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    "textoCompleto" => "INTRODUCTION <br></br><br></br>B-cell proliferative diseases are usually associated to production and secretion into blood of a monoclonal immunoglobulin&#44; or a fragment of it&#44; that may be deposited in the organs in an organized form as crystals&#44; fibrils or microtubules&#44; or in a non-organized&#160; form&#160; &#40;granular&#41;&#46;&#160; This&#160; immunoglobulin&#160; will mainly be deposited in the kidney&#44; not only because this is a highly vascularized organ&#44; but also because&#160; the&#160; renal&#160; tubule has&#160; a&#160; predominant&#160; role&#160; in&#160; immunoglobulin&#160; metabolism&#46;1 Diagnosis of renal involvement due to immunoglobulin deposition is being expanded with development and routine implementation&#160; of&#160; different&#160; laboratory&#160; procedures&#160; &#40;staining&#160; with <br></br>antibodies specific to kappa and lambda light chains&#44; electron microscopy study&#44; development of procedures with an increasing&#160; sensitivity&#160; for&#160; detecting&#160; the monoclonal&#160; component&#160; in blood&#160; or&#160; urine&#41;&#46;2 Light&#160; chain&#160; deposition&#160; disease&#160; &#40;LCDD&#41;&#160; is characterized&#160; by&#160; generalized&#160; deposition&#160; of&#160; a&#160; single&#160; type&#160; of light&#160; chain&#160; along&#160; the&#160; renal&#160; basement&#160; membrane&#46;&#160; LCDD&#160; is usually&#160; reported&#160; to&#160; occur&#160; during&#160; plasma&#160; cell&#160; dyscrasia&#160; or another&#160; lymphoproliferative disorder&#44; but may&#160; also occur&#160; in the absence of any hematological disorder&#44; in which case it is <br></br>called idiopathic LCDD&#46; Severe renal insufficiency occurs in most patients despite&#160; treatment&#46;3&#44;4 The most&#160; typical&#160; renal&#160; lesion is nodular glomerulosclerosis&#44; in which mesangial nodules and deposition of a single chain type occur along the glomerular&#160; basement&#160; membrane&#46;&#160; However&#44;&#160; diagnosis&#160; will&#160; be based&#160; on&#160; light&#160; chain&#160; deposition&#160; along&#160; the&#160; tubular&#160; basement membrane&#46; The aim of this study was to review our experience with this uncommon disease&#46; <br></br><br></br>MATERIALS AND METHODS <br></br><br></br>Cases&#160; of&#160; LCDD&#160; diagnosed&#160; at&#160; our&#160; hospital&#160; among&#160; all&#160; adult biopsies performed&#160; in&#160; the 1978-2005 period were&#160; analyzed&#46; Clinical and pathological data and patient course were studied based on clinical records and on the information provided by physicians with whom the patient was in direct contact at the time of study closure&#46; All renal biopsies taken during the abovementioned&#160; period were&#160; read&#160; by&#160; the&#160; same&#160; pathologist&#46; All samples&#160; diagnosed&#160; as&#160; LCDD&#160; had&#160; been&#160; processed&#160; for&#160; study with&#160; light microscopy&#160; &#40;LM&#41;&#160; and&#160; immunofluorescence&#160; &#40;IF&#41;&#46; <br></br>Samples from five patients were also analyzed using electron microscopy&#160; &#40;EM&#41;&#46; Diagnosis&#160; of LCDD was made&#160; by&#160; an&#160; IF study&#44; in which sera against kappa and lambda light chains are used&#46; This procedure has been routinely performed at our hospital since 2002&#46; The presence of nodular glomerulosclerosis&#44; chain&#160; deposit&#160; distribution&#44;&#160; thickening&#160; of&#160; basement membranes&#44; extent of tubulointerstitial involvement&#44; and the presence of&#160; associated&#160; renal&#160; involvement&#160; from&#160; myeloma&#160; kidney&#160; are analyzed&#46; Acute&#160; renal&#160; failure&#160; &#40;ARF&#41;&#160; or&#160; rapidly&#160; progressive renal&#160; insufficiency&#160; &#40;RPRI&#41;&#160; was&#160; defined&#160; as&#160; the&#160; presence&#160; of renal&#160; failure at&#160; the&#160; time of&#160; renal biopsy with a normal prior renal function or a doubling of basal creatinine in a short time period &#40;less than 30 days&#41;&#46; Patients who had known renal damage&#160; for&#160; a&#160; period&#160; longer&#160; than&#160; 30&#160; days with&#160; renal&#160; function data similar to those found at diagnosis and patients with ultrasonographically&#160; small&#160; and&#160; poorly&#160; differentiated&#160; kidneys were&#160; diagnosed&#160; chronic&#160; renal&#160; failure&#160; &#40;CRF&#41;&#46;&#160; Screening&#160; for plasma&#160; cell&#160; dyscrasia&#160; was&#160; based&#160; on&#160; previously&#160; established <br></br>criteria&#46;5 Bone marrow cellularity was studied in five patients&#44; and&#160; flow cytometry was also performed&#160; in&#160; two of&#160; these patients&#46;&#160;&#160; Blood&#160; electrophoresis was performed&#160; in&#160; all patients&#44; and&#160; light&#160; chains were&#160; studied&#160; in&#160; the&#160; urine&#160; of&#160; five&#160; of&#160; them using&#160; nephelometry&#46;&#160; Immunofixation&#160; in&#160; blood&#160; or&#160; urine was not available&#160; in any case&#46; Patients with clinical data suggesting involvement of other organs were considered to have extrarenal involvement due to light chain deposition&#46; <br></br><br></br>RESULTS <br></br><br></br>Six cases of LCDD&#44; all of them diagnosed in the 1999-2005 period&#44; were identified in four female and two male patients with a mean age of 57 years &#40;range&#44; 37-74&#41;&#46; All patients had advanced&#160; renal&#160; failure&#160; and&#160; proteinuria&#160; at&#160; diagnosis&#44;&#160; with mean plasma creatinine levels of 4&#46;3 &#177; 1&#46;59 mg&#47;dL and proteinuria of 4&#46;3 &#177; 1&#46;7 g&#47;24 h&#46; Four patients &#40;66&#37;&#41; showed acute or rapidly progressive renal damage&#44; and two patients had chronic renal failure&#46; Blood electrophoresis detected no monoclonal peaks in any case&#46; Hypogammaglobulinemia was found in 5 patients &#40;83&#46;3&#37;&#41;&#46; In a patient&#44; light chain study in urine showed a selective elevation of the kappa light chain suggesting a monoclonal peak &#40;this patient was subsequently diagnosed of myeloma&#41;&#46;&#160; <br></br><br></br>Bone marrow was studied in 5 patients&#46; Of these&#44; three patients were&#160; diagnosed&#160; of myeloma&#44;&#160; two&#160; based&#160; on&#160; the&#160; bone marrow study and one on the lytic images seen&#46; All bone marrow aspirates showed a proportion of plasma cells lower than 10&#37;&#44;&#160; and&#160; myeloma&#160; was&#160; diagnosed&#160; based&#160; on&#160; a&#160; cytometry study in the two patients in whom this was performed&#46; LCDD was the first sign of the disease in all 3 patients with myeloma&#46; No evidence of myeloma or other plasma cell dyscrasia was found in 3 patients&#46; <br></br><br></br>Table I shows the characteristics of renal biopsies&#46; They all showed&#160; thickening&#160; and&#160; kappa&#160; chain&#160; deposits&#160; in&#160; the&#160; tubular basement membrane&#46; A nodular&#160; pattern&#160; &#40;fig&#46;&#160; 1&#41; with&#160; kappa chain&#160; deposition&#160; in&#160; GBM&#44;&#160; Bowman&#191;s&#160; capsule&#44;&#160; and&#160; mesangium&#160; &#40;fig&#46; 2&#41;&#44; was seen in 5 patients &#40;83&#46;3&#37;&#41;&#46; A patient showed&#160; tubulointerstitial&#160; involvement with&#160; lymphoplasmocytic infiltrate in interstitium and casts with peripheral cellular reaction&#160; and&#160; kappa&#160; chain&#160; deposition&#160; in&#160; casts&#160; and&#160; TBM&#46; <br></br><br></br>Hemodialysis had&#160; to be started&#160; in 5 patients &#40;4 with ARF or RPRI and one with CRF&#41;&#46; Two of&#160; these patients had myeloma&#46; Mean time from diagnosis to start of dialysis was 46 days &#40;range&#44; 0-180&#41;&#46; In patients diagnosed of myeloma and idiopathic LCDD&#44; times to start of dialysis were 96 days &#40;range&#44; 13-180&#41; and 7 days &#40;range&#44; 0-13&#41; respectively&#46; Apatient had heart failure&#160; and&#160; episodes&#160; of&#160; paroxysmal&#160; atrial&#160; fibrillation&#160; with echocardiographic &#40;left ventricular hypertrophy&#41; and electrocardiographic&#160; &#40;relative&#160; microvoltage&#41;&#160; evidence&#160; suggesting cardiac involvement from immunoglobulin deposit&#46; <br></br><br></br>Three&#160; patients were&#160; given&#160; immunosuppressive&#160; treatment&#46; Two&#160; patients&#160; diagnosed&#160; of&#160; myeloma&#160; received&#160; VAD&#160; cycles &#40;vincristine&#44;&#160; adriamycin&#44;&#160; dexamethasone&#41;&#44;&#160; and&#160; one&#160; patient with&#160; idiopathic LCDD was&#160; treated with corticosteroids&#46; The third patient diagnosed of myeloma died 15 days after admission from an infectious complication and did not receive chemotherapy&#46; The patient with idiopathic LCDD was treated for only&#160; four months&#160; because&#160; she&#160; developed&#160; catheter-induced bacteremia&#160; that&#160; required&#160; corticosteroid&#160; discontinuation&#46;&#160; She died at two years of follow-up&#46; <br></br><br></br>Follow-up&#160; time&#160; from&#160; diagnosis&#160; to&#160; death&#160; or&#160; study&#160; closure was highly variable &#40;15 days-59 months&#41;&#44; with a mean of 27 months&#46; Mean follow-up time was 15 months in myeloma patients &#40;15 days-40 months&#41; and 38 months &#40;26-54 months&#41; in idiopathic LCDD&#46; Of the 4 patients who died &#40;66&#46;6&#37;&#41;&#44; 2 had myeloma&#46; Mean survival of myeloma patients was 13 weeks &#40;one died at 15 days and the other at six months&#41;&#44; as compared to a mean survival of 110 weeks in patients with idiopathic&#160; LCDD&#160; &#40;one&#160; died&#160; at&#160; 26&#160; months&#160; and&#160; the&#160; other&#160; at&#160; 29 months&#41;&#46; <br></br><br></br>Two&#160; of&#160; the&#160; 6&#160; patients&#160; studied&#44;&#160; one&#160; diagnosed&#160; idiopathic LCDD and the other myeloma&#44; were still alive at study closure&#46; The patient diagnosed of&#160; idiopathic LCDD&#160; continues on dialysis after almost 5 years of follow-up&#44; has not experienced involvement of other organs or a malignant hematological disease&#44; and is in a waiting list for receiving a kidney transplant&#44; though&#160; indication&#160; of&#160; this&#160; treatment&#160; is&#160; doubtful&#160; according&#160; to some authors&#46;6&#44;7 The myeloma patient has a stable renal function after receiving chemotherapy&#44; with no requirement of replacement therapy after 40 months of follow-up&#46; <br></br><br></br>DISCUSSION <br></br><br></br>Necropsy studies in myeloma patients have found renal involvement&#160; by LCDD&#160; in&#160; 5&#37;&#160; of&#160; cases&#46; However&#44;&#160; the&#160; frequency with which&#160; the disease&#160; is diagnosed&#160; is much&#160; lower&#46;8 All our cases were diagnosed from 1999 to 2005 among the total 640 renal&#160; biopsies&#160; performed&#160; during&#160; this&#160; period&#44;&#160; representing&#160; a LCDD&#160; rate of 1&#37;&#44; greater&#160; than&#160; reported&#160; in other series&#46;8&#44;9 In our analysis&#44; LCDD was more frequent in females and occurred&#160; in middle-aged people&#44;&#160; though&#160; it cannot be ruled out&#160; in young people&#46; <br></br><br></br>Unlike&#160; the&#160; findings&#160; in&#160; other&#160; series&#44;&#160; a&#160; monoclonal&#160; peak could&#160; not&#160; be&#160; detected&#160; in&#160; blood&#160; or&#160; urine&#160; from&#160; any&#160; patient&#44; which may possibly be related&#160; to&#160; the sensitivity of&#160; the diagnostic procedures used&#46; Hence&#44; blood and urine immunofixation should be requested&#44; either routinely or if a strong clinical&#160; suspicion&#160; exists&#44;&#160; even&#160; if&#160; electrophoresis&#160; is&#160; normal&#46; However&#44; even sensitive procedures such as immunofixation are&#160; not&#160; able&#160; to&#160; detect&#160; a monoclonal&#160; peak&#160; in&#160; up&#160; to&#160; 30&#37;&#160; of cases&#46; Renal biopsy therefore plays an essential role in diagnosis of LCDD and its associated dysproteinemia&#44; as evidenced by this and other studies&#46;10 Most of our patients had hypogammaglobulinemia&#160; and&#160; albuminuria&#44;&#160; and&#160; these&#160; findings should therefore lead to suspect some form of immunoglobulin deposition disease&#44; as suggested by other studies&#46;11&#44;12 Clinically&#44; LCDD started in most of our patients as an ARF&#47;RPRI associated&#160; to&#160; nephrotic&#160; proteinuria&#46;&#160; Since&#160; no&#160; monoclonal peak was&#160; detected&#160; in&#160; blood&#160; and&#160; urine&#160; from&#160; any&#160; of&#160; our&#160; patients&#44; LCDD should also be suspected&#160; in&#160; the event of&#160; renal failure and proteinuria of an unknown origin&#46; Renal biopsy is required for diagnosis&#46; <br></br><br></br>In all our patients&#44; the chain deposited in the kidney was of the&#160; kappa&#160; type&#46;&#160; This&#160; is&#160; the&#160; chain&#160; primarily&#160; deposited&#160; in LCDD&#160; according&#160; to&#160; all&#160; series&#44; unlike&#160; in&#160; amyloidosis&#44; where the lambda chain is deposited&#46;13&#44;14 The classical pattern of nodular&#160; glomerulosclerosis was&#160; found&#160; in most&#160; of&#160; our&#160; biopsies &#40;83&#37;&#41;&#44;&#160; and&#160; tubulointerstitial&#160; involvement&#160; alone&#44; with&#160; lymphoplasmocytic infiltration&#44; occurred in one patient&#46; Bone marrow study is not always diagnostic&#44; and routine staining with anti-kappa and anti-lambda sera would therefore be required to prevent the disease from being undiagnosed&#46; <br></br><br></br>Diagnosis of myeloma may be made with a flow cytometry study&#44;5 but as this analysis is not performed in many hospitals&#44; marrow&#160; cellularity&#160; continues&#160; to be&#160; a diagnostic&#160; criterion&#160; together with the monoclonal peak in blood or urine and involvement&#160; of&#160; other&#160; organs&#46;&#160; The&#160; proportion&#160; of&#160; plasma&#160; cells&#160; in bone marrow was&#160; not&#160; higher&#160; than&#160; 10&#37;&#160; in&#160; none&#160; of&#160; our&#160; patients&#44; so&#160; that&#160; if&#160; this criterion&#160; is applied&#44; myeloma diagnosis may&#160; be&#160; delayed&#46; Thus&#44; when&#160; plasma&#160; cell&#160; dyscrasia&#160; such&#160; as LCDD&#160; is&#160; strongly&#160; suspected&#44;&#160; plasma&#160; cell&#160; phenotype would have to be determined to rule out a hematological tumor pathology&#46; This happened with one of our patients&#44; who underwent two bone marrow aspirations with a one month interval&#46; Cellularity was similar in both samples &#40;5&#37;&#41;&#44; but flow cytometry performed in the second aspirate provided a diagnosis of myeloma&#46; <br></br><br></br>Dialysis&#160; requirements&#160; were&#160; high&#160; &#40;83&#37;&#41;&#160; for&#160; the&#160; whole group&#44; but particularly for patients with idiopathic LCDD&#44; all of whom required dialysis and at an earlier time than the myeloma group&#46; <br></br><br></br>Chemotherapy&#44;&#160; which&#160; is&#160; unquestionable&#160; in myeloma&#160; patients&#44; is controversial when no malignant disease exists&#46; However&#44; general practice has consisted of treatment with corticosteroids&#160; plus&#160; melphalan&#44;&#160; regardless&#160; of&#160; the&#160; associated hematological disease&#44; although myeloma patients more&#160; frequently receive VAD cycles&#44; which appear to have a protective effect upon patient survival&#46;15-18 Recent studies showed disappearance&#160; of&#160; light&#160; chain&#160; deposits&#160; in&#160; the&#160; kidney&#160; after treatment with&#160; chemotherapy&#160; and bone marrow&#160; transplantation&#44;17&#44;19 which&#160; would&#160; support&#160; intensive&#160; therapy&#160; in&#160; patients with LCDD&#46; Two of our myeloma patients received VAD cycles&#44; and renal function improvement was achieved in one of them&#46; <br></br><br></br>Mortality was high in our patients&#46; The same number of patients died in the myeloma and the idiopathic LCDD groups&#44; but the mean follow-up time from diagnosis to death was longer&#160; in&#160; patients&#160; with&#160; idiopathic&#160; LCDD&#160; &#40;110&#160; vs 13&#160; weeks&#41;&#46; This&#44;&#160; combined&#160; with&#160; the&#160; longer&#160; follow-up&#160; time&#160; in&#160; patients with&#160; idiopathic LCDD&#160; &#40;38&#160; vs 15 months&#41;&#44;&#160; suggests&#160; that&#160; the presence of myeloma in the setting of LCDD shortens patient survival&#44; as seen in other series &#40;20&#41;&#44; though there are reports that do not support these data &#40;4&#44;6&#41;&#46; <br></br><br></br>To&#160; sum&#160; up&#44;&#160; the&#160; number&#160; of&#160; patients&#160; with&#160; a&#160; diagnosis&#160; of LCDD detected in our series was higher than expected according to literature&#46; Wider studies would be required to confirm these results&#46; LCDD was associated to a myeloma in half of our&#160; patients&#44;&#160; and&#160; its&#160; first&#160; manifestation&#160; was&#160; renal&#160; involvement&#46; The predominant clinical sign was acute renal function impairment with nephrotic proteinuria&#160; that required dialysis&#46; The&#160; structural&#160; lesion most&#160; commonly&#160; associated&#160; is&#160; nodular glomerulosclerosis&#46; Renal and patient survival was poor&#46; <br></br>"
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        "resumen" => "La enfermedad por dep&#243;sito de cadenas ligeras &#40;EDCL&#41; es una entidad rara&#44; caracterizada por el dep&#243;sito de un solo tipo de cadena ligera en la membrana basal del ri&#241;&#243;n&#46; Puede asociarse a una discrasia de c&#233;lulas plasm&#225;ticas&#44; aunque en ocasiones no se detecta patolog&#237;a hematol&#243;gica y se denomina idiop&#225;tica&#46; Suele manifestarse como una insuficiencia renal severa con proteinuria nefr&#243;tica&#44; no tiene tratamiento claramente establecido y el pron&#243;stico es malo&#46; El objetivo de este trabajo es analizar las caracter&#237;sticas de los casos de EDCL diagnosticados en nuestro medio&#46; Se identifican 6 casos&#44; todos entre 1999 y 2005 de un total de 640 biopsias realizadas en ese periodo&#44; 4 mujeres y 2 varones&#44; media de 57 a&#241;os&#46; Se detect&#243; un mieloma en 3 pacientes &#40;50&#37;&#41;&#46; La insuficiencia renal aguda o de r&#225;pida evoluci&#243;n fue la presentaci&#243;n cl&#237;nica m&#225;s frecuente &#40;66&#37;&#41; junto con proteinuria nefr&#243;tica &#40;66&#37;&#41;&#46; Todas las biopsias mostraban engrosamiento de la membrana basal tubular y dep&#243;sito lineal de cadenas kappa en la misma&#46; La lesi&#243;n glomerular m&#225;s frecuente fue la glomeruloesclerosis nodular &#40;83&#37;&#41;&#46; En un caso la afectaci&#243;n fue exclusivamente t&#250;bulo intersticial con cilindros tubulares asociados&#46; Se trataron 3 pacientes&#44; 2 con mieloma&#46; Requirieron di&#225;lisis 5 pacientes&#58; 3 con EDCL idiop&#225;tica con un tiempo medio desde el diagn&#243;stico hasta recibir la misma de 7 d&#237;as&#44; y 2 con mieloma que tardaron una media de 46 d&#237;as en requerir di&#225;lisis&#46; Fallecieron 4 pacientes&#44; 2 con mieloma&#46; El tiempo de seguimiento hasta el exitus fue de 13 semanas para los pacientes con mieloma y de 110 semanas para el resto&#46; Conclusi&#243;n&#44; la EDCL parece mas frecuente de lo publicado y se asocia a mieloma en la mitad de los casos&#46; Se presenta con da&#241;o renal severo y la evoluci&#243;n renal y del paciente es mala&#46; "
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        "resumen" => "The Light chain deposition disease &#40;LCDD&#41; is a strange entity characterised by the deposition of only one type of light chain in the renal tubular basement membranes&#46; It can be associated to a plasma cell dyscrasia&#44; however&#44; it can occur in the absence of any detectable hematological disorder and it is called idiopathic LCDD&#46; The clinical manifestation is renal insufficiency and nephrotic proteinuria&#44; it does not have a clearly fixed treatment and has a severe prognosis&#46; The aim of this work is to analyse the characteristics of the LCDD cases diagnosed within our environment&#46; Six cases were identified&#44; all of them between 1999 and 2005&#44; from a total amount of 640 renal biopsies performed during this period&#44; 4 women and 2 men&#44; average age of 57&#46; Multiple myeloma in 3 patients was detected &#40;50&#37;&#41;&#46; The acute renal failure or rapidly progressive renal insufficiency was the most frequent clinical presentation &#40;66&#37;&#41; together with nephrotic proteinuria &#40;66&#37;&#41;&#46; All the biopsies showed tubular basement membranes thickening and kappa chains with a linear distribution within the same&#46; The most frequent glomerular pathological finding was the nodular sclerosing glomerulopathy &#40;83&#37;&#41;&#46; In one of the cases the affectation was exclusively tubular interstitial with tubular casts&#46; 3 patients were treated&#44; 2 with multiple myeloma&#46; 5 patients needed dialysis&#58; 3 with idiopathic LCDD within an average time of 7 days from the diagnosis to its reception&#44; and 2 with myeloma&#44; who started needing dialysis in an average of 46 days&#46; 4 patients died&#44; 2 of them with myeloma&#46; The monitoring time until the death was 13 weeks for the patients with myeloma and 110 weeks for the rest&#46; Conclusion&#58; The LCDD seems to be more frequent than what has been published and it is associated to the myeloma in half of the cases&#46; It appears together with severe renal insufficiency and the patient&#191;s and renal prognosis is poor&#46;"
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Light chain deposition disease. Experience in our environment
Enfermedad por depósito de cadenas ligeras. Experiencia en nuestro medio.
Carmen Martin Herreraa, Marta Suñer Pobleta, Margarita Diaz Pedreroa, Jorge Fernandez Alonsoa, Rocio Cabrerab
a Servicios de Nefrología, Hospital Universitario Virgen del Rocío, Sevilla, Sevilla, España,
b Servicios de Anatomía Patológica, Hospital Universitario Virgen del Rocío, Sevilla, Sevilla, España,
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    "textoCompleto" => "INTRODUCTION <br></br><br></br>B-cell proliferative diseases are usually associated to production and secretion into blood of a monoclonal immunoglobulin&#44; or a fragment of it&#44; that may be deposited in the organs in an organized form as crystals&#44; fibrils or microtubules&#44; or in a non-organized&#160; form&#160; &#40;granular&#41;&#46;&#160; This&#160; immunoglobulin&#160; will mainly be deposited in the kidney&#44; not only because this is a highly vascularized organ&#44; but also because&#160; the&#160; renal&#160; tubule has&#160; a&#160; predominant&#160; role&#160; in&#160; immunoglobulin&#160; metabolism&#46;1 Diagnosis of renal involvement due to immunoglobulin deposition is being expanded with development and routine implementation&#160; of&#160; different&#160; laboratory&#160; procedures&#160; &#40;staining&#160; with <br></br>antibodies specific to kappa and lambda light chains&#44; electron microscopy study&#44; development of procedures with an increasing&#160; sensitivity&#160; for&#160; detecting&#160; the monoclonal&#160; component&#160; in blood&#160; or&#160; urine&#41;&#46;2 Light&#160; chain&#160; deposition&#160; disease&#160; &#40;LCDD&#41;&#160; is characterized&#160; by&#160; generalized&#160; deposition&#160; of&#160; a&#160; single&#160; type&#160; of light&#160; chain&#160; along&#160; the&#160; renal&#160; basement&#160; membrane&#46;&#160; LCDD&#160; is usually&#160; reported&#160; to&#160; occur&#160; during&#160; plasma&#160; cell&#160; dyscrasia&#160; or another&#160; lymphoproliferative disorder&#44; but may&#160; also occur&#160; in the absence of any hematological disorder&#44; in which case it is <br></br>called idiopathic LCDD&#46; Severe renal insufficiency occurs in most patients despite&#160; treatment&#46;3&#44;4 The most&#160; typical&#160; renal&#160; lesion is nodular glomerulosclerosis&#44; in which mesangial nodules and deposition of a single chain type occur along the glomerular&#160; basement&#160; membrane&#46;&#160; However&#44;&#160; diagnosis&#160; will&#160; be based&#160; on&#160; light&#160; chain&#160; deposition&#160; along&#160; the&#160; tubular&#160; basement membrane&#46; The aim of this study was to review our experience with this uncommon disease&#46; <br></br><br></br>MATERIALS AND METHODS <br></br><br></br>Cases&#160; of&#160; LCDD&#160; diagnosed&#160; at&#160; our&#160; hospital&#160; among&#160; all&#160; adult biopsies performed&#160; in&#160; the 1978-2005 period were&#160; analyzed&#46; Clinical and pathological data and patient course were studied based on clinical records and on the information provided by physicians with whom the patient was in direct contact at the time of study closure&#46; All renal biopsies taken during the abovementioned&#160; period were&#160; read&#160; by&#160; the&#160; same&#160; pathologist&#46; All samples&#160; diagnosed&#160; as&#160; LCDD&#160; had&#160; been&#160; processed&#160; for&#160; study with&#160; light microscopy&#160; &#40;LM&#41;&#160; and&#160; immunofluorescence&#160; &#40;IF&#41;&#46; <br></br>Samples from five patients were also analyzed using electron microscopy&#160; &#40;EM&#41;&#46; Diagnosis&#160; of LCDD was made&#160; by&#160; an&#160; IF study&#44; in which sera against kappa and lambda light chains are used&#46; This procedure has been routinely performed at our hospital since 2002&#46; The presence of nodular glomerulosclerosis&#44; chain&#160; deposit&#160; distribution&#44;&#160; thickening&#160; of&#160; basement membranes&#44; extent of tubulointerstitial involvement&#44; and the presence of&#160; associated&#160; renal&#160; involvement&#160; from&#160; myeloma&#160; kidney&#160; are analyzed&#46; Acute&#160; renal&#160; failure&#160; &#40;ARF&#41;&#160; or&#160; rapidly&#160; progressive renal&#160; insufficiency&#160; &#40;RPRI&#41;&#160; was&#160; defined&#160; as&#160; the&#160; presence&#160; of renal&#160; failure at&#160; the&#160; time of&#160; renal biopsy with a normal prior renal function or a doubling of basal creatinine in a short time period &#40;less than 30 days&#41;&#46; Patients who had known renal damage&#160; for&#160; a&#160; period&#160; longer&#160; than&#160; 30&#160; days with&#160; renal&#160; function data similar to those found at diagnosis and patients with ultrasonographically&#160; small&#160; and&#160; poorly&#160; differentiated&#160; kidneys were&#160; diagnosed&#160; chronic&#160; renal&#160; failure&#160; &#40;CRF&#41;&#46;&#160; Screening&#160; for plasma&#160; cell&#160; dyscrasia&#160; was&#160; based&#160; on&#160; previously&#160; established <br></br>criteria&#46;5 Bone marrow cellularity was studied in five patients&#44; and&#160; flow cytometry was also performed&#160; in&#160; two of&#160; these patients&#46;&#160;&#160; Blood&#160; electrophoresis was performed&#160; in&#160; all patients&#44; and&#160; light&#160; chains were&#160; studied&#160; in&#160; the&#160; urine&#160; of&#160; five&#160; of&#160; them using&#160; nephelometry&#46;&#160; Immunofixation&#160; in&#160; blood&#160; or&#160; urine was not available&#160; in any case&#46; Patients with clinical data suggesting involvement of other organs were considered to have extrarenal involvement due to light chain deposition&#46; <br></br><br></br>RESULTS <br></br><br></br>Six cases of LCDD&#44; all of them diagnosed in the 1999-2005 period&#44; were identified in four female and two male patients with a mean age of 57 years &#40;range&#44; 37-74&#41;&#46; All patients had advanced&#160; renal&#160; failure&#160; and&#160; proteinuria&#160; at&#160; diagnosis&#44;&#160; with mean plasma creatinine levels of 4&#46;3 &#177; 1&#46;59 mg&#47;dL and proteinuria of 4&#46;3 &#177; 1&#46;7 g&#47;24 h&#46; Four patients &#40;66&#37;&#41; showed acute or rapidly progressive renal damage&#44; and two patients had chronic renal failure&#46; Blood electrophoresis detected no monoclonal peaks in any case&#46; Hypogammaglobulinemia was found in 5 patients &#40;83&#46;3&#37;&#41;&#46; In a patient&#44; light chain study in urine showed a selective elevation of the kappa light chain suggesting a monoclonal peak &#40;this patient was subsequently diagnosed of myeloma&#41;&#46;&#160; <br></br><br></br>Bone marrow was studied in 5 patients&#46; Of these&#44; three patients were&#160; diagnosed&#160; of myeloma&#44;&#160; two&#160; based&#160; on&#160; the&#160; bone marrow study and one on the lytic images seen&#46; All bone marrow aspirates showed a proportion of plasma cells lower than 10&#37;&#44;&#160; and&#160; myeloma&#160; was&#160; diagnosed&#160; based&#160; on&#160; a&#160; cytometry study in the two patients in whom this was performed&#46; LCDD was the first sign of the disease in all 3 patients with myeloma&#46; No evidence of myeloma or other plasma cell dyscrasia was found in 3 patients&#46; <br></br><br></br>Table I shows the characteristics of renal biopsies&#46; They all showed&#160; thickening&#160; and&#160; kappa&#160; chain&#160; deposits&#160; in&#160; the&#160; tubular basement membrane&#46; A nodular&#160; pattern&#160; &#40;fig&#46;&#160; 1&#41; with&#160; kappa chain&#160; deposition&#160; in&#160; GBM&#44;&#160; Bowman&#191;s&#160; capsule&#44;&#160; and&#160; mesangium&#160; &#40;fig&#46; 2&#41;&#44; was seen in 5 patients &#40;83&#46;3&#37;&#41;&#46; A patient showed&#160; tubulointerstitial&#160; involvement with&#160; lymphoplasmocytic infiltrate in interstitium and casts with peripheral cellular reaction&#160; and&#160; kappa&#160; chain&#160; deposition&#160; in&#160; casts&#160; and&#160; TBM&#46; <br></br><br></br>Hemodialysis had&#160; to be started&#160; in 5 patients &#40;4 with ARF or RPRI and one with CRF&#41;&#46; Two of&#160; these patients had myeloma&#46; Mean time from diagnosis to start of dialysis was 46 days &#40;range&#44; 0-180&#41;&#46; In patients diagnosed of myeloma and idiopathic LCDD&#44; times to start of dialysis were 96 days &#40;range&#44; 13-180&#41; and 7 days &#40;range&#44; 0-13&#41; respectively&#46; Apatient had heart failure&#160; and&#160; episodes&#160; of&#160; paroxysmal&#160; atrial&#160; fibrillation&#160; with echocardiographic &#40;left ventricular hypertrophy&#41; and electrocardiographic&#160; &#40;relative&#160; microvoltage&#41;&#160; evidence&#160; suggesting cardiac involvement from immunoglobulin deposit&#46; <br></br><br></br>Three&#160; patients were&#160; given&#160; immunosuppressive&#160; treatment&#46; Two&#160; patients&#160; diagnosed&#160; of&#160; myeloma&#160; received&#160; VAD&#160; cycles &#40;vincristine&#44;&#160; adriamycin&#44;&#160; dexamethasone&#41;&#44;&#160; and&#160; one&#160; patient with&#160; idiopathic LCDD was&#160; treated with corticosteroids&#46; The third patient diagnosed of myeloma died 15 days after admission from an infectious complication and did not receive chemotherapy&#46; The patient with idiopathic LCDD was treated for only&#160; four months&#160; because&#160; she&#160; developed&#160; catheter-induced bacteremia&#160; that&#160; required&#160; corticosteroid&#160; discontinuation&#46;&#160; She died at two years of follow-up&#46; <br></br><br></br>Follow-up&#160; time&#160; from&#160; diagnosis&#160; to&#160; death&#160; or&#160; study&#160; closure was highly variable &#40;15 days-59 months&#41;&#44; with a mean of 27 months&#46; Mean follow-up time was 15 months in myeloma patients &#40;15 days-40 months&#41; and 38 months &#40;26-54 months&#41; in idiopathic LCDD&#46; Of the 4 patients who died &#40;66&#46;6&#37;&#41;&#44; 2 had myeloma&#46; Mean survival of myeloma patients was 13 weeks &#40;one died at 15 days and the other at six months&#41;&#44; as compared to a mean survival of 110 weeks in patients with idiopathic&#160; LCDD&#160; &#40;one&#160; died&#160; at&#160; 26&#160; months&#160; and&#160; the&#160; other&#160; at&#160; 29 months&#41;&#46; <br></br><br></br>Two&#160; of&#160; the&#160; 6&#160; patients&#160; studied&#44;&#160; one&#160; diagnosed&#160; idiopathic LCDD and the other myeloma&#44; were still alive at study closure&#46; The patient diagnosed of&#160; idiopathic LCDD&#160; continues on dialysis after almost 5 years of follow-up&#44; has not experienced involvement of other organs or a malignant hematological disease&#44; and is in a waiting list for receiving a kidney transplant&#44; though&#160; indication&#160; of&#160; this&#160; treatment&#160; is&#160; doubtful&#160; according&#160; to some authors&#46;6&#44;7 The myeloma patient has a stable renal function after receiving chemotherapy&#44; with no requirement of replacement therapy after 40 months of follow-up&#46; <br></br><br></br>DISCUSSION <br></br><br></br>Necropsy studies in myeloma patients have found renal involvement&#160; by LCDD&#160; in&#160; 5&#37;&#160; of&#160; cases&#46; However&#44;&#160; the&#160; frequency with which&#160; the disease&#160; is diagnosed&#160; is much&#160; lower&#46;8 All our cases were diagnosed from 1999 to 2005 among the total 640 renal&#160; biopsies&#160; performed&#160; during&#160; this&#160; period&#44;&#160; representing&#160; a LCDD&#160; rate of 1&#37;&#44; greater&#160; than&#160; reported&#160; in other series&#46;8&#44;9 In our analysis&#44; LCDD was more frequent in females and occurred&#160; in middle-aged people&#44;&#160; though&#160; it cannot be ruled out&#160; in young people&#46; <br></br><br></br>Unlike&#160; the&#160; findings&#160; in&#160; other&#160; series&#44;&#160; a&#160; monoclonal&#160; peak could&#160; not&#160; be&#160; detected&#160; in&#160; blood&#160; or&#160; urine&#160; from&#160; any&#160; patient&#44; which may possibly be related&#160; to&#160; the sensitivity of&#160; the diagnostic procedures used&#46; Hence&#44; blood and urine immunofixation should be requested&#44; either routinely or if a strong clinical&#160; suspicion&#160; exists&#44;&#160; even&#160; if&#160; electrophoresis&#160; is&#160; normal&#46; However&#44; even sensitive procedures such as immunofixation are&#160; not&#160; able&#160; to&#160; detect&#160; a monoclonal&#160; peak&#160; in&#160; up&#160; to&#160; 30&#37;&#160; of cases&#46; Renal biopsy therefore plays an essential role in diagnosis of LCDD and its associated dysproteinemia&#44; as evidenced by this and other studies&#46;10 Most of our patients had hypogammaglobulinemia&#160; and&#160; albuminuria&#44;&#160; and&#160; these&#160; findings should therefore lead to suspect some form of immunoglobulin deposition disease&#44; as suggested by other studies&#46;11&#44;12 Clinically&#44; LCDD started in most of our patients as an ARF&#47;RPRI associated&#160; to&#160; nephrotic&#160; proteinuria&#46;&#160; Since&#160; no&#160; monoclonal peak was&#160; detected&#160; in&#160; blood&#160; and&#160; urine&#160; from&#160; any&#160; of&#160; our&#160; patients&#44; LCDD should also be suspected&#160; in&#160; the event of&#160; renal failure and proteinuria of an unknown origin&#46; Renal biopsy is required for diagnosis&#46; <br></br><br></br>In all our patients&#44; the chain deposited in the kidney was of the&#160; kappa&#160; type&#46;&#160; This&#160; is&#160; the&#160; chain&#160; primarily&#160; deposited&#160; in LCDD&#160; according&#160; to&#160; all&#160; series&#44; unlike&#160; in&#160; amyloidosis&#44; where the lambda chain is deposited&#46;13&#44;14 The classical pattern of nodular&#160; glomerulosclerosis was&#160; found&#160; in most&#160; of&#160; our&#160; biopsies &#40;83&#37;&#41;&#44;&#160; and&#160; tubulointerstitial&#160; involvement&#160; alone&#44; with&#160; lymphoplasmocytic infiltration&#44; occurred in one patient&#46; Bone marrow study is not always diagnostic&#44; and routine staining with anti-kappa and anti-lambda sera would therefore be required to prevent the disease from being undiagnosed&#46; <br></br><br></br>Diagnosis of myeloma may be made with a flow cytometry study&#44;5 but as this analysis is not performed in many hospitals&#44; marrow&#160; cellularity&#160; continues&#160; to be&#160; a diagnostic&#160; criterion&#160; together with the monoclonal peak in blood or urine and involvement&#160; of&#160; other&#160; organs&#46;&#160; The&#160; proportion&#160; of&#160; plasma&#160; cells&#160; in bone marrow was&#160; not&#160; higher&#160; than&#160; 10&#37;&#160; in&#160; none&#160; of&#160; our&#160; patients&#44; so&#160; that&#160; if&#160; this criterion&#160; is applied&#44; myeloma diagnosis may&#160; be&#160; delayed&#46; Thus&#44; when&#160; plasma&#160; cell&#160; dyscrasia&#160; such&#160; as LCDD&#160; is&#160; strongly&#160; suspected&#44;&#160; plasma&#160; cell&#160; phenotype would have to be determined to rule out a hematological tumor pathology&#46; This happened with one of our patients&#44; who underwent two bone marrow aspirations with a one month interval&#46; Cellularity was similar in both samples &#40;5&#37;&#41;&#44; but flow cytometry performed in the second aspirate provided a diagnosis of myeloma&#46; <br></br><br></br>Dialysis&#160; requirements&#160; were&#160; high&#160; &#40;83&#37;&#41;&#160; for&#160; the&#160; whole group&#44; but particularly for patients with idiopathic LCDD&#44; all of whom required dialysis and at an earlier time than the myeloma group&#46; <br></br><br></br>Chemotherapy&#44;&#160; which&#160; is&#160; unquestionable&#160; in myeloma&#160; patients&#44; is controversial when no malignant disease exists&#46; However&#44; general practice has consisted of treatment with corticosteroids&#160; plus&#160; melphalan&#44;&#160; regardless&#160; of&#160; the&#160; associated hematological disease&#44; although myeloma patients more&#160; frequently receive VAD cycles&#44; which appear to have a protective effect upon patient survival&#46;15-18 Recent studies showed disappearance&#160; of&#160; light&#160; chain&#160; deposits&#160; in&#160; the&#160; kidney&#160; after treatment with&#160; chemotherapy&#160; and bone marrow&#160; transplantation&#44;17&#44;19 which&#160; would&#160; support&#160; intensive&#160; therapy&#160; in&#160; patients with LCDD&#46; Two of our myeloma patients received VAD cycles&#44; and renal function improvement was achieved in one of them&#46; <br></br><br></br>Mortality was high in our patients&#46; The same number of patients died in the myeloma and the idiopathic LCDD groups&#44; but the mean follow-up time from diagnosis to death was longer&#160; in&#160; patients&#160; with&#160; idiopathic&#160; LCDD&#160; &#40;110&#160; vs 13&#160; weeks&#41;&#46; This&#44;&#160; combined&#160; with&#160; the&#160; longer&#160; follow-up&#160; time&#160; in&#160; patients with&#160; idiopathic LCDD&#160; &#40;38&#160; vs 15 months&#41;&#44;&#160; suggests&#160; that&#160; the presence of myeloma in the setting of LCDD shortens patient survival&#44; as seen in other series &#40;20&#41;&#44; though there are reports that do not support these data &#40;4&#44;6&#41;&#46; <br></br><br></br>To&#160; sum&#160; up&#44;&#160; the&#160; number&#160; of&#160; patients&#160; with&#160; a&#160; diagnosis&#160; of LCDD detected in our series was higher than expected according to literature&#46; Wider studies would be required to confirm these results&#46; LCDD was associated to a myeloma in half of our&#160; patients&#44;&#160; and&#160; its&#160; first&#160; manifestation&#160; was&#160; renal&#160; involvement&#46; The predominant clinical sign was acute renal function impairment with nephrotic proteinuria&#160; 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        "resumen" => "La enfermedad por dep&#243;sito de cadenas ligeras &#40;EDCL&#41; es una entidad rara&#44; caracterizada por el dep&#243;sito de un solo tipo de cadena ligera en la membrana basal del ri&#241;&#243;n&#46; Puede asociarse a una discrasia de c&#233;lulas plasm&#225;ticas&#44; aunque en ocasiones no se detecta patolog&#237;a hematol&#243;gica y se denomina idiop&#225;tica&#46; Suele manifestarse como una insuficiencia renal severa con proteinuria nefr&#243;tica&#44; no tiene tratamiento claramente establecido y el pron&#243;stico es malo&#46; El objetivo de este trabajo es analizar las caracter&#237;sticas de los casos de EDCL diagnosticados en nuestro medio&#46; Se identifican 6 casos&#44; todos entre 1999 y 2005 de un total de 640 biopsias realizadas en ese periodo&#44; 4 mujeres y 2 varones&#44; media de 57 a&#241;os&#46; Se detect&#243; un mieloma en 3 pacientes &#40;50&#37;&#41;&#46; La insuficiencia renal aguda o de r&#225;pida evoluci&#243;n fue la presentaci&#243;n cl&#237;nica m&#225;s frecuente &#40;66&#37;&#41; junto con proteinuria nefr&#243;tica &#40;66&#37;&#41;&#46; Todas las biopsias mostraban engrosamiento de la membrana basal tubular y dep&#243;sito lineal de cadenas kappa en la misma&#46; La lesi&#243;n glomerular m&#225;s frecuente fue la glomeruloesclerosis nodular &#40;83&#37;&#41;&#46; En un caso la afectaci&#243;n fue exclusivamente t&#250;bulo intersticial con cilindros tubulares asociados&#46; Se trataron 3 pacientes&#44; 2 con mieloma&#46; Requirieron di&#225;lisis 5 pacientes&#58; 3 con EDCL idiop&#225;tica con un tiempo medio desde el diagn&#243;stico hasta recibir la misma de 7 d&#237;as&#44; y 2 con mieloma que tardaron una media de 46 d&#237;as en requerir di&#225;lisis&#46; Fallecieron 4 pacientes&#44; 2 con mieloma&#46; El tiempo de seguimiento hasta el exitus fue de 13 semanas para los pacientes con mieloma y de 110 semanas para el resto&#46; Conclusi&#243;n&#44; la EDCL parece mas frecuente de lo publicado y se asocia a mieloma en la mitad de los casos&#46; Se presenta con da&#241;o renal severo y la evoluci&#243;n renal y del paciente es mala&#46; "
      ]
      "en" => array:1 [
        "resumen" => "The Light chain deposition disease &#40;LCDD&#41; is a strange entity characterised by the deposition of only one type of light chain in the renal tubular basement membranes&#46; It can be associated to a plasma cell dyscrasia&#44; however&#44; it can occur in the absence of any detectable hematological disorder and it is called idiopathic LCDD&#46; The clinical manifestation is renal insufficiency and nephrotic proteinuria&#44; it does not have a clearly fixed treatment and has a severe prognosis&#46; The aim of this work is to analyse the characteristics of the LCDD cases diagnosed within our environment&#46; Six cases were identified&#44; all of them between 1999 and 2005&#44; from a total amount of 640 renal biopsies performed during this period&#44; 4 women and 2 men&#44; average age of 57&#46; Multiple myeloma in 3 patients was detected &#40;50&#37;&#41;&#46; The acute renal failure or rapidly progressive renal insufficiency was the most frequent clinical presentation &#40;66&#37;&#41; together with nephrotic proteinuria &#40;66&#37;&#41;&#46; All the biopsies showed tubular basement membranes thickening and kappa chains with a linear distribution within the same&#46; The most frequent glomerular pathological finding was the nodular sclerosing glomerulopathy &#40;83&#37;&#41;&#46; In one of the cases the affectation was exclusively tubular interstitial with tubular casts&#46; 3 patients were treated&#44; 2 with multiple myeloma&#46; 5 patients needed dialysis&#58; 3 with idiopathic LCDD within an average time of 7 days from the diagnosis to its reception&#44; and 2 with myeloma&#44; who started needing dialysis in an average of 46 days&#46; 4 patients died&#44; 2 of them with myeloma&#46; The monitoring time until the death was 13 weeks for the patients with myeloma and 110 weeks for the rest&#46; Conclusion&#58; The LCDD seems to be more frequent than what has been published and it is associated to the myeloma in half of the cases&#46; It appears together with severe renal insufficiency and the patient&#191;s and renal prognosis is poor&#46;"
      ]
    ]
  ]
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    "identificador" => "35441"
    "tipo" => "SECCION"
    "en" => array:2 [
      "titulo" => "Originals"
      "idiomaDefecto" => true
    ]
    "idiomaDefecto" => "en"
  ]
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]
Article information
ISSN: 20132514
Original language: English
DOI:
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