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Long-Term Outcome of Renal Transplantationin Light-Chain Deposition Disease

Nelson Leung, MD, Donna J. Lager, MD, Morie A. Gertz, MD, Kirk Wilson, Sharan Kanakiriya, MD,and Fernando C. Fervenza, MD

Background: Light-chain deposition disease (LCDD) is a monoclonal gammopathy characterized by nonamyloideposition of light chain in various organs. A small number of kidney transplantations have been performed onCDD patients in whom end-stage renal disease (ESRD) developed. Methods: The authors retrospectively reviewedhe clinical and histologic findings and outcome of 7 patients with LCDD who underwent kidney transplantation atur institution. Results: Renal insufficiency, hypertension, and proteinuria were present in all 7 patients. Proteinuria

evel was greater than 3.5 g/24 h in 3 patients. Three patients had microscopic hematuria. Monoclonal protein wasetected in the serum in 3 patients, urine in 5, and was undetectable in 2. Median age at presentation was 42.7range, 33 to 58) years. The most common renal biopsy findings were mesangial expansion, mesangial nodules,ubular basement membrane thickening, and tubular atrophy. Kappa light chain was detected in all 7 renal biopsyesults. Five patients were on dialysis before transplantation. LCDD recurred after a median of 33.3 (range, 2 to 45)onths in 5 of the 7 patients. One patient remains on dialysis, whereas the other 4 have died. One patient died ofrogression of multiple myeloma 3 months after kidney transplantation without evidence of recurrence. Only 1atient remains recurrence free after 13 years with normal renal allograft function. Conclusion: Although long-termenefits are occasionally seen, renal allograft survival is reduced significantly in LCDD patients. Kidney transplan-ation should not be an option for LCDD patients unless measures have been taken to reduce light chain production.m J Kidney Dis 43:147-153.2004 by the National Kidney Foundation, Inc.

NDEX WORDS: Light-chain deposition disease (LCDD); kidney transplantation; monoclonal gammopathy.

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IGHT-CHAIN deposition disease (LCDD) isa rare manifestation of monoclonal gam-

opathy analogous to primary amyloidosis (AL).1

his renal lesion was first described in patientsith multiple myeloma, but the systemic diseaseas not recognized until 1976.2,3 Like AL, LCDDay be associated with multiple myeloma, al-

hough approximately 50% of the LCDD pa-ients do not exhibit evidence of a neoplasticlasma cell proliferation.4,5 Autopsy and renaliopsy data of patients with monoclonal gam-opathy suggest the rate of occurrence is similar

o AL (8 per million per year), but the truencidence is unknown.6-9 The kidney is the mostommonly affected organ in both diseases oftenesulting in proteinuria and renal insufficiency.2,10

he proteinuria can be severe, reaching ne-hrotic range in about half of the cases. A circu-ating monoclonal protein can usually be de-ected, but up to 15% may have no demonstrableonoclonal light chains in the serum or urine by

mmunoelectrophoresis.1,2,10-12

Despite the similarities, there are distinct dif-erences between LCDD and AL. In contrast toL in which most patients are hypotensive, mostCDD patients are hypertensive.2,11-13 Two thirdsf the light chains in AL are �, whereas � lighthains account for roughly 85% of the cases in

13

CDD. However, it is the ultrastructural char-

merican Journal of Kidney Diseases, Vol 43, No 1 (January), 200

cteristics of the light chain deposits that ulti-ately distinguish LCDD from AL.1 The light

hains in AL form �-pleated sheets that appear asbrils on electron microscopy. This property iselieved to be responsible for the characteristicpple-green birefringence on Congo red stain-ng.14 LCDD, however, is deposited in a fineranular pattern and does not show Congo redirefringence. This difference may contribute tohe individual clinical manifestations and thergan involvement pattern between the 2 dis-ases. By immunofluorescence (IF) examination,lose to 100% of the biopsy results will showharacteristic linear deposition of light chainlong the tubular basement membrane, although

From the Division of Nephrology, Department of Internaledicine; Division of Anatomic Pathology, Department of

aboratory Medicine and Pathology; and Division of Hema-ology, Department of Internal Medicine, Mayo Clinic, Roch-ster, MN.

Received June 30, 2003; accepted in revised form Septem-er 30, 2003.Address reprint requests to Dr Fernando C. Fervenza,

epartment of Internal Medicine, Division of Nephrology,ayo Clinic, Eisenberg S-24, 200 First Street, SW, Roches-

er, MN 55905. E-mail: fervenza.fernando@mayo.edu© 2004 by the National Kidney Foundation, Inc.0272-6386/04/4301-0016$30.00/0

doi:10.1053/j.ajkd.2003.09.020

4: pp 147-153 147

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few cases of IF-negative LCDD have beeneported.1,2,5

The prognosis of patients with LCDD is simi-ar to those with multiple myeloma. In mosteported series, prognosis is poor especially inhose who had renal failure.2,10 The median sur-ival rate of patients with LCDD on dialysis isnly 4 years, which is slightly better than thoseith multiple myeloma or amyloidosis.8 In theast, a small number of patients with multipleyeloma have received renal allografts.15,16 Life

xpectancy was significantly reduced when com-ared with nonmyeloma patients despite rareases of prolonged survival. The results wereiscouraging because many of these patientsied of overwhelming sepsis. With little data onong-term outcome, several transplant centersncluding our own have performed kidney trans-lant in LCDD patients.16-18 We present the clini-al features, the renal biopsy findings, and theong-term outcome of 7 patients with LCDDho had undergone renal transplantation at our

nstitution.

METHODS

From the files of the Division of Anatomic Pathology, allenal biopsy records from 1972 through 1999 were re-iewed. Patients with the diagnosis of LCDD were selectedor this study. Patients with multiple myeloma were includedn this study, but those with AL were excluded. Pertinentlinical and laboratory data and information on the kidneyransplantation, treatment, and follow-up were extractedrom patient records. Outside medical records and deathecords were obtained for patients who received care atutside facilities after transplantation.The diagnosis of LCDD was based on the characteristic

inear light-chain deposition by IF histology and the typicalnely granular deposition on electron microscopy.2 Theame criteria were used to establish recurrence of LCDD inhe allograft. After transplantation, renal allograft biopsyas performed as indicated by a decline in renal function or

n increase in proteinuria. All recurrences were confirmedy allograft biopsy.All renal biopsies were processed for light microscopy

nd immunofluorescence. Electron microscopy was per-ormed on 6 of the 7 native biopsies and 4 of 5 allografts.mmunoperoxidase stains with antibodies against light chainsere performed on paraffin-embedded tissue sections in 2atients who had negative IF results. Serum and urinerotein immunoelectrophoresis was performed using mono-pecific antisera to IgG, IgA, IgM, and � and � light chains.ntisera from several sources were used to enhance anti-enic recognition. Immunofixation electrophoresis was usedhen the results of immunoelectrophoresis were equivocal.lomerular filtration rate (GFR) was assessed by either

nulin clearance, iothalamate clearance, or 24-hour creati- e

ine clearance. Albumin was measured by serum proteinlectrophoresis.

RESULTS

linical and Laboratory Findings atresentation

Fifty-six patients were identified with LCDDs a diagnosis from the pathology database. Ofhose, 8 patients had received a kidney transplantnd were chosen for this study. One was discov-red to have only heavy-chain deposition diseaseith no evidence of light-chain deposition andas excluded. No patient was lost to follow-up.f the remaining 7 patients, there were 5 women

nd 2 men. Their mean age at presentation was6.0 (range, 34 to 58) years. On average, 6range, 0.8 to 9.9) years passed between diagno-is and kidney transplantation. The mean age atransplantation was 52 (range, 40 to 64) years.nly 1 patient who had a plasmacytoma in her

ib developed multiple myeloma. She receivedhemotherapy before renal transplantation. Oneatient had previously donated a kidney to heraughter who had end-stage renal disease (ESRD)rom poststreptococcal glomerulonephritis.

Hypertension, proteinuria, and renal insuffi-iency were common presenting features in ouratients (Table 1). Proteinuria ranged from 0.45o 2.1 g/24 h, (mean, 1.2 g/24 h), and 3 laterrogressed to nephrotic-range proteinuria. Theverage serum creatinine level was 4.4 mg/dL389 �mol/L) ranging from 6.6 to 13.7 mg/dL583 to 1,211 �mol/L). GFR measured in 5atients averaged 24.8 mL/min/1.73 m2 (range, 6o 33 mL/min/1.73 m2). Two patients withoutFR measurement at the time of diagnosis had

erum creatinine level of 2.2 mg/dL (194 �mol/L)nd 7.9 mg/dL (698 �mol/L). Renal functionrogressively declined in everyone. Two patientseceived preemptive kidney transplant 7.0 and.8 years after diagnosis with serum creatinineevels greater than 9.5 mg/dL (840 �mol/L). Fiveatients received hemodialysis before transplan-ation. The median time from diagnosis of LCDDo dialysis was 2.6 years (range, 0.3 to 6.5 years).our were on long-term hemodialysis, and 1 wasialyzed once before kidney transplantation. Theedian hemoglobin level was 9.8 g/dL (98 g/L)ith a range of 6.6 to 12.8 g/dL (66 to 128 g/L).ther common features include hypoalbumin-

mia and microscopic hematuria (Table 1).

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enal Biopsy Findings

The most common glomerular lesions wereesangial expansion and nodules (Table 2). Many

iopsy results also showed varying degrees oflobal sclerosis. Other glomerular findings in-luded mesangiocapillary proliferation (n � 1),brocellular crescents (n � 2), and cellular cres-ents (n � 1). Thickened tubular basement mem-ranes and moderate to severe tubular atrophyere seen in almost all biopsy findings. Tubulitisas present on the biopsy specimen with thebrocellular and cellular crescents. One biopsyesult showed casts that were positive for �, butiant cell reaction associated with cast nephropa-hy was not present. Mild to moderate hyalinerteriolosclerosis was noted on all biopsy results.F stained for � in 5 of 7 biopsies (Table 2). Oneiopsy sample for IF did not contain any glomeru-us. On the 2 biopsy samples that were negativey IF, immunoperoxidase showed � in the tubu-

Table 1. Clinical Characteristics at Presentation inPatients With LCDD

FindingsNo. of Patients

(N � 7) % of Total

enal presentationRenal insufficiency 7 100Proteinuria 7 100Nephrotic proteinuria

(� 3.5 g/d) 3 42.9Acute renal failure 1 12.5

hysical examinationHypertension (�140/

90 mm Hg) 7 100aboratory findingsAnemia (hemoglobin

� 12.0 g/dL) 5 71.4Serum creatinine

� 2 mg/dL 0 02.1 to 3.9 mg/dL 5 71.4� 4.0 mg/dL 2 28.6

Microscopichematuria 3 37.5

Hypoalbuminemia� 3.5 g/dL 5 71.4

ialysisBefore kidney

transplantation 5 71.4After kidney

transplantation 5 71.4

NOTE. To convert hemoglobin and albumin in g/dL to/L, multiply by 10; creatinine in mg/dL to �mol/L, multiplyy 88.4.

ar and glomerular basement membrane in 1 and

ubular basement membrane only in the other.ongo red birefringence was negative in allases. Electron microscopy performed on 6 ofhe 7 biopsy specimens showed granular, electron-ense deposits in the tubular basement mem-rane, glomerular basement membrane, and mes-ngial deposits. Nonamyloid fibrils were foundn 2 biopsy specimens along with the granulareposits.Kappa light chain was seen in the renal biopsy

esult in all 7 patients. Serum protein electro-horesis found � in 3 patients (Table 3). Urinerotein electrophoresis was positive for � in 4.oth � and IgA � were found in the urine of 1atient who had IgA � in the serum. No monoclo-al protein was detected in either serum or urinen 2 patients, but � light chain was seen bymmunofluorescence in their kidney biopsy speci-

ens. Bone marrow biopsy findings were nor-al in 2 of the 7 patients. Four had bone marrow

iopsy results consistent with a plasma cell pro-iferative disorder, and 1 had a plasmacytic my-loma. One patient developed multiple myelomayears after the diagnosis of LCDD but 4 years

rior to her kidney transplant. Three patientsere treated with melphalan and prednisone be-

ore kidney transplant, but only the patient withultiple myeloma was treated posttransplant.

enal Transplantation and Long-Term Outcome

Four patients received cadaveric and 3 re-eived living-related donor kidney transplants

Table 2. Renal Biopsy Findings in PatientsWith LCDD

FindingsNo. of

Patients% ofTotal

ight microscopy (n � 7)Mesangial expansions 4 57.1Well-developed mesangial nodules 5 71.4Moderate-to-severe tubular atrophy 6 85.7Moderate arteriolar hyalinosis 3 42.9

mmunofluorescence (5 patients) andimmunoperoxidase (2 patients)

� 7 100� 0 0

Tubular basement membrane 7 100Glomerular basement membrane 5 71.4Mesangium 3 42.9

lectron microscopy (deposits; n � 6)Mesangial 4 66.6Glomerular basement membrane 4 66.6Tubular basement membrane 5 83.3

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Table 3). Immunosuppressive regimen includedyclosporin A or tacrolimus, mycophenolateofetil or azathioprine, and prednisone. Theost common antirejection regimen was the com-

ination of cyclosporin A, azathioprine, and pred-isone. Early acute cellular rejection developedn 3 patients. The acute rejection was treateduccessfully with Muromonab CD-3 antibodyOKT3, Ortho Biotech) in 1 patient. Anotheratient had mental status changes with OKT3nd was switched to lymphocyte immune globu-in (ALG). Later, a partial allograft nephrectomyas performed in this patient when an acute

hrombosis developed in one of the renal arteries.llograft nephrectomy performed in another with

evere acute rejection and intimal arteriolitishowed recurrence of LCDD. One patient wasound to have acute rejection along with recur-ence of LCDD 34 months posttransplant.

Recurrence of LCDD was discovered in 5 ofhe 7 kidney allografts. Median time after trans-lant was 33.3 months (range, 2.9 to 45.9onths). The median time to reach ESRD after

ecurrence was 10.9 months (range, 0.9 to 15.5onths). Including the 2 patients who died with

unctioning allografts, the overall median allo-raft survival rate was 37.3 months. One patientho lost his allograft because of recurrencenderwent a second cadaveric kidney transplantyears after his first. He died 2 years later as a

esult of complications of an allogeneic bonearrow transplant for myelodysplastic syn-

rome, which was felt to be the result of priorelphalan therapy. Three patients with recur-

ence died after returning to hemodialysis. Onlypatient with recurrence is still alive and has

een on peritoneal dialysis for 5 years.LCDD patient survival appeared worse than

ge-matched kidney transplant recipients with-ut LCDD.19 Five of the 7 patients died afteridney transplantation. Four of these patientsad recurrence of LCDD, and the fifth died as aesult of progression of multiple myeloma 3onths after her kidney transplant. She had no

vidence of recurrence of LCDD at the time ofer death and had a normally functioning allo-raft. The median survival rates after diagnosisnd renal transplant were 12.0 years (range, 3.9o 19.3 years) and 6.1 years (range, 0.3 to 12.8ears), respectively. The median survival rate

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ears). Of the 2 survivors, 1 had early recurrencend graft loss. She had been on peritoneal dialy-is for 5 years and was recently found to have �myloid arthropathy. The other patient is doingell nearly 13 years after kidney transplantation.er monoclonal gammopathy was last checked 4ears after her transplant; the result was stillositive, but she never had any sign of recur-ence of LCDD. Her most recent serum creati-ine level was 1.2 mg/dL (106 �mol/L), and her4-hour urine findings showed 55 mg of protein.nterestingly, she had donated a kidney to heraughter many years before LCDD developed.he had received 41 months of melphalan andrednisone before her transplant but no chemo-herapy posttransplant. She also was 1 of 2atients who had a normal bone marrow biopsyesult. Whether the combination of normal bonearrow biopsy and long duration of treatment

ontributed to her recurrence-free survival can-ot be determined with the limited number ofatients.

DISCUSSION

Much remains unknown about LCDD 3 de-ades after its initial description. The lack of annimal model and its low incidence make study-ng this disease extremely difficult. Renal involve-

ent appears universal because nearly everyatient with LCDD has some degree of renalnsufficiency.1,2,4,5,7,8,10-12,20 On biopsy, LCDDlassically presents with nodular mesangial scle-osis that mimics the lesion of diabetes mellitus,ense deposit disease, and AL.2,10,21 Thickeningf the glomerular basement membrane, mesan-ial expansion, mesangial proliferation, and en-ocapillary proliferation are other common glo-erular findings. Occasionally, crescentic and aembranoproliferative pattern glomerulonephri-

is may be present. Thickening of the tubularasement membrane is the most common tubulo-nterstitial finding, which may be accompaniedy interstitial infiltrates of varying intensity. Tu-ular casts as seen in light-chain cast nephropa-hy may also be seen. Vascular hyalinosis isommon and is seen in more than half of theenal biopsy findings. Deposition of � or � lighthains is almost universally detected by IF histol-gy. The most common staining pattern is aright, linear staining of tubular basement mem-

rane with less intense staining of capillary walls r

nd mesangium.2,5 Light-chain deposits can beound also in the vessel wall and in the intersti-ium. The diagnosis of LCDD is confirmed byegative Congo red birefringence and granularlectron-dense deposits that are located withinhe tubular basement membranes, the glomerularasement membrane, the mesangial nodules, andccasionally in the vessel walls. Nonamyloidbrils have been identified along with the granu-

ar deposits.Advanced renal failure requiring dialysis is

ot an uncommon finding at presentation inCDD. Melphalan and prednisone have beenhown to be capable of temporarily maintainingnd even improving renal function in many pa-ients. However, rapid progression to ESRD canccur even with multidrug regimens.1,2,4,8,10,12

verall survival rate for LCDD is unknown.eported median survival rates can range from8 months to more than 5 years.10,12 Severallinical features have been found to be predictivef poorer outcome in recent studies. These in-lude age, initial serum creatinine, serum cal-ium, types of immunoglobulin deposits, andypes of lesions seen on renal biopsy.4,8 Theseifferences may account for the discrepancy inife expectancy reported in the literature.

With the possibility of prolonging survival, amall number of renal transplants had been per-ormed in LCDD patients with ESRD.16,17,22

avid-Neto et al17 reported a patient who has novidence of recurrence 44 months after receivingn HLA identical kidney from a sibling. On theontrary, Short et al22 recently reviewed this andadditional cases and found the case of David-eto17 to be the only one without recurrence.ecurrence was detected 5 to 50 months post-

ransplant.16,22 Three patients eventually lost theirraft and were started on dialysis, whereas an-ther patient exhibited severe allograft dysfunc-ion with a serum creatinine level of 6.5 mg/dL575 �mol/L). Three patients died of complica-ions of multiple myeloma, 2 with functioningraft. Multiple myeloma developed after renalransplantation in 1 patient. Overall, 4 of the 7ied after renal transplantation, 2 after going onialysis. Disseminated myeloma and sepsis werehe most common cause of death.

Our results mirrored the experience of thether transplant centers.16,17,22 The 71% recur-

ence rate in our patients corresponds to the 86%

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eported by Short et al.22 Median graft survivalate (37 versus 34 months, respectively) is nearlydentical. Recurrence of LCDD in both seriesesulted in either graft loss or death. Every pa-ient with multiple myeloma before transplanta-ion died as a result of progression of his or herisease. Similar mortality rate was noted be-ween our patients (71.4%) and that reported inhe literature (66%). Finally, each series has 1atient with prolonged recurrence-free survival,ut it is the exception rather than the rule.17

The current available data do not supportidney transplantation in LCDD patients. This isspecially true for patients who have concurrentultiple myeloma who have even shorter sur-

ival. For patients who do not have multipleyeloma, the median allograft survival rate is

nly 37.5 months. This increases to 47.9 monthsf deaths with a functioning graft are excluded,ut it is far from the 12.5 to 19.9 years projectedor median allograft survival rate in patientsithout LCDD.19 Because of this, we do not

ecommend kidney transplantation alone in theseatients. We recognize that this decision is basedn limited data and that quality of life waseither assessed nor factored into the decision.owever, we feel that the high recurrence rate

nd graft loss are too prohibitive to offer aidney allograft to all LCDD patients. Kidneyransplantation should be reserved for those pa-ients whose light chain production can be con-rolled and remission sustained. Conventionalhemotherapy may not be effective becauseCDD recurred in 2 of the 3 patients who re-eived low-intensity melphalan and prednisone.hemotherapy after renal transplantation has beenifficult to implement because of complicationf increasing immunosuppression and problemsith pancytopenia. We recommend that reduc-

ion of light-chain production should be accom-lished before commitment for renal transplanta-ion. Dose-intensive chemotherapy followed byeripheral blood stem cell transplantation maye an option. This therapy has been used success-ully in patients with multiple myeloma, AL, andecently LCDD.9,23-25 If a kidney transplant isonsidered, both donors and patients must beade thoroughly aware of the reduced life expect-

ncy of the allograft so that an informed decisionould be made by both parties.

To our knowledge, this represents the largest a

ingle-center experience of renal transplantationn patients with LCDD. It supports the highncidence of recurrence and graft loss seen insolated case reports. The reduced allograft andatient survival rates make renal transplantationn these patients controversial especially in pa-ients without suppression of their light chainroduction. Until effective measures are utilizedo prevent recurrence in the kidney allograft,idney transplantation should be performed pri-arily on those patients who have attained hema-

ologic remission or who will receive aggressiveherapy capable of achieving remission. Studieso facilitate the understanding of recurrence andts prevention are needed before kidney transplan-ation can be offered as a standard treatment forCDD patients who have ESRD.

REFERENCES

1. Ganeval D, Noel LH, Preud’homme JL, Droz D,runfeld JP: Light-chain deposition disease: Its relationith AL-type amyloidosis. Kidney Int 26:1-9, 19842. Confalonieri R, Barbiano di Belgiojoso G, Banfi G, et

l: Light chain nephropathy: Histological and clinical as-ects in 15 cases. Nephrol Dial Transplant 3:150-156, 19883. Randall RE, Williamson WC Jr, Mullinax F, Tung MY,

till WJ: Manifestations of systemic light chain deposition.m J Med 60:293-299, 19764. Lin J, Markowitz GS, Valeri AM, et al: Renal monoclo-

al immunoglobulin deposition disease: The disease spec-rum. J Am Soc Nephrol 12:1482-1492, 2001

5. Strom EH, Fogazzi GB, Banfi G, Pozzi C, MihatschJ: Light chain deposition disease of the kidney: Morpho-

ogical aspects in 24 patients. Virchows Arch 425:271-280,9946. Ivanyi B: Frequency of light chain deposition nephrop-

thy relative to renal amyloidosis and Bence Jones castephropathy in a necropsy study of patients with myeloma.rch Pathol Lab Med 114:986-987, 19907. Comotti C, Mazzon M, Valli A, Rovati C, Vivaldi P:

ight chain deposition nephropathy in multiple myeloma.ontrib Nephrol 105:133-138, 19938. Montseny JJ, Kleinknecht D, Meyrier A, et al: Long-

erm outcome according to renal histological lesions in 118atients with monoclonal gammopathies. Nephrol Dial Trans-lant 13:1438-1445, 19989. Gertz MA, Lacy MQ, Dispenzieri A: Myeloablative

hemotherapy with stem cell rescue for the treatment ofrimary systemic amyloidosis: A status report. Bone Mar-ow Transplant 25:465-470, 2000

10. Pozzi C, Fogazzi GB, Banfi G, et al: Renal diseasend patient survival in light chain deposition disease. Clinephrol 43:281-287, 199511. Buxbaum JN, Chuba JV, Hellman GC, Solomon A,

allo GR: Monoclonal immunoglobulin deposition disease:ight chain and light and heavy chain deposition diseases

nd their relation to light chain amyloidosis. Clinical fea-

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LCDD FOLLOWING KIDNEY TRANSPLANT 153

ures, immunopathology, and molecular analysis. Ann Interned 112:455-464, 199012. Heilman RL, Velosa JA, Holley KE, Offord KP, Kyle

A: Long-term follow-up and response to chemotherapy inatients with light-chain deposition disease. Am J Kidneyis 20:34-41, 199213. Kyle RA, Gertz MA: Primary systemic amyloidosis:

linical and laboratory features in 474 cases. Semin Hema-ol 32:45-59, 1995

14. Bonar L, Cohen AS, Skinner MM: Characterizationf the amyloid fibril as a cross-beta protein. Proc Soc Expiol Med 131:1373-1375, 196915. Dagher F, Sammett D, Abbi R, et al: Renal transplan-

ation in multiple myeloma: Case report and review of theiterature. Transplantation 62:1577-1580, 1996

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