Primary Amyloidosis Ntawm Cov Kab Mob genitourinary
Mar 15, 2022
Yog xav paub ntxiv:ali.ma@wecistanche.com
Khaleel I. Al-Obaidy, MD; David J. Grignon, MD †
Amyloidosis yog ib pab pawg neeg tsis sib haum xeeb uas tshwm sim los ntawm kev tso tawm ntawm cov misfolded proteins nyob rau hauv cov ntaub so ntswg extracellular.1 Cov misfolded proteins no yog tsim los ntawm cov amino acid peptides sib txawv uas qhia ib tug ntau yam caj qaum ntawm b-daim ntawv conformation. Qhov no ua rau lawv insoluble thiab resistant rau proteolytic kev ua ub no.2,3Raws li cov amino acid muaj pes tsawg leeg, daim ntawv ntawm amyloid deposit sib txawv ntawm qhov hnyav thiab kev koom tes hauv lub cev. Txog rau tam sim no, ntau tshaj 25 proteins tau piav qhiaamyloidogenic, tag nrho cov uas yog indistinguishable los ntawm lub teeb los yog electronmicroscopickev tshuaj xyuas.4,5
Lub etiology ntawm localized amyloidosis tsis paub. Nws tau raug postulated tias recurrent mob mob nrog txuam migration ntawmlymphoplasmacyticcov hlwb tuaj yeem ua rau muaj qhov tsis zoo ntawm monoclonal proliferation thiab hauv zos secretion ntawm lub teeb chains. Cov saw teeb no tuaj yeem sib sau thiab hloov mus ua amyloid los ntawm lysosomal degradation.11Qhov kev tshuaj xyuas no yuav qhia txog cov kev nthuav qhia kho mob thiab cov kev tshawb pom muaj feem cuam tshuam rau thawj amyloidosis hauv txhua lub cev ntawm cov kab mob urinary. Qhov no tseem yuav suav nrog cov ntsiab lus tseem ceeb ntawm cov koom haum txawv txawv raws li peb cov kev paub hauv tsev.
Cistanche UK rau kab mob raum, nyem qhov no kom tau txais cov qauv
DIAGNOSIS OF AMYLOIDOSIS
Radiologically, tsis muaj kev tshawb nrhiav tshwj xeeb los kuajamyloidosis; Txawm li cas los xij, qee cov ntaub ntawv xov xwm xov tooj cua qhia tias amyloidosis yuav tsum xav tias thaum qhov mob pom tau tias yog qhov tsis txaus ntseeg ntawm T2- hnyav cov duab sib nqus resonance thiab tsis muaj qhov pom tseeb tau txais txiaj ntsig, hos lwm tus xav txog qhov muaj calcifications raws li kev suav tomography scan. kos npe ntawm amyloidosis, txawm tias nws tsis yog tshwj xeeb thiab tshwm sim hauv ntau lwm yam mob.12,13
Kev kuaj pom tseeb ntawm amyloidosis yuav tsum tau kuaj histological. Microscopically, amyloidosis deposits nyob rau hauv cov ntaub so ntswg raws li ib tug extracellular liab liab, homogenous, amorphous, thiab acellular khoom. Txawm hais tias nws cov tsos mob tuaj yeem qhia qhov kev kuaj mob, qhov muaj ntawm amyloidosis yuav tsum tau kuaj xyuas los ntawm cov kab mob tshwj xeeb histochemical. Congo liab stain, uas feem ntau renders amyloid deposits ib tug salmon liab xim, yog cov feem ntau siv stain rau nws nrhiav tau. Txawm hais tias tus yam ntxwv zoo li no ntawm Congo liab stain, nws yuav tsum tau tshuaj xyuas nyob rau hauv lub teeb polarized, uas qhia tau hais tias kua-ntsuab birefringent deposits.14 Txhua hom amyloid tuaj yeem kuaj pom los ntawm Congo liab stain; Txawm li cas los xij, hom thib ob (amyloid A) poob qhov affinity rau stain rau Congo liab thaum nws yog pretreated nrog poov tshuaj permanganate.15,16 Amyloid nrhiav tau kuj ua tiav siv immunohistochemical txoj kev. Txawm li cas los xij, qhov no kuj tseem tuaj yeem nthuav tawm cov txiaj ntsig tsis muaj txiaj ntsig ntsig txog cov yam ntxwv ntawm epitopic lossis keeb kwm staining.
Txawm hais tias qhov kev kuaj mob yooj yim ntawm amyloidosis, ntxiv subtyping yog xav tau rau kev kho mob. Qhov no subtyping yog ua tiav los ntawm laser microdissection, tom qab ntawd tsom xam los ntawm cov kua chromatography / mass spectrometry technology. Cov txheej txheem no muaj nws tus nqi hauv kev lees paub qhov kev kuaj mob ntxiv rau subtyping raws li peptide muaj pes tsawg leeg ntawm amyloidogenic protein nrog siab rhiab heev thiab tshwj xeeb.17
PRIMARY AMYLOIDOSIS ntawm lub raum
CovraumNws yog qhov feem ntau cuam tshuam cov kab mob hauv lub genitourinary system. Kev kwv yees ntawm lub raum amyloidosis yog 2 feem pua mus rau 3 feem pua ntawm lub raum biopsies, feem ntau ntawm amyloid lub teeb-chain lossis amyloid A hom.5,18 Thaum cov tsos mob, cov neeg mob muaj qhov sib txawv ntawm cov proteinuria, xws li subnephrotic mus rau proteinuria loj, nrog rau lwm cov yam ntxwv ntawm nephrotic syndrome. Qhov hnyav ntawm cov tsos mob yog nyob ntawm ntau yam, nrog rau kev faib tawm ntawm cov kab mob hauv lub cev. Piv txwv li, amyloid light-chain amyloidosis feem ntau muaj glomerular deposit thiab nthuav tawm nrog proteinuria txog li 75 feem pua ntawm cov neeg mob, hos amyloid A feem ntau qhia nyob rau hauv crescentic glomerulonephritis. Kev yees duab tsis yog tshwj xeeb thiab yuav pom qhov qub lossis lojlub raum.19–23Txog rau tam sim no, ib kis ntawm thawj qhov chaw hauv cheeb tsam (amyloid light-chain) lub raum amyloidosis tau tshaj tawm los ntawm Fuah thiab Lim24 hauv tus txiv neej hnub nyoog 53- tus neeg mob uas muaj proteinuria.
Microscopically, amyloid deposition tuaj yeem ua glomerular, interstitial, tubular, lossis vascular, nrog lossis tsis muaj kev cuam tshuam rau lub raum.25 Nyob rau hauv kev npaj kev faib tawm los ntawm Sxen thiab Sarsık,26Lub raum amyloidoses tau muab faib ua 7 chav kawm histomorphologic (0-VI), suav nrog tsis muaj amyloid deposition, tsawg heev (,10 feem pua) focal lossis segmental deposition hauv mesangium lossis vascular ncej, mesangial tsawg (10 feem pua - 25 feem pua), focal mesangial capillary amyloid deposition (26 feem pua – 50 feem pua), diffuse mesangiocapillary deposition (51 feem pua – 75 feem pua), membranous amyloid deposition, thiab advanced amyloidosis.
Txawm hais tias kev sim ua kom sib koom ua ke ntawm kev faib tawm histomorphologic, kev sib raug zoo ntawm qib thiab cov qauv ntawm lub raum pathologic kev koom tes thiab cov txiaj ntsig kev kho mob tseem tsis tau meej. Maj mam ncemob raum mobpom nyob rau hauv cov neeg mob uas muaj vascular thiab tubular amyloidosis. Txawm li cas los xij, los ntawm qhov deb, glomerular deposit zoo nkaus li yog qhov tseem ceeb tshaj plaws los txiav txim siab qhov txiaj ntsig ntawm kev kho mob, tshwj xeeb tshaj yog thaum proteinuria nrog rau lwm cov yam ntxwv ntawm nephrotic syndrome.25,27 Tsis tas li ntawd, intratubular amyloid nag lossis daus (amyloid cast) tsis ntev los no tau piav qhia tias muaj kev pheej hmoo. qhov tseem ceeb rau lub teeb-chain amyloidosis thiab tej zaum yuav ua raumob raum raug mob.5,22
PRIMARY AMYLOIDOSIS NTAWM LUB URETER
Hauv ureter, amyloidosis tsis tshua muaj thiab feem ntau luam tawm raws li cov ntaub ntawv qhia.28Nws tshwm sim feem ntau hauv cov poj niam, nrog poj niam rau txiv neej piv ntawm 1.9: 1. Lub hnub nyoog nruab nrab ntawm kev kuaj mob yog 58 xyoo (ntau, 17-81 xyoo), thiab feem ntau ntawm cov neeg mob uas muajflank mob, ua raws li hematuria.13 Hauv tsab ntawv ceeb toom los ntawm Ding li al, 28 txog 60 feem pua ntawm cov ureteric kev koom tes tau pom nyob rau hauv qis ureter, ua raws li ntawm sab sauv.
Ib qho kev sib txawv txawv ntawm thaj chaw ureteric amyloidosis thiab nephrogenic adenoma tau sau tseg hauv ib qho ntawm cov kab mob uas tau txheeb xyuas hauv peb lub koom haum. Lub ureteric lumen yog ib ncig ntawm cov khoom siv acellular hyaline, sib xyaw nrog ntau qhov sib txawv me me (PAX-8 zoo) tubules nyob rau hauv keeb kwm yav dhau los ntawm ntau lymphoplasmacytic o. Cov khoom siv acellular hyaline muaj xim liab-liab ntawm Congo liab stain thiab pom cov kua-ntsuab birefringence ntawm polarization (Figures 1, A txog D, thiab 2, A thiab B). Qhov no tau tsim ib qho polypoid loj uas txuas ntxiv thaiv lub ureteral lumen. Nws tsis paub meej tias qhov mob antecedes rau lwm tus, tab sis qhov mob ntev thiab rov tshwm sim tuaj yeem ua rau amyloidosis hauv cheeb tsam nrog rau tom qab ntawm lub raum tubular hlwb thiab kev loj hlob ntawm nephrogenic adenoma, ntxiv dag zog rau ureteric obstruction.
PRIMARY AMYLOIDOSIS ntawm lub zais zis tso zis
Thawj qhov chaw amyloidosis nyob rau hauv lub zais zis feem ntau tshwm sim nyob rau hauv cov txiv neej laus (lub hnub nyoog nruab nrab, 55 xyoo), thiab feem ntau cov neeg mob uas muaj tag nrho cov hematuria, urinary irritation, los yog urinary obstructive tsos mob. ntawm lub zais zis, nyob rau hauv ib qho los yog ntau qhov kev faib tawm.29Lub zais zis tuaj yeem tshwm sim li qub ntawm kev kuaj cystoscopic, tuaj yeem pom ntau qhov chaw edematous lossis tuaj yeem pom qhov mob hnyav. Tsis tas li ntawd, nws tsis yog qhov tsis tshua muaj tshwm sim rau thaj chaw amyloidosis los ua cov txheej txheem neoplastic nrog cov nyhuv loj ua rau malignancy.31–34Cov duab thaij duab kuj tsis yog tshwj xeeb thiab tej zaum yuav pom muaj qhov mob loj, ua kom lub zais zis ntawm phab ntsa, lossis thaj chaw ntawm calcifications.34,35 Qhov kev nthuav qhia no yuav ua rau lub zais zis transurethral resection. Microscopically, amyloidosis pom nyob rau hauv lamina propria raws li ib tug ntau hyalinized, acellular extracellular khoom. Qhov chaw urothelium yog benign thiab tej zaum yuav qhia reactive hloov. Congo liab histochemical stain yuav tsum qhia cov xim liab-liab thiab muab cov kua-ntsuab birefringence ntawm polarization.
Fibromyxoid nephrogenic adenoma yog ib qho mob benign uas yuav tsum tau txiav txim siab nyob rau hauv kev kuaj mob sib txawv ntawm amyloidosis, tshwj xeeb tshaj yog nyob rau hauv lub zais zis. Nws zoo nkaus li compressed tubules thiab spindled hlwb nyob rau hauv ib tug tseem ceeb fifibromyxoid eosinophilic extracellular matrix.36Hauv cov xwm txheej no, PAX-8 immunostain muaj txiaj ntsig los tsim qhov kev kuaj mob.37Plasmacytoid urothelial carcinoma kuj tseem muaj qhov zoo li cystoscopic zoo li amyloidosis. Lub mucosa nyob rau hauv qhov mob no yuav tshwm sim ib txwm los yog edematous yam tsis muaj qhov txhab. Histologically, cov qog hlwb yog discohesive, muaj ib tug plasmacytoid tsos, thiab pom infiltrating mus rau hauv ib tug edematous thiab myxoid stroma. Ib qho kev txuam nrog qib siab urothelial carcinoma feem ntau pom nyob rau hauv cov xwm txheej no.38
Primary amyloidosis ntawm lub zais zis muaj qhov rov ua haujlwm siab, nce mus txog 54 feem pua raws li qhia los ntawm Tirzaman li al,29Txawm hais tias ntau tus neeg mob yuav nyob twj ywm tsis rov muaj dua rau lub sijhawm txuas ntxiv.
PRIMARY AMYLOIDOSIS ntawm Prostate thiab Seminal VESICLES
Thawj amyloidosis ntawm prostate yog qhov tsawg kawg nkaus, nrog tsawg dua 10 tus neeg mob tau tshaj tawm hauv cov ntaub ntawv Askiv. Nws tau pom muaj txog li 1.5 feem pua ntawm cov prostatectomies (n ¼ 4 ntawm 262) raws li qhia los ntawm Lupovitch.39 Hauv cov neeg mob uas muaj cov kab mob hauv lub cev xws li ntau yam myeloma, Wilson thiab al40 tau tshaj tawm tias ntau li 47 feem pua ntawm cov qog nqaij hlav tau koom nrog. amyloidosis. Kev nce ntxiv mus rau kev kuaj mob prostatic yuav tsum cuam tshuam qhov xwm txheej no dhau sijhawm, txawm hais tias tsis muaj cov ntaub ntawv qhia txog xwm txheej tshiab. Hauv kev kuaj mob, cov neeg mob tuaj yeem muaj cov kab mob prostatic nodularity thiab feem ntau muaj cov tshuaj tiv thaiv kab mob prostate tshwj xeeb cov tshuaj tiv thaiv kab mob, feem ntau yuav tshwm sim vim muaj kev mob tshwm sim.41,42Microscopically, amyloid deposition feem ntau pom nyob rau hauv stroma thiab qee zaum nyob ib ncig ntawm cov hlab ntsha.42Feem ntau, amyloidosis tau pom los cuam tshuam rau cov hlab ntsha hauv lub cev. Qhov no sawv cev rau qhov thib ob feem ntau urinary kab mob los koom nrog amyloidosis, tom qab lublub raum. Amyloidosis ntawm cov hlab ntsha thiab ejaculatory ducts yog qhov teeb meem nyob rau hauv feem coob ntawm cov neeg mob, thiab kev kuaj mob yog tsim los ntawm prostatectomy specimens ua rau prostatic adenocarcinoma. Nws tau kwv yees tias cov hlab ntsha thiab cov ejaculatory ducts koom nrog amyloidosis txog li 16 feem pua ntawm cov kab mob autopsy thiab 1.1 feem pua mus rau 4.7 feem pua ntawm cov qog qog nqaij hlav.43–46
![Figure 1. A through C, Ureteric wall with amorphous hyaline material deposited circumferentially underneath the mucosa, along with tubular and cystic proliferation seen within the lamina propria in a background of inflammation. D, Proliferating cysts and tubules further expanded the lamina propria and are positive for PAX-8 immunostain (hematoxylin-eosin, original magnifications 3100 [A and B] and 3200 [C]; original magnification 3200 [D]).](/Content/uploads/2021842169/202112301542126c03748db0ee495a9748b76226930f83.png "Figure 1. A through C, Ureteric wall with amorphous hyaline material deposited circumferentially underneath the mucosa, along with tubular and cystic proliferation seen within the lamina propria in a background of inflammation. D, Proliferating cysts and tubules further expanded the lamina propria and are positive for PAX-8 immunostain (hematoxylin-eosin, original magnifications 3100 [A and B] and 3200 [C]; original magnification 3200 [D]).")
Cov kev tshawb fawb ntawm cov hlwv ntawm cov hlab ntsha tuaj yeem pom cov teeb liab qis ua rau muaj kev cuam tshuam ntawm neoplastic los ntawm adenocarcinoma.47,48Qhov no tuaj yeem cuam tshuam rau kev txiav txim siab kho mob hauv cov neeg mob uas muaj tus mob prostatic adenocarcinoma, raws li nws tuaj yeem txhais tau tias yog prostatic carcinoma txuas ntxiv mus rau hauv cov hlab ntsha hauv lub cev. Microscopically, amyloidosis feem ntau cuam tshuam nrog cov hlab ntsha ntawm ob sab, nyob rau hauv subepithelial faib nrog sib txawv luminal compression thiab nqaim. Kev koom tes ntawm cov hlab ntsha thiab cov leeg nqaij feem ntau tsis tuaj, tej zaum piav qhia txog qhov tsis muaj kev ua haujlwm tsis zoo thiab kev nthuav qhia cov tsos mob.
PRIMARY AMYLOIDOSIS NTAWM KEV KAWM NTAWV THIAB KEV PAB CUAM
Testicular kev koom tes los ntawm amyloidosis yog tsis tshua muaj heev, thiab tag nrho cov ntaub ntawv qhia tau tshwm sim los ntawm lwm yam kev koom tes los ntawm systemic amyloidosis.42,49,50Tsis muaj thawj lub zos amyloidosis ntawm testicular adnexa tau tshaj tawm rau hnub no.
TSEEM CEEB
Amyloidosis ntawm cov kab mob genitourinary yog ib yam kab mob tsis tshua muaj uas tuaj yeem ua rau cov txheej txheem neoplastic ob qho tib si kho mob thiab hluav taws xob. Kev paub ntau dua ntawm qhov chaw no yog xav tau los ntawm cov kws kho mob thiab kws kho mob kom paub tseeb thiab tus neeg mob.
kev tswj hwm.
Cov ntaub ntawv
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