Raws li ICD-10, paraproteinemic hemoblastosis yog cais raws li chav kawm 2 neoplasm (C00-D48), ntu C81-C96. Qhov no suav nrog cov qog nqaij hlav ntawm hematopoietic, lymphoid thiab lwm yam ntaub so ntswg.
Lawv koom nrog pawg ntawm cov kab mob qog ntawm cov hlab ntshav, cov tsos mob tseem ceeb uas yog kev tso tawm ntawm paraproteins lossis / thiab lawv cov khoom tawg. Hauv cov neeg mob sib txawv, cov tshuaj paraproteins tuaj yeem ncav cuag qhov tseem ceeb hauv cov ntshav cov ntshav thiab koom nrog cov chav kawm sib txawv. Lub hauv paus ntawm cov qog loj hlob yog B-lymphocytes.
Paraproteinemic hemoblastoses muaj ntau thoob plaws ntiaj teb. Nrog rau hnub nyoog ntawm tus neeg mob, lawv zaus tsuas yog nce.
Kev faib tawm ntawm paraproteinemic hemoblastoses
Cov hom hemoblastoses raug cais nyob ntawm seb lawv cov immunoglobulins tso tawm, thiab ntawm cov yam ntxwv ntawm cov qog nqaij hlav:
- lg-secreting lymphomas;
- ntau tus mob myeloma;
- hnyav kab mob hnyav;
- mob plasmablastic leukemia;
- YmacroglobulinemiaWaldenström;
- Ysolitary plasmacytoma.
Cov cim qhia tshuaj kho mob
Cov cim qhia ntawm paraproteinemic hemoblastoses yog dab tsi?
Daim duab kho mob yog tus yam ntxwv los ntawm qhov muaj cov qog uas tsim cov tshuaj paraprotein, thiab theem nrab humoral immunodeficiency, uas tshwm sim hauv txhua tus neeg mob thaum muaj qhov nce hauv qog loj. Raws li qhov xwm txheej ntawm tus kab mob, theem yog mob ntev (ntev) thiab mob hnyav (terminal).
Paraproteinemia ua rau pom tshwm sim rau pg:
- Peripheral neuropathy.
- Npaj ntshav viscosity.
- Microcirculatory ntshawv siab.
- YHemorrhagic Syndrome.
- Kidney puas.
- Cryoglobulinemia hom 1-2, amyloidosis.
Qhov no yog kev faib tawm ntau tshaj plaws ntawm paraproteinemic hemoblastoses. Cia peb xav txog txhua yam kom ntxaws ntxiv.
Multiple myeloma
Ntau tus myeloma yog suav tias yog PG ntau tshaj plaws uas tsis paub meej vim li cas rau nws txoj kev loj hlob. Daim duab morphological yog sawv cev los ntawm cov ntshav plasma ntawm qee qhov kev loj hlob, feem ntau nrog cov yam ntxwv ntawm atypism. Cov theem nthuav dav yog tus cwj pwm los ntawm qhov chaw ntawm cov qog hauv cov pob txha pob txha, qee zaum hauv cov qog ntshav, spleen, siab.
Qhov xwm txheej ntawm kev faib cov kab mob hauv cov pob txha tso cai rau peb paub qhov txawv ntawm ntau hom myeloma: diffuse-focal, diffuse thiab ntau-focal.
Pob txha nyob ib ncig ntawm cov qog foci raug rhuav tshem, vim ntau yammyeloma txhawb kev ua haujlwm osteoclast. Cov txheej txheem osteolytic hauv ntau hom muaj cov yam ntxwv. Piv txwv li, daim ntawv diffuse-focal yog tus yam ntxwv ntawm osteoporosis, uas ua rau cov tsos ntawm foci ntawm osteolysis; diffuse - kev soj ntsuam ntawm osteoporosis; ntau-focal - ua tau ib tus neeg foci yog osteolytic. Cov theem nthuav dav ntawm cov qog feem ntau tsis cuam tshuam rau kev puas tsuaj ntawm cortical txheej ntawm cov pob txha. Nws thins nws thiab nqa nws, tsim o ntawm pob txha taub hau, sternum, thiab tav. Lub davhlau ya nyob twg ntawm cov qog yog tus yam ntxwv los ntawm kev tsim los ntawm qhov tsis xws luag thiab germination nyob rau hauv cov ntaub so ntswg ib ncig.
Type of myeloma
Cov chav kawm ntawm cov tshuaj tiv thaiv kab mob zais cia cuam tshuam rau kev sib cais ntawm ntau hom myeloma: A-, D-, G-, E-myeloma, Bence-Jones hom l lossis c, tsis zais.
Myeloma muab faib ua 3 theem raws li kev soj ntsuam ntawm cov ntshav creatinine, hemoglobin, paraproteins hauv cov zis thiab ntshav ntshav, kuaj pob txha.
- Stage 1 - cov qog hnyav dua 600 g/m2.
- 2 theem - 600 txog 1200 g / m 2.
- 3 theem - tshaj 1200 g/m2.
Qhov tsis muaj lossis muaj lub raum tsis ua haujlwm cuam tshuam rau kev ua haujlwm ntawm lub cim theem A lossis B. Cov tsos mob ntawm daim ntawv no ntawm paraproteinemic hemoblastosis yog dab tsi?
Tumor muaj ntau daim duab kho mob. Cov tsos mob ntawm thawj cov tsos mob feem ntau tshwm sim nyob rau theem peb (tsis muaj zog, qaug zog, mob). Qhov tshwm simCov txheej txheem osteodestructive yog kev txhim kho ntawm qhov mob. Qhov mob feem ntau nyob rau hauv swb ntawm sacrum thiab qaum. Feem ntau, cov tav, ntu ntawm lub xub pwg nyom thiab femur pob txha, cuam tshuam los ntawm cov qog, raug mob. Extradural localization ntawm foci yog tus cwj pwm los ntawm qhov tseeb tias compression ntawm tus txha caj qaum ua raws li qhov mob tshwm sim.
Kev kuaj mob yog tsim los ntawm cov ntaub ntawv tau txais tom qab sternal puncture thiab kuaj pom ntawm monoclonal immunoglobulins hauv cov zis thiab / lossis ntshav ntshav. X-ray kuaj ntawm lub cev pob txha yog qhov tseem ceeb ntxiv. Yog tias tus neeg mob muaj ntau daim foos, tom qab ntawd qhov kev sib tsoo ntawm lub sternum yuav tsis pom cov qog.
Tom qab kev kuaj mob ntawm paraproteinemic hemoblastoses (daim duab ntawm cov qog tuaj yeem pom hauv tsab xov xwm) tau tsim, kev tshuaj xyuas fluoroscopic ntawm lub pob txha yog ua ua ntej pib kho, kev ua haujlwm ntawm lub siab thiab lub raum yog kuaj. Kev siv excretory urography thiab lwm yam kev txhoj puab heev ntawm kev tshuaj xyuas lub raum tsis raug tso cai, vim tias lawv tuaj yeem ua rau muaj kev loj hlob ntawm lawv qhov kev ua tsis tau zoo.
Myeloma kho mob
Feem ntau, kev kho mob pib hauv tsev kho mob hematology, thiab tom qab ntawd nws tuaj yeem ua tiav ntawm tus neeg mob sab nraud.
Nyob rau hauv rooj plaub uas tus neeg foci ntawm osteolysis tsim kev hem thawj ntawm kev puas tsuaj ntawm cov kab mob pathological, feem ntau ntawm cov kev txhawb nqa ntawm cov pob txha, yog tias muaj cov qog loj cais ntawm ib qho chaw hauv zos, thawj cov tsos mob ntawm tus txha caj qaum, lub sijhawm postoperative. tom qabdecompressive laminectomy, ces pom zoo siv hluav taws xob kho.
YSolitary plasmacytoma
Lwm yam paraproteinemic hemoblastose yog dab tsi?
Solitary plasmacytoma yog ib qho qog hauv zos. Daim duab kho mob nyob ntawm qhov loj thiab qhov chaw. Feem ntau, ib leeg plasmacytomas yog qhov pib ntawm ntau yam myeloma. Pob txha tib leeg plasmacytoma yog nquag mus rau generalization, kuaj pom ntau yam myeloma 1-25 xyoo tom qab radical kho tau ua.
Extraosseous solitary plasmacytoma tuaj yeem nyob hauv txhua lub hauv nruab nrog cev, tab sis feem ntau yog nyob rau sab sauv ua pa thiab nasopharynx. 40-50% ntawm cov neeg mob raug kev txom nyem los ntawm pob txha metastases.
Kev kuaj mob yog nyob ntawm cov ntaub ntawv ntawm kev kuaj morphological ntawm biopsy lossis puncture khoom. Ntau myeloma yuav tsum tau txiav tawm ua ntej pib kho. Radical phais thiab / lossis kev kho hluav taws xob yog siv rau kev kho mob, nrog kev pab los ntawm 50% ntawm cov neeg mob tau kho tag nrho. Cov neeg mob uas muaj plasmacytoma nyob ib leeg nyob rau hauv kev soj ntsuam rau lub neej vim qhov tseeb tias qhov kev nthuav dav ntawm cov txheej txheem yog ua tau.
Qhov no tsis yog txhua hom paraproteinemic hemoblastoses.
YWaldenström macroglobulinemia
Waldenström's macroglobulinemia yog mob ntev, feem ntau, subleukemic lossis aleukemic lymphocytic leukemia. Localization ntawm lgM-secreting qog tshwm sim nyob rau hauv cov pob txha pob txha. Characterizes nws cov lymphocytic muaj pes tsawg leeg ntawm cov hlwb nrog admixturentshav ntshav. Ntxiv rau monoclonal lgM, Bence-Jones protein yog zais los ntawm cov qog hlwb hauv kwv yees li 60% ntawm cov neeg mob. Waldenström's macroglobulinemia tsis tshua muaj ntau dua li ntau yam myeloma.
Cov kev kho mob tshwm sim feem ntau suav nrog los ntshav thiab hyperviscosity syndrome. Koj tuaj yeem soj ntsuam peripheral neuropathy, theem nrab immunodeficiency, amyloidosis, raum puas. Tsis tshua muaj, lub raum tsis ua haujlwm tshwm sim. Cov theem siab tshaj plaws yog tus cwj pwm los ntawm kev loj hlob ntawm daim siab, spleen thiab / lossis cov qog nqaij hlav, qhov hnyav poob hauv 50% ntawm cov neeg mob. Cov kab mob ntshav qab zib tau tshwm sim lig, leukocytes yuav zoo li qub, cov mis leukocyte tsis hloov pauv, lymphocytosis nrog leukocytosis nruab nrab yog qhov tshwm sim, thiab qee qhov neutropenia tuaj yeem ua tau. Ib qho ESR nce ntxiv yog qhov tshwm sim.
Kev kuaj mob yog tsim los ntawm kev txiav txim siab immunochemical ntawm monoclonal lgM hauv cov ntshav, cov ntaub ntawv trepanobiopsy lossis sternal puncture, tso zis thiab ntshav cov protein electrophoresis. Kev kho mob yog nqa tawm hauv tsev kho mob hematological. Dab tsi ntxiv yog paraproteinemic hemoblastosis?
Heavy chain disease
Cov kab mob hnyav hnyav muaj ntau yam hauv kev kho mob thiab morphological yam ntxwv. Lawv cov yam ntxwv muaj xws li muaj cov protein ntau txawv txav hauv cov zis thiab / lossis ntshav ntshav. A-, g-, m-heavy chain kab mob yog qhov txawv.
A-kab mob feem ntau tshwm sim, feem ntau cuam tshuam rau menyuam yaus thiab cov tub ntxhais hluas hnub nyoog qis dua 30 xyoo. Tus kab mob no tshwm sim nyob rau hauv Middle thiab ze East, nyob rau hauv lub teb chaws ntawm lub Mediterranean phiab. Muaj obCov ntaub ntawv xau: pulmonary thiab plab (pulmonary tsis tshua muaj). Daim duab kho mob yog txiav txim siab los ntawm cov tsos mob ntawm kev tsis haum rau kev nqus, amenorrhea, mob raws plab, baldness, steatorrhea, hypokalemia, qaug zog, hypocalcemia, edema. Mob plab thiab kub taub hau.
Kev piav qhia ntawm g hnyav saw kab mob (Franklin tus kab mob) tshwm sim hauv ob peb lub kaum os cov neeg mob nkaus xwb. Cov ntaub ntawv morphological thiab cov duab kho mob muaj ntau haiv neeg, tsis meej. Feem ntau sau tseg proteinuria, txheeb ze neutropenia, thrombocytopenia, nce ntshav ntshav, ua npaws txawv txav, Waldeyer lub nplhaib nrog o ntawm tus nplaig, mos palate thiab erythema, daim siab loj, lymph nodes thiab spleen. Cov chav kawm ntawm tus kab mob no feem ntau yog sai thiab hnyav. Hauv ob peb lub hlis, kev tuag tau teeb tsa.
Heavy chain disease m yog daim ntawv rarest. Raws li txoj cai, cov neeg laus raug cuam tshuam los ntawm tus kab mob. Tus kab mob manifests nws tus kheej nyob rau hauv daim ntawv ntawm subleukemic los yog aleukemic lymphocytic leukemia, feem ntau cov lymph nodes tsis loj, tab sis tus po thiab / los yog daim siab loj. Qee cov neeg mob muaj amyloidosis thiab osteodestruction. Thiab nyob rau hauv cov pob txha pob txha ntawm yuav luag txhua tus neeg mob, lymphocytic infiltration raug kuaj pom. Ntau cov lymphocytes yog vacuolated, kuj tseem yuav muaj kev sib xyaw ntawm plasma thiab lymphoblasts, plasma cells.
Nws nyuaj heev los kuaj xyuas raws li daim duab kho mob. Nws qhov chaw tsim khoom tshwm sim los ntawm cov txheej txheem immunochemical uas kuaj pom hnyav immunoglobulin a-, g-los yog m-chains. Kev kho mob ntawm paraproteinemic hemoblastoses ntawm hom no tshwm sim hauv tsev kho mob hematological.
lg-secreting lymphoma
lg-secreting lymphoma - qog nqaij hlav uas muaj feem ntau tshaj-medullary localization, feem ntau lawv sib txawv heev (lymphoplasmocytic, lymphocytic), tsis tshua muaj-tawg, uas yog, sarcomas. Qhov sib txawv ntawm lwm yam lymphomas yog tso tawm ntawm monoclonal immunoglobulins, feem ntau ntawm M-chav kawm, me ntsis ntawm G-chav kawm thiab tsis tshua muaj neeg A, suav nrog Bence-Jones protein. Nws raug kuaj xyuas thiab kho raws li cov hauv paus ntsiab lus xws li lymphomas uas tsis tso cov immunoglobulins. Yog tias muaj cov tsos mob tshwm sim vim yog paraproteinemia, ces cov kev tiv thaiv thiab kev kho mob zoo ib yam li Waldenström's macroglobulinemia thiab ntau yam myelomas.
Tom ntej, cia peb saib cov ua rau paraproteinemic hemoblastoses.
Yog vim li cas
Qhov laj thawj tseem ceeb ntawm kev txhim kho pathology:
- Ionizing hluav taws xob.
- tshuaj mutagens.
- Viruses.
- Hereditary factor.
Kev kuaj mob rau paraproteinemic hemoblastoses
Pathology kuaj tau nrog:
- kuaj ntshav kuaj. Hemoglobin, blast cells hauv cov ntshav yuav raug txo, theem ntawm leukocytes, ESR, thiab platelets yuav nce.
- Kev tshawb fawb txog zis.
- Biochemistry ntawm cov ntshav loj rau electrolytes, uric acid ntsiab, creatinine thiab roj cholesterol.
- Kev tshawb nrhiav cov quav.
- X-ray nrogtsom rau cov qog nqaij hlav, uas yuav nthuav dav.
- Ultrasound ntawm lub cev hauv nruab nrog cev.
- ECG.
- Virological diagnostics.
- pob txha pob txha trephine biopsy lossis lumbar puncture.
- Ntshav qog nqaij hlav.
- Studies of cellular pob txha muaj pes tsawg leeg.
- Cytological diagnostics.
- YCoagulograms.
Kev kho mob
Kev kho mob suav nrog kev kho tshuaj khomob, tiv thaiv hluav taws xob thiab tshem tawm cov ntshav ntxiv. Chemotherapy yog hom kev kho mob tseem ceeb rau hemoblastoses. Cov tshuaj tshwj xeeb raug xaiv nyob ntawm tus txheej txheem qog. Cov tiam tshiab ntawm cytostatics xws li "Sarcolysin" lossis "Cyclophosphan" yog siv. Kuj haum yog Vincristine, Prednisolone, Asparaginase thiab Rubomycin. Kev hloov cov pob txha pob txha, uas tau ua thaum muaj kev zam txim, tuaj yeem tshem tau tus kab mob mus tas li.
Kev Tiv Thaiv
Nws yog ib qho tsim nyog thaum lub sij hawm tshem tawm ntawm paraproteinemic hemoblastoses los tiv thaiv exacerbations, ua ntej ntawm tag nrho cov, kom tshem tawm cov txhais tau tias ua rau lawv. Kev tiv thaiv Rh-xws li kev txhaj tshuaj tiv thaiv kab mob - ceev faj txog kev txhaj tshuaj ntshav thaum txhaj cov ntshav Rh-zoo. Exacerbations kuj tiv thaiv nrog kev pab los ntawm ib tug ntev tab sis tsis muaj zog cytostatic nyhuv.
Peb tau tshuaj xyuas lub ntsiab paraproteinemic hemoblastoses.
