawm kev loj hlob, ces feem ntau nws muaj peev xwm tiv thaiv nws txoj kev vam meej. Txawm hais tias cov neeg mob no tsis muaj sijhawm rau kev kho kom tiav, kev siv tshuaj kho mob ntawm cytostatics tseem tuaj yeem ua rau lawv lub neej expectancy.
Qhov xwm txheej phem dua rau cov neeg uas muaj teebmeem Waldenström's macroglobulinemia. Cov tsos mob, qhov kev tshwm sim uas yog qhov kev poob siab heev - amyloid kev puas tsuaj rau daim siab thiab ob lub raum, los ntshav tsis tu ncua thiab kev loj hlob ntawm paraproteinemic coma. Yog tias cov neeg mob no tsis kho raws sijhawm, qhov tshwm sim ntawm kev tuag yog siab.
Qhov nruab nrab lub neej expectancy rau cov neeg mob uas muaj tus kab mob no yog hais txog 4-5 xyoo. Nrog rau txoj kev npaj kho mob kom zoo, nws muaj peev xwm nce mus txog 9-12 xyoo.
Kev phom sij
Vim tias tus kab mob no tshwm sim tsis tshua muaj, nws nyuaj heev rau kev xav tias nws txoj kev loj hlob rau tus neeg uas tau xyaum tsis tau ntsib nws. Xav txog tias, ua ntej tshaj plaws, cov kws kho mob hauv nroog tau ntsib nrog tus neeg mob, nws yog nyob ntawm lawv lub xub pwg nyom uas lub luag haujlwm tau muab tso rau kom ntseeg tau tiasWaldenström's macroglobulinemia tau kuaj pom. Tsis yog txhua tus kws kho mob yuav nco ntsoov tias nws yog tus kab mob dab tsi, txawm li cas los xij, ib tus kws kho mob yuav tsum xav tias tus neeg mob muaj cov kab mob tshwj xeeb no, suav nrog tag nrho nws cov kev kho mob tshwm sim.
Yav tom ntej, tus neeg mob no yuav raug tswj hwm los ntawm hematologists, txawm li cas los xij, qhov kev kuaj mob thawj zaug tsuas yog nyob ntawm lub xub pwg ntawm tus kws kho mob polyclinic.
Kev txhais tsis raws sijhawm ntawm tus kab mob no ua rau muaj qhov tshwm sim loj heev thaum cov tshuaj khomob tsis zoo lawm. Yog vim li ntawd koj yuav tsum paub txhua yam txog tus kab mob no kom tsis txhob nco nws thiab tsis txhob coj mus rau lub xeev tsis saib xyuas.
