Yam tsawg kawg nkaus Hloov Kab Mob Secondary Rau Graft-piv rau-Host Kab Mob Tom Qab Allogeneic Hematopoietic Cell Hloov Rau Myelodysplastic Syndrome

Abstract

Chronic graft-versus-host disease (cGVHD) yog ib qho ua rau cov neeg tsis muaj mob rov qab los ntawm cov neeg tau txais kev hloov pauv hauv cov neeg tau txais kev hloov pauv ntawm cov tshuaj allogeneic hematopoietic cell (HCT). Txawm hais tias tus qauv kev saib xyuas tam sim no yog qhov tseem ceeb hauv kev tshawb nrhiav cGVHD hauv lub ntsws, daim siab, thiab daim tawv nqaij, cGVHD cuam tshuam rau lub raum yog qhov tsis lees paub thiab yuav tsis pom qhov ua rau tom qab HCT lub raum tsis ua haujlwm. Nephrotic syndrome (NS) yog ib qho teeb meem tsawg heev ntawm HCT uas yog postulated los ua glomerular manifestation ntawm cGVHD. Ntawm no, peb tshaj tawm 2 tus neeg mob tom qab-HCT qhov hloov pauv tsawg tshaj plaws yuav kis mus rau cGVHD. Nyob rau hauv ob qho tib si, qhov pib ntawm NS coincided nrog tapering ntawm calcineurin inhibitors, thiab 1 tus neeg mob yav tas los tau kuaj pom muaj cGVHD ntawm lub ntsws. Ib tus neeg mob tau kho nrog corticosteroids ib leeg thiab lwm tus nrog corticosteroids thiab tacrolimus. Ua kom tiav, kev zam txim tau ua tiav hauv ob qho tib si. Peb cov xwm txheej qhia txog qhov cuam tshuam ntawm kev sib koom ua ke ntawm cGVHD thiab tom qab HCT NS rau kev saib xyuas tus neeg mob, suav nrog qhov tseem ceeb ntawm kev tau txais lub raum biopsy los tsim kom muaj kev kuaj mob histopathological thiab qhia txog kev kho mob.

Ntsiab lus

Nephrotic syndrome · Cov kab mob hloov tsawg kawg · Graft-vim-tus kab mob · raum biopsy · Allogeneic hematopoietic cell hloov.

Nyem qhov no mus yuav Cistanche ntxiv

Taw qhia

Txawm hais tias kev nce qib hauv hematopoietic cell transplants (HCTs) tau txhim kho cov txiaj ntsig zoo rau cov neeg mob uas muaj myelodysplastic syndrome (MDS) thiab mob leukemia, HCT tseem muaj kev pheej hmoo loj heev rau cov teeb meem tom qab hloov pauv loj xws li rov tshwm sim, kab mob kis tau zoo, thiab kab mob qog noj ntshav (GVHD. ). Txawm hais tias tus qauv kev saib xyuas tam sim no yog qhov muaj txiaj ntsig zoo hauv kev lees paub qhov tshwm sim ntau dua ntawm GVHD hauv lub ntsws, tawv nqaij, thiab daim siab, kev nthuav qhia atypical cuam tshuam nrog lwm cov kab mob hauv nruab nrog cev ua rau muaj kev sib tw ntau dua. Ib qho piv txwv yog GVHD ntawm lub raum, ib qho tsis tshua muaj thiab tsis to taub qhov tshwm sim ntawm GVHD. Vim tias qhov tsis txaus ntseeg ntawm cov xwm txheej tau tshaj tawm, tag nrho cov kev tshwm sim muaj peev xwm tshwm sim tseem yuav pom, thiab tsis muaj tus qauv tsim kho. Txawm li cas los xij, muaj pov thawj qhia tias mob GVHD (cGVHD) tuaj yeem yog qhov tsis txaus ntseeg ua rau tom qab HCT lub raum tsis ua haujlwm.

Cov npe ntawm cov laj thawj ua rau lub raum tsis ua haujlwm nyob rau hauv cov neeg tau txais HCT ntev thiab suav nrog hypovolemia, sepsis, qog lysis syndrome, tshuaj tiv thaiv nephrotoxicity, thiab GVHD. Ib qho tshwj xeeb tsis tshua muaj tab sis muaj peev xwm ua rau tuag taus ntawm lub raum tsis ua haujlwm hauv HCT cov neeg tau txais yog nephrotic syndrome (NS). Ntawm cov neeg mob tom qab HCT NS, membranous nephropathy (MN) thiab cov kab mob hloov pauv tsawg (MCD) yog nyob deb ntawm cov feem ntau pom cov kab mob histopathological; Txawm li cas los xij, cov ntaub ntawv qhia txog cov kab mob membranoproliferative glomerulonephritis, chav kawm III lupus nephritis, focal segmental glomerulosclerosis, thiab IgA nephropathy kuj tau luam tawm [1-4]. Lub cev loj hlob ntawm cov pov thawj qhia tau hais tias ntau qhov xwm txheej ntawm post-HCT NS tuaj yeem sawv cev rau glomerular manifestation ntawm cGVHD. Qhov kev lees paub no tau txais kev txhawb nqa los ntawm kev soj ntsuam tias ntau dua 87 feem pua ​​​​ntawm cov neeg mob tom qab HCT NS tshwm sim hauv cov neeg mob cGVHD, nrog rau kev sib raug zoo ntawm lub cev ntawm lub cev ntawm cov tshuaj tiv thaiv kab mob thiab qhov pib ntawm NS [5, 6]. Cov pov thawj ntxiv tau tshawb pom los ntawm kev tshawb fawb txog kev tshawb fawb hauv murine qauv ntawm cGVHD uas qhia txog kev hloov pauv membranous, kev puas tsuaj ncaj qha, thiab kev tiv thaiv kab mob tsis zoo [7–9].

Cov txheej txheem uas cGVHD ua rau kev txhim kho tom qab HCT NS tsis to taub, thiab tag nrho cov peev xwm kho mob tshwm sim tseem yuav pom. Vim tias qhov tsis txaus ntawm cov xwm txheej tau tshaj tawm, tsis muaj tus qauv tsim kev saib xyuas rau cov neeg tau txais HCT uas muaj cov tsos mob thiab cov tsos mob ntawm NS. Ntawm no, peb nthuav tawm 2 qhov xwm txheej ntawm post-HCT MCD zoo li qhov thib ob rau cGVHD ntawm lub raum.

Cistanche tubulosa

Case Report/Case Presentation

Case 1

Ib tug txiv neej muaj hnub nyoog 64- xyoo uas muaj keeb kwm ntawm T-cell loj granular lymphocyte disorder thiab gout raug xa mus rau lub tsev kho mob rau cov mob leukopenia thiab rov tshwm sim granuloma annulare nodules ntawm lub cev, caj npab, thiab lub ntsej muag. Cov pob txha pob txha biopsy tau qhia me me erythroid thiab megakaryocytic dysplasia nrog qhov nce siab me ntsis (5 feem pua) qhia ntawm MDS nrog qhov kev pheej hmoo nruab nrab ntawm IPSS-R tus qhab nia ntawm 3.5. Tus neeg mob ua tiav peb lub voj voog ntawm azacitidine yam tsis muaj kev cuam tshuam loj, thiab nws tom qab ntawd txo qis kev siv allogeneic HCT los ntawm kev sib tw, tsis cuam tshuam, poj niam pub dawb nrog me me ABO incompatibility (O ntxiv pub rau A ntxiv tus neeg txais). Nws tau txais tus qauv posttransplant GVDH prophylaxis nrog tacrolimus thiab methotrexate.

Xya caum-peb hnub tom qab hloov pauv, tus neeg mob tau nthuav tawm nrog 1-lub lim tiam keeb kwm ntawm kev qaug zog, qaug zog, ua tsis taus pa, mob plab, thiab mob caj dab tsis tshwj xeeb, feem ntau tseem ceeb hauv lub hauv caug thiab pob taws. Tsis ntev tom qab ntawd, nws tau ntsib kev mob tshwm sim ntawm kev hloov pauv ntawm lub hlwb thiab aphasia, ua rau CT thiab MRI ntawm lub hlwb, uas tsis pom muaj pov thawj ntawm mob intracranial pathology. Vim muaj kev txhawj xeeb rau tus mob encephalitis, tus neeg mob tau kho empirically nrog cefepime, azithromycin, acyclovir, thiab ampicillin. Peb hnub tom qab, nws tsim mob hypoxic ua pa tsis ua hauj lwm uas, xav txog nws lub xeev immunocompromised, tsa kev txhawj xeeb rau atypical pneumonia; Txawm li cas los xij, cov kab mob kis kab mob tsis zoo. Thaum kawg, lub ntsws biopsy tau qhia tawm qhov kev raug mob alveolar uas muaj xws li idiopathic pulmonary syndrome raws li cGVHD. Nws tau kho nrog etanercept thiab koob tshuaj steroids thiab khaws cia ntawm tacrolimus txog hnub 211 tom qab hloov pauv.

244 hnub tom qab hloov pauv, tus neeg mob tau nthuav tawm mus rau lub chaw kho mob xwm txheej ceev nrog rau 1-lub lim tiam keeb kwm ntawm kev ua tsis taus pa, ua daus no, tsis muaj zog, hnoos, mob taub hau, thiab qaug zog. Cov ntshav pib ua haujlwm tseem ceeb rau cov ntshav qab zib ntau (hemoglobin 4.1 g / dL), reticulocyte suav ntawm 10 feem pua, tag nrho cov ntshav bilirubin ntawm 3.3 g / dL, thiab nce ntshav creatinine ntawm 2.0 mg / dL, nrog rau spherocytosis ntawm peripheral ntshav smear. Kev tshawb fawb autoagglutination thiab antiglobulin tau ua tiav vim kev txhawj xeeb rau hemolysis, thiab cov txiaj ntsig tau zoo ib yam nrog mob khaub thuas agglutinin syndrome. Tus neeg mob tau teb zoo rau kev kho mob nrog corticosteroids, IVIG, thiab kev hloov pauv ntawm 2 units ntawm cov qe ntshav liab, thiab los ntawm hnub nws tawm, nws cov hemoglobin tau ruaj khov ntawm 7.0 g / dL. Tom qab tso tawm, nws ua tiav qhov ncauj prednisone taper thiab tau txais tag nrho plaub koob tshuaj rituximab.

280 hnub tom qab hloov pauv, tus neeg mob rov hais dua rau lub chaw kho mob xwm txheej ceev nrog kub siab, qaug zog, thiab peripheral edema uas tau pib los ntawm nws txoj kev kho glucocorticoid, tab sis sai sai mus rau frank NS nrog anasarca, frothy tso zis, mob ntshav siab, thiab ib tug {{1} }lb qhov hnyav. Cov kev tshawb fawb hauv cov ntshav qhia pom creatinine ntawm 3.3 mg / dL, ntshav urea nitrogen ntawm 86 mg / dL, thiab ntshav albumin ntawm 2.2 g / dL. Cov kev tshawb fawb tso zis tau pom tias muaj proteinuria loj heev nrog rau cov zis tso zis protein-rau-creatinine piv ntawm 26.13 g / g, qis hyaline casts, thiab lub ntiajteb txawj nqus ntawm 1.017. Lub raum ultrasound pom diffuse zais zis phab ntsa thickening, nce echogenicity yam tsis muaj hydronephrosis, thiab me me o prostate. Lub raum biopsy tau ua tiav (Fig. 1), thiab electron microscopy qhia qhov ua tau zoo ntawm cov txheej txheem podocyte ko taw uas tsis muaj kab mob txawv txav hauv cov tubules ze ze thiab peritubular capillaries. Lub teeb microscopy pom cov glomeruli zoo li qub thiab tsis muaj pov thawj ntawm kev tiv thaiv kab mob hauv cov kab mob immunofluorescence staining, ua rau kuaj pom MCD.

Tus neeg mob tau kho nrog diuresis thiab qhov ncauj prednisone 1 mg / kg thiab rov pib dua ntawm tacrolimus nrog kev txhim kho hauv nws qhov edema. Prednisone tau nrawm dua 3 lub hlis, raws li tus neeg mob nyiam, thiab tacrolimus tau txuas ntxiv mus rau tag nrho 9 lub hlis. Tsis pub dhau 3 lub hlis ntawm kev pib kho, cov zis protein-rau-creatinine piv tau txo qis rau<1 g/g. Kidney function continued to improve over the subsequent 6 months, reaching a new serum creatinine baseline of 1.6–1.9 mg/dL (estimated glomerular filtration rate 36–44 mL/ min/1.73 m2), consistent with chronic kidney disease stage III. Since the last office visit on day 680 posttransplant, the patient has experienced no further complications.

Cistanche ntsiav tshuaj

Case 2

Ib tug poj niam muaj hnub nyoog 65- xyoo uas muaj keeb kwm ntawm kev kub siab thiab lub plawv dhia tau raug xa mus rau lub tsev kho mob rau kev soj ntsuam ntawm qhov ua tau MDS pom ntawm cov pob txha pob txha uas tau ua los ntawm kev ua haujlwm rau ntshav ntshav. Kev tshuaj xyuas ntawm biopsy tau nthuav tawm qhov feem pua ​​​​mob ntau ntxiv (12 feem pua) zoo ib yam nrog MDS subtype EB-2 nrog rau qhov kev pheej hmoo nruab nrab ntawm IPSS-R tus qhab nia ntawm 4. Tus neeg mob tau zam 6 lub voj voog ntawm azacitidine yam tsis muaj kev mob tshwm sim loj thiab tom qab ntawd ua tiav. txo-siv txias txias allogeneic HCT los ntawm kev sib tw, tsis sib xws, ABO-tshaj pub dawb (RA-/D ntxiv rau A ntxiv). Nws tau txais cov qauv tom qab hloov GVHD prophylaxis nrog cyclosporine thiab methotrexate. Nws chav tsev kho mob nyuaj los ntawm kev kuaj mob atrial fibrillation tshiab uas tau daws nrog metoprolol, thiab nws raug tso tawm ntawm qhov tsim nyog prophylaxis. Lub cyclosporine taper tau pib rau hnub 135 posttransplant.

268 hnub tom qab hloov pauv, tus neeg mob tau nthuav tawm mus rau tsev kho mob nrog oliguria thiab anasarca. Thaum lub sij hawm nkag, nws cov cyclosporine tau txo qis mus rau 25 mg txhua hnub, thiab nws tau tiv thaiv kev tiv thaiv nrog TMP-SMX 160 mg 3 zaug hauv ib lub lis piam. Kev soj ntsuam ntawm kev nkag mus yog qhov tseem ceeb rau kev nce ntshav creatinine (6.0 mg / dL los ntawm lub hauv paus ntawm 1.0 mg / dL) thiab cov proteinuria loj heev (pob zis protein-rau-creatinine piv 13.65 g / g). Ib lub raum biopsy tau ua tiav (Fig. 2), thiab electron microscopy pom qhov ua tau zoo ntawm cov txheej txheem podocyte ko taw nrog qhov tshwm sim ntawm lub ntsej muag ntawm lub teeb microscopy thiab tsis muaj qhov sib ntxiv lossis immunoglobulin deposits ntawm immunofluorescence, raws li MCD.

Tus neeg mob tau txais hemodialysis (HD) los ntawm ib ntus HD catheter thiab tau pib ntawm tacrolimus 0.5 mg ob zaug ib hnub thiab methylprednisolone 1 g / kg / hnub. Methylprednisolone tau txuas ntxiv rau 11 hnub, thiab tacrolimus raug txiav tawm tom qab 7 hnub vim muaj kev txhawj xeeb txog kev tsis zoo rau lub raum rov qab rau hauv qhov chaw mob hnyav vim yog siv tshuaj vasoconstriction. Lub tunneled dialysis catheter tau muab tso rau hauv kev cia siab ntawm HD ntev ntev. Nws tau tawm hauv tsev thaum kawg ntawm prednisone 60 mg txhua hnub, nrog rau txoj kev npaj mus rau HD 3 zaug hauv ib lub lis piam. Hauv 1 lub lis piam ntawm kev tso tawm, tus neeg mob cov zis tso zis thiab cov kua dej tshem tawm tau zoo dua, thiab kev lim ntshav tau txiav tawm tom qab tag nrho 6 zaug. Ib qho tag nrho prednisone taper tau ua tiav los ntawm 4 lub hlis tom qab tso tawm, nrog kev daws teeb meem ntawm nws cov nephrotic-ntau proteinuria. Kwv yees li, 570 hnub tom qab hloov pauv, nws tsis tas yuav HD ntxiv, thiab nws cov ntshav creatinine tam sim no ruaj khov ntawm 1.1 mg / dL.

Herba Cistanche

Kev sib tham

Kev sib raug zoo ntawm cGVHD thiab NS muaj qhov cuam tshuam tseem ceeb rau kev tswj hwm tom qab HCT NS. Tam sim no qhov tseem ceeb ntawm kev kho mob rau MCD yog glucocorticoid monotherapy, uas ua rau kev tso tawm tiav hauv 80-95 feem pua ​​​​ntawm cov neeg mob MCD hauv cov neeg laus [10]. Hauv qhov sib piv, post-HCT MCD feem ntau yog glucocorticoid-resistant, nrog cov lus teb ntawm kwv yees li 22 feem pua ​​[9]. Cov neeg mob uas tseem tshuav xav tau kev kho ua ke nrog calcineurin inhibitors, mycophenolate mofetil, lossis rituximab [11–15]. Ib yam li ntawd, tom qab HCT MN nyiam ua rau cov tshuaj tiv thaiv glucocorticoids, nrog cov lus teb piv rau cov pom hauv idiopathic MGN, kwv yees li 11 feem pua ​​[9]. Thaum cov kev kho mob rau MCD thiab MN hauv cov neeg tau txais HCT zoo sib xws, muaj qhov sib txawv tseem ceeb hauv kev kwv yees ntawm 2 qhov chaw. Hauv kev tshuaj xyuas zoo ntawm 116 tus neeg mob kho tom qab HCT NS, cov neeg mob MCD tau zoo nyob ntawm qhov nruab nrab ntawm 1.75 lub hlis, thaum lub sijhawm nruab nrab ntawm kev rov zoo rau cov neeg mob MN yog 7 lub hlis. Tag nrho cov txiaj ntsig kev kho mob kuj sib txawv, nrog kev tso tawm tiav tiav hauv 81.3 feem pua ​​​​ntawm cov neeg mob MCD thiab 59.1 feem pua ​​​​ntawm cov neeg mob MN [9].

Xav txog cov kev sib txawv no hauv kev kho mob thiab kev kwv yees, lub raum biopsy yog qhov cuab yeej rau kev tswj hwm ntawm HCT cov neeg tau txais txiaj ntsig uas muaj nephrotic-range proteinuria. Txawm li cas los xij, cov neeg tau txais HCT feem ntau yog kev kho mob nyuaj, thiab lub raum biopsy yuav ua tsis tau zoo hauv cov neeg no vim muaj kev pheej hmoo siab ntawm cov teeb meem xws li los ntshav lossis kab mob. Lub raum biopsy yuav tsum tau txiav txim siab rau txhua tus neeg mob uas muaj kev txhawj xeeb rau tom qab HCT NS thiab yuav tsum tau ua kom zoo ua ntej pib siv tshuaj tiv thaiv kab mob thaum twg los tau. Hauv cov neeg mob uas muaj qhov pom tseeb contraindication rau kev kuaj ntshav biopsy, yuav tsum tau txiav txim siab txog qhov kev sim ntawm corticosteroids. Txawm li cas los xij, vim tias qhov muaj feem ntau ntawm cov kab mob corticosteroid-resistant, cov kws kho mob yuav tsum muaj qhov pib qis rau kev pib cov tshuaj tiv thaiv kab mob ntxiv. Beyar-Katz et al. [9] tau thov kev kho mob empiric nrog corticosteroids, kev soj ntsuam ze, thiab rov ntsuam xyuas cov proteinuria ntawm 12-16 lub lis piam los txiav txim qhov tsim nyog ntawm cov tshuaj tiv thaiv kab mob ntxiv, txawm hais tias tsis tau muaj kev tshawb fawb tshawb xyuas qhov ua tau zoo ntawm txoj hauv kev no. Tsis tas li ntawd, txawm hais tias lub luag haujlwm ntawm kev tiv thaiv kab mob mus ntev hauv cov khoom siv hauv lub raum tsis txaus, muaj cov ntaub ntawv tsis txaus hauv cov ntaub ntawv piav qhia txog qhov zoo tshaj plaws prophylaxis rau GVHD ntawm lub raum lossis cov txheej txheem zoo rau kev kho mob mus sij hawm ntev ntawm cGVHD ntawm lub raum.

Cistanche capsules

Ntawm no, peb tau piav txog 2 qhov xwm txheej ntawm post-HCT MCD nyiam los ntawm cGVHD ntawm lub raum. Nyob rau hauv ob qho tib si, qhov pib ntawm NS coincided nrog lub tapering ntawm calcineurin inhibitors, tsa kev xav tsis thoob rau GVHD raws li hauv paus ntsiab lus. Biopsy tau txais sai sai tom qab pib cov tsos mob, tso cai rau kev kuaj mob raws sijhawm ntawm MCD. Ob tus neeg mob tau pib ntawm corticosteroids thiab tacrolimus, txawm hais tias tacrolimus raug txiav tawm tom qab ib lub lis piam hauv tus neeg mob 2, thiab ob tus neeg mob tau txais txiaj ntsig zoo nrog kev tso tawm tom qab kev kho mob. Cov xwm txheej no qhia txog qhov tseem ceeb ntawm kev sib txuas ntawm cGVHD thiab NS, thiab nws qhov cuam tshuam rau kev saib xyuas neeg mob. Cov kws kho mob yuav tsum paub tias cGVHD muaj nyob rau hauv feem ntau cov neeg mob tom qab HCT NS, feem coob ntawm cov neeg yuav xav tau kev tiv thaiv kab mob ntxiv dua li glucocorticoid monotherapy. Tsis tas li ntawd, lub raum biopsy yuav tsum tsis txhob ncua sij hawm tshwj tsis yog tias tsim nyog, vim nws yog qhov tseem ceeb rau kev ua kom muaj kev kuaj mob kom raug, kev kho mob tsim nyog, thiab tau txais txiaj ntsig zoo dua.

Cov ntaub ntawv 

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Omar Elghawy 1, John S. Wang 1, Alexander C. Hafey 1, Amand 1, D. Renaghan 2 Rachel M. Whitehair 3, Tamila L. Kindwall-Keller 4,

1 Tsev Kawm Ntawv Tshuaj, University of Virginia, Charlottesville, VA, USA;

2 Division of Nephrology, University of Virginia, Charlottesville, VA, USA;

3 Department of Pathology, University of Virginia, Charlottesville, VA, USA;

4 Division of Hematology and Oncology, University of Virginia, Charlottesville, VA, USA