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and an erythrocyte sedimentation rate of 20 mm&#47;h &#40;normal 15-30 mm&#47;h&#41;&#46; Total serum proteins were 5 g&#47;dl and albumin 3&#46;1 g&#47;dl&#46; Urinalysis was normal and serum creatinine ranged from 1&#46;2 to 1&#46;4 mg&#47;dl&#46; He continued receiving treatment with omeprazole&#44; and sixteen months later a barium study of the upper gastrointestinal tract showed normal findings&#46;</p><p class="elsevierStylePara">At admission physical examination was unremarkable&#46; Laboratory data showed hematocrit 34&#46;5 &#37;&#44; hemoglobin 11 g&#47;dl&#44; white blood cells 5950&#47;ml with normal differential count&#44; platelets 161000&#47;ml&#44; and an erythrocyte sedimentation rate of 100 mm&#47;h&#46; Blood coagulation was normal&#46; Urinalysis revealed normal sediment and proteinuria 1&#46;2 g&#47;24 h&#46; Serum urea was 70 mg&#47;dl&#44; serum creatinine 2&#46;2 mg&#47;dl&#44; and creatinine clearance 45 ml&#47;min&#46; The total serum proteins were 9&#46;6 g&#47;dl&#44; albumin 4&#46;1 g&#47;dl&#44; and gammaglobulin 3&#46;5 g&#47;dl &#40;with a monoclonal peak&#41;&#46; The serum immunoglobulin levels measured by nephelometry were IgM 4640 mg&#47;dl &#40;normal 38-231 mg&#47;dl&#41;&#44; IgG 858 mg&#47;dl &#40;normal 650-1700 mg&#47;dl&#41;&#44; and IgA 168 mg&#47;dl &#40;normal 103-568 mg&#47;dl&#41;&#44; kappa 641 mg&#47;dl &#40;normal 170-370 mg&#47;dl&#41;&#44; lambda 106 mg&#47;dl &#40;normal 90-210 mg&#47;dl&#41;&#46; Monoclonal IgM kappa was detected in the serum by immunofixation&#46; Serum cryoglobulin was negative and Bence-Jones proteinuria was positive&#46; Other laboratory data showed C reactive protein 4&#46;9 mg&#47;dl&#44; beta 2-microglobulin 4&#46;9 mg&#47;l&#44; lactate dehydrogenase 286 U&#47;l&#44; serum calcium 9&#46;9 mg&#47;dl&#44; and serum phosphate 3&#46;2 mg&#47;dl&#46; EBV and HCV were negative&#46; Abdominal ultrasound examination showed a solid mass in the left kidney&#46; A skeletal roentgenogram and a bone scan revealed no abnormalities&#46; A thoracoabdominal CT revealed a 7 cm mass located in the midportion of the left kidney &#40;Fig&#46; 1&#41;&#46; There was no evidence of lymphadenopathy&#46; A bone marrow aspirate showed a normocellular patter and revealed 12&#46;5 &#37; atypical plasma cells&#46; Bone marrow flow cytometry showed 3 &#37; polyclonal T lymphocytes and 1 &#37; polyclonal B lymphocytes&#46; The plasma cells were CD38&#43;&#43;&#44; CD138-&#44; CD19&#43;&#44; CD45&#43;&#44; CD56-&#44; CD117-&#44; and monoclonal kappa&#46;</p><p class="elsevierStylePara">On September 2000&#44; a left nephrectomy was performed&#46; The kidney weighed 240 g &#40;including the adrenal gland&#41;&#44; measured 20 x 12 x 8 cm&#44; and contained an 8 x 8 x 2 cm well-circumscribed mass involving the subcortical mid-portion of the kidney&#46; Histologically&#44; there was a lymphoid infiltrate extending diffusely through the pericapsular region and involving the renal cortex&#46; Kidney architecture was effaced by the infiltrate within which occasional residual tubules and glomeruli were evident &#40;Fig 2 A&#41;&#46; In some areas&#44; the infiltrate was diffuse between the reactive lymphoid follicles&#46; Many reactive germinal centers were colonized by neoplastic cell with a residual mantle zone preserved &#40;Fig 2 B&#41;&#46; In other areas&#44; the neoplasm was nodular&#44; composed of reactive lymphoid follicles surrounded by pale zones of small lymphoid cells with pale or clear cytoplasm and slightly irregular nuclear contours &#40;monocytoid and centrocyte-like cells&#41; &#40;Fig 2 C&#41;&#46; In some areas&#44; a prominent plasma cell and plasmacytoid lymphocytes component with occasional intranuclear inclusions &#40;Dutcher bodies&#41; and Russell bodies was observed &#40;Fig 2 D&#41;&#46; The neoplastic cells expressed monotypic cytoplasmic IgM kappa by immunoperoxidase&#46; The neoplastic cells also reacted with the pan-B-cell antibody L26 &#40;CD20&#44; CD79a&#41;&#44; coexpression of CD43 and stain of atypical lymphocytes with bcl-2 consistent with a low-grade B-cell lymphoma of MALT&#46; Lymphoepithelial lesions were occasionally observed with cytokeratin immunostain&#46; In the areas of the kidney not involved by lymphoma&#44; there were histologic signs of nephrosclerosis&#46;</p><p class="elsevierStylePara">&#160;&#160;&#160;&#160;&#160;&#160;&#160;&#160;&#160;&#160;&#160; Two months after nephrectomy the serum creatinine was 3 mg&#47;dl and the erythrocyte sedimentation rate remained elevated to 141 mm&#47;h&#46; The total serum proteins were 8&#46;4 g&#47;dl&#44; albumin 4&#46;1 g&#47;dl&#44; and gammaglobulin 2&#46;4 g&#47;dl with a monoclonal peak&#46; The serum immunoglobulin levels were IgM 2970 mg&#47;ml&#44; IgG 865 mg&#47;ml&#44; and IgA 161 mg&#47;ml&#46; The patient was well and symptoms free&#44; and he refused any invasive exploration&#46; He received treatment with chlorambucil 5 mg&#47;week and despite of this the monoclonal peak &#40;IgM kappa&#41; persisted and the serum IgM level remained elevated&#46; The patient was followed by monthly routine blood examinations&#46; On April 2003&#44; an abdominal CT revealed a mass located in his solitary right kidney&#46; A fine needle aspiration biopsy of the kidney revealed a B clonal kappa&#44; CD19&#43;&#44; CD10&#43; and CD20&#43; cell infiltration&#46; A high-grade MALT lymphoma transformation was considered and the patient was treated simultaneously with CHOP &#40;cyclophosphamide&#44; doxorubicin&#44;<span class="elsevierStyleSup"> </span>vincristine and prednisone&#41; chemotherapy and a course of rituximab&#46; On May 26&#44; a second cycle of CHOP was initiated but he developed severe neutropenia and multiple complications&#44; including sepsis and acute renal failure necessitating hemodialysis&#46; On October 2003&#44; dissemination of the lymphoma occurred and the patient died with multi-organ failure&#46;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">&#160;</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">Discussion</span></p><p class="elsevierStylePara">Extranodal B-cell lymphomas of MALT arise at extranodal sites&#44; are usually associated with chronic inflammation as a result of an infection or autoimmune disorder&#44; and share histologic and immunophenotypic features&#46; At these anatomic sites&#44; MALT lymphomas are commonly associated with inflammatory conditions that predispose to lymphomagenesis&#46; This is best established in the stomach where MALT lymphomas are commonly associated with <span class="elsevierStyleItalic">H&#46;</span><span class="elsevierStyleItalic"> </span><span class="elsevierStyleItalic">pylori </span>infection &#91;1&#93;&#46; The absence of lymphoid tissue in the normal renal parenchyma and the inability to rule out absolutely the presence of microscopic foci of tumor elsewhere&#44; has led to controversy about the existence of primary renal lymphoma as a distinct disease&#46; However&#44; it has been suggested that lymphoma may arise from the renal hilum or from foci of inflammation that attract lymphocytes to the area&#44; such as chronic pyelonephritis &#91;8&#44; 20&#93;&#46; Other related factors contributing to the genesis of renal MALT lymphoma were Sj&#246;gren&#39;s syndrome &#91;4&#93;&#44; IgA nephropathy &#91;7&#93;&#44; membranoproliferative glomerulonephritis &#91;12&#93;&#44; Epstein-Barr virus &#91;10&#93;&#44; actinomycosis &#91;18&#93;&#44; sarcoidosis &#91;15&#93;&#44; systemic lupus erythematosus &#91;17&#93;&#44; and gastric <span class="elsevierStyleItalic">H&#46;</span><span class="elsevierStyleItalic"> </span><span class="elsevierStyleItalic">pylori </span>infection &#91;9&#44; 18&#93;&#46; The presence of concomitant renal cell carcinoma &#91;18&#93;&#44; transitional cell carcinoma &#91;18&#93; or colonic adenocarcinoma &#91;18&#93; no appears to be a contributing factor&#46;</p><p class="elsevierStylePara">To our knowledge&#44; only 30 unequivocal cases&#44; non including the one reported here&#44; of B-cell lymphomas of MALT involving the kidney have been reported in the English literature &#91;4-20&#93; &#40;Table I&#41;&#46; Except a recent report by Garcia et al&#46; &#91;18&#93; of a series of 10 cases the majority were case reports&#46; It is difficult in these patients to tell in which organ the lymphoma originated&#46; Thus&#44; whereas in 5 cases the primary tumor was localized in the salivary glands&#44; the orbit or the gastrointestinal tract and the kidney lesion represented dissemination &#91;6&#44; 7&#44; 8&#44; 18&#93;&#44; in other 2 cases the tumor was simultaneously diagnosed in the kidney and the parotid gland &#91;4&#44; 16&#93;&#44; and in other 3 cases MALT lymphoma involving the kidney was associated to enlarged lymph nodes &#91;10&#44; 17&#44; 18&#93;&#46; In all these cases secondary involvement of the kidney cannot be excluded&#46; Only in 18 cases the tumor could have been originated in the kidney &#91;5&#44; 9&#44; 11-15&#44; 18-20&#93;&#46;</p><p class="elsevierStylePara">MALT lymphomas are usually confined to the sites of origin when diagnosed and are slow to disseminate&#46; If disseminated&#44; have a tendency to involve other mucosal organs&#46;&#160; This explains the prolonged clinical course and the efficacy of surgical excision with no further therapy in some patients &#91;9&#44; 11&#44; 13-15&#44; 18-20&#93;&#46; Thus&#44; in 8 cases the neoplasm involving the kidney did not recur or disseminate following nephrectomy &#91;9&#44; 11&#44; 13-15&#44; 18-20&#93;&#46; In other case there was a remission following nephrectomy and irradiation &#91;5&#93;&#46; In one case with definitive resolution of the lymphoma following to chlorambucil therapy there was disappearance of serum M-component and improvement in symptomatology &#91;7&#93;&#46; In other case with renal actinomycosis there was a remission following to antibiotics &#91;18&#93;&#46;&#160; In other case there was a partial remission at 15 months following nephrectomy and chlorambucil administration &#91;4&#93;&#46; In some cases the tumor had indolent behavior and very slow clinical course&#46; In one case the lymphoma progressed very slowly and the patient remained alive without any treatment for 5 years &#91;8&#93;&#46; Nine patients were alive with no evidence of disease from 9 to 53 months &#91;18&#44; 19&#93;&#46; Three patients&#44; including our case&#44; died of disease from 3 to 13 years after initial diagnosis &#91;6&#44; 10&#93;&#44; 2 of who presented high-grade transformation and dissemination of the lymphoma&#46;</p><p class="elsevierStylePara">The lymphoma cells express monotypic surface immunoglobulins or&#44; to a lesser extent&#44; cytoplasmic immunoglobulins&#44; usually IgM &#91;15&#93;&#46; On the other hand&#44; monoclonal gammopathy is a common phenomenon in patients with MALT lymphoma&#44; most probably due to paraprotein production by the clonal lymphoplasmacytic cells &#91;21-23&#93;&#46; In a study the monoclonal gammopathy corresponded with the light chains detected on biopsy by immunohistochemical on the majority of cases &#91;22&#93;&#46; In 18 of 31 cases of B-cell MALT-type lymphoma of the kidney &#40;including the one reported here&#41; monotypic cytoplasmic immunoglobulins kappa or lambda were present&#46; However&#44; only 3 cases of monoclonal gammopathy IgM&#44; one with the light chain of the lambda type and 2 with kappa&#44; have been reported so far &#91;7&#44; 12&#93;&#46;</p><p class="elsevierStylePara">The pathogenesis of B-cell lymphoma in our case is unknown&#44; as there was no chronic inflammation identified in the kidney&#46; The case reported here is singular as it showed the morphologic&#44; immunologic&#44; and phenotypic features of a B-cell MALT-type lymphoma of the kidney occurring in a patient with a monoclonal gammopathy IgM kappa&#46; Thus&#44; in our case&#44; monoclonal gammopathy corresponded with the light chain detected on kidney by immunohistochemical&#46; On the other hand&#44; the demonstration of bone marrow cells of B-lineage expressing the same monoclonal protein as the tumor suggested bone marrow involvement&#44; even in the absence of identical morphology&#46; In the literature we identified only another case of MALT lymphoma involving the kidney and bone marrow &#91;18&#93;&#46; In addition&#44; at 2 months of the nephrectomy the persistence of the serum M-component was suggestive of incomplete remission&#46; Despite chemotherapy and rituximab treatment&#44; monoclonal gammopathy IgM kappa persisted&#46; Clinical follow-up showed right kidney extension with high-grade transformation&#44; and finally systemic dissemination&#46; Therefore&#44; the presence of monoclonal gammopathy was associated with more advanced disease&#44; with bone marrow involvement and high-grade transformation &#91;23&#93;&#46; Although MALT lymphoma seems to be a relatively benign disease in most patients&#44; the clinical course of our patient clearly demonstrated the malignant potential and the importance of prompt and aggressive treatment&#46; As occurred in our patient&#44; adverse prognosis factors may include presence of monoclonal gammopathy&#44; bone marrow involvement&#44; high tumor burden&#44; high-grade transformation&#44; or dissemination of disease &#91;24&#93;&#46;</p><p class="elsevierStylePara">In summary&#44; this case illustrates that the kidney is among the sites that may be involved by MALT B-cell lymphomas in a primary or secondary fashion&#44; and the need for expanded investigation of the possible relation with systemic involvement&#46; These lymphomas&#44; like other extranodal MALT-type lymphomas&#44; have indolent behaviour and slow clinical course as demonstrated by the available literature&#46; However&#44; in some cases high-grade transformation and&#47;or dissemination of disease occur&#46; The treatment choice should be patient-tailored&#44; taking into account the site&#44; the stage and the clinical characteristics of the individual patient&#46;</p><p class="elsevierStylePara"><a href="grande&#47;10428&#95;18107&#95;6545&#95;en&#95;10428&#95;18107&#95;1935&#95;es&#95;10309&#95;332&#95;1935&#95;en&#95;figure11&#46;jpg" class="elsevierStyleCrossRefs"><img src="10428_18107_6545_en_10428_18107_1935_es_10309_332_1935_en_figure11.jpg" alt="CT showing tumoral growth through the renal cortex of the left kidney&#46;"></img></a></p><p class="elsevierStylePara">Figure 1&#46; CT showing tumoral growth through the renal cortex of the left kidney&#46;</p><p class="elsevierStylePara"><a href="grande&#47;10428&#95;18107&#95;6546&#95;en&#95;10309&#95;332&#95;1936&#95;en&#95;composicion&#46;jpg" class="elsevierStyleCrossRefs"><img src="10428_18107_6546_en_10309_332_1936_en_composicion.jpg"></img></a></p><p class="elsevierStylePara">Figure 2&#46; </p><p class="elsevierStylePara"><a href="10428&#95;18107&#95;6542&#95;en&#95;10428&#95;table&#95;1&#46;doc" class="elsevierStyleCrossRefs">10428&#95;18107&#95;6542&#95;en&#95;10428&#95;table&#95;1&#46;doc</a></p><p class="elsevierStylePara">Table 1&#46; Summary of clinical and pathologic features of B-cell lymphoma of MALT involving the kidney</p>"
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        "resumen" => "<p class="elsevierStylePara">We report a case of low-grade B-cell lymphoma of mucosa-associated lymphoid tissue &#40;MALT&#41; involving the left kidney and simultaneous onset of a monoclonal gammopathy IgM kappa&#46; No predisposing local inflammatory condition was identified&#46; Following left nephrectomy&#44; the renal specimen showed the centrocyte like cells and lymphoid cells in the lymphoepithelial lesions were positive for CD20 and CD79&#945;&#46; The neoplastic cells expressed monotypic cytoplasmic IgM kappa&#46; The demonstration of bone marrow cells of B-lineage expressing the same monoclonal protein as the tumor suggested bone marrow involvement&#44; even in the absence of identical morphology&#46; Despite chemotherapy and rituximab treatment&#44; clinical follow-up showed right kidney extension with high-grade transformation&#44; and finally systemic dissemination&#46; This case illustrates that the kidney is among the sites that may be involved by MALT B-cell lymphomas in a primary or secondary fashion&#44; and the need for expanded investigation of the possible dissemination&#46; We review the literature on this unusual extranodal lymphoma&#46;</p>"
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        "resumen" => "<p class="elsevierStylePara">Se presenta un caso de linfoma de c&#233;lulas B de bajo grado del tejido linfoide asociado a mucosas &#40;MALT&#41;&#44; afectando al ri&#241;&#243;n izquierdo&#44; y comienzo simult&#225;neo de una gammapat&#237;a monoclonal IgM kappa&#46; En este paciente no pudo identificarse ning&#250;n proceso inflamatorio predisponente local&#46; Tras la nefrectom&#237;a izquierda&#44; el esp&#233;cimen renal mostr&#243; c&#233;lulas centrocito-<span class="elsevierStyleItalic">like</span> y c&#233;lulas linfoides en las lesiones linfoepiteliales que fueron positivas para CD20 y CD79 alfa&#46; Las c&#233;lulas neopl&#225;sicas expresaron IgM kappa monot&#237;pica citopl&#225;smica&#46; La demostraci&#243;n de c&#233;lulas de estirpe B de la m&#233;dula &#243;sea expresando la misma prote&#237;na monoclonal que el tumor sugiri&#243; la afectaci&#243;n de la m&#233;dula &#243;sea incluso en ausencia de id&#233;ntica morfolog&#237;a&#46; A pesar del tratamiento con quimioterapia y rituximab&#44; el seguimiento cl&#237;nico demostr&#243; extensi&#243;n al ri&#241;&#243;n derecho&#44; con transformaci&#243;n a linfoma de alto grado y&#44; finalmente&#44; diseminaci&#243;n sist&#233;mica&#46; Este caso ilustra que el ri&#241;&#243;n se encuentra entre las localizaciones que pueden verse afectadas por los linfomas de c&#233;lulas B de tipo MALT&#44; de forma primaria o secundaria&#44; y explica&#160;la necesidad de extender la investigaci&#243;n para detectar su posible diseminaci&#243;n&#46; Se revis&#243; la literatura sobre este infrecuente linfoma extranodal&#46;</p>"
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B-cell lymphoma of MALT involving the kidney and monoclonal gammopathy: a case report with revision of the literature
Linfoma de células B tipo MALT con afectación renal y gammapatía monoclonal: presentación de un caso y revisión de la literatura
, R.. Pecesb, C.. Vega-Cabrerab, C.. Pecesc, A.. Pobesd, M.F.. Fresnoe
b Servicio de Nefrología, Hospital Universitario La Paz, Madrid,
c Área de Tecnología de la Información, SESCAM, Toledo,
d Sección de Nefrología, Hospital de Cabueñes, Gijón, Asturias,
e Servicio de Anatomía Patológica, Hospital Universitario Central de Asturias Oviedo, Asturias,
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    "textoCompleto" => "<p class="elsevierStylePara"><span class="elsevierStyleBold">Introduction</span></p><p class="elsevierStylePara">Isaacson and Wright &#91;1&#93; initially defined malignant lymphoma of the mucosa-associated lymphoid tissue &#40;MALT&#41; in the gastrointestinal tract&#44; and subsequently in the thyroid&#44; lung&#44; and salivary gland&#44; as a neoplastic proliferation of centrocyte-like cells&#44; with or without lymphoplasmacytic cells&#44; usually accompanied by lymphoepithelial lesions and benign-appearing germinal centers &#91;2&#93;&#46; More recently&#44; B-cell lymphomas of MALT arising of a variety of sites have been described&#44; including the breast&#44; orbit&#44; conjunctiva&#44; skin&#44; gallbladder&#44; cervix&#44; larynx&#44; and trachea &#91;2-4&#93;&#46; Urogenital tract is among the various sites involved in MALT lymphoma &#91;3-4&#93;&#46; However&#44; B-cell lymphomas of MALT type involving the kidney are relatively rare &#91;4-20&#93;&#46; The present paper describes the clinical presentation&#44; pathological features and disease course of a patient with MALT B-cell lymphoma involving the kidney and a serum M-component of IgM kappa&#46;</p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">Case report</span></p><p class="elsevierStylePara">A 77-year-old man with a 30 years history of hypertension was referred to our hospital for evaluation of chronic renal failure&#46; Four years before the patient had been diagnosed of Barrett&#39;s esophagus and duodenal ulcer with pyloric stenosis by upper gastrointestinal endoscopy&#46; Esophagus biopsies showed esophagitis with koilocytosis&#46; The biopsy urease test was negative but he received specific treatment to <span class="elsevierStyleItalic">H&#46; pylori</span> eradication&#46; At that time laboratory data revealed hematocrit 40 &#37;&#44; hemoglobin 12&#46;5 g&#47;dl&#44; white blood cells 5380&#47;ml with normal differential count&#44; and an erythrocyte sedimentation rate of 20 mm&#47;h &#40;normal 15-30 mm&#47;h&#41;&#46; Total serum proteins were 5 g&#47;dl and albumin 3&#46;1 g&#47;dl&#46; Urinalysis was normal and serum creatinine ranged from 1&#46;2 to 1&#46;4 mg&#47;dl&#46; He continued receiving treatment with omeprazole&#44; and sixteen months later a barium study of the upper gastrointestinal tract showed normal findings&#46;</p><p class="elsevierStylePara">At admission physical examination was unremarkable&#46; Laboratory data showed hematocrit 34&#46;5 &#37;&#44; hemoglobin 11 g&#47;dl&#44; white blood cells 5950&#47;ml with normal differential count&#44; platelets 161000&#47;ml&#44; and an erythrocyte sedimentation rate of 100 mm&#47;h&#46; Blood coagulation was normal&#46; Urinalysis revealed normal sediment and proteinuria 1&#46;2 g&#47;24 h&#46; Serum urea was 70 mg&#47;dl&#44; serum creatinine 2&#46;2 mg&#47;dl&#44; and creatinine clearance 45 ml&#47;min&#46; The total serum proteins were 9&#46;6 g&#47;dl&#44; albumin 4&#46;1 g&#47;dl&#44; and gammaglobulin 3&#46;5 g&#47;dl &#40;with a monoclonal peak&#41;&#46; The serum immunoglobulin levels measured by nephelometry were IgM 4640 mg&#47;dl &#40;normal 38-231 mg&#47;dl&#41;&#44; IgG 858 mg&#47;dl &#40;normal 650-1700 mg&#47;dl&#41;&#44; and IgA 168 mg&#47;dl &#40;normal 103-568 mg&#47;dl&#41;&#44; kappa 641 mg&#47;dl &#40;normal 170-370 mg&#47;dl&#41;&#44; lambda 106 mg&#47;dl &#40;normal 90-210 mg&#47;dl&#41;&#46; Monoclonal IgM kappa was detected in the serum by immunofixation&#46; Serum cryoglobulin was negative and Bence-Jones proteinuria was positive&#46; Other laboratory data showed C reactive protein 4&#46;9 mg&#47;dl&#44; beta 2-microglobulin 4&#46;9 mg&#47;l&#44; lactate dehydrogenase 286 U&#47;l&#44; serum calcium 9&#46;9 mg&#47;dl&#44; and serum phosphate 3&#46;2 mg&#47;dl&#46; EBV and HCV were negative&#46; Abdominal ultrasound examination showed a solid mass in the left kidney&#46; A skeletal roentgenogram and a bone scan revealed no abnormalities&#46; A thoracoabdominal CT revealed a 7 cm mass located in the midportion of the left kidney &#40;Fig&#46; 1&#41;&#46; There was no evidence of lymphadenopathy&#46; A bone marrow aspirate showed a normocellular patter and revealed 12&#46;5 &#37; atypical plasma cells&#46; Bone marrow flow cytometry showed 3 &#37; polyclonal T lymphocytes and 1 &#37; polyclonal B lymphocytes&#46; The plasma cells were CD38&#43;&#43;&#44; CD138-&#44; CD19&#43;&#44; CD45&#43;&#44; CD56-&#44; CD117-&#44; and monoclonal kappa&#46;</p><p class="elsevierStylePara">On September 2000&#44; a left nephrectomy was performed&#46; The kidney weighed 240 g &#40;including the adrenal gland&#41;&#44; measured 20 x 12 x 8 cm&#44; and contained an 8 x 8 x 2 cm well-circumscribed mass involving the subcortical mid-portion of the kidney&#46; Histologically&#44; there was a lymphoid infiltrate extending diffusely through the pericapsular region and involving the renal cortex&#46; Kidney architecture was effaced by the infiltrate within which occasional residual tubules and glomeruli were evident &#40;Fig 2 A&#41;&#46; In some areas&#44; the infiltrate was diffuse between the reactive lymphoid follicles&#46; Many reactive germinal centers were colonized by neoplastic cell with a residual mantle zone preserved &#40;Fig 2 B&#41;&#46; In other areas&#44; the neoplasm was nodular&#44; composed of reactive lymphoid follicles surrounded by pale zones of small lymphoid cells with pale or clear cytoplasm and slightly irregular nuclear contours &#40;monocytoid and centrocyte-like cells&#41; &#40;Fig 2 C&#41;&#46; In some areas&#44; a prominent plasma cell and plasmacytoid lymphocytes component with occasional intranuclear inclusions &#40;Dutcher bodies&#41; and Russell bodies was observed &#40;Fig 2 D&#41;&#46; The neoplastic cells expressed monotypic cytoplasmic IgM kappa by immunoperoxidase&#46; The neoplastic cells also reacted with the pan-B-cell antibody L26 &#40;CD20&#44; CD79a&#41;&#44; coexpression of CD43 and stain of atypical lymphocytes with bcl-2 consistent with a low-grade B-cell lymphoma of MALT&#46; Lymphoepithelial lesions were occasionally observed with cytokeratin immunostain&#46; In the areas of the kidney not involved by lymphoma&#44; there were histologic signs of nephrosclerosis&#46;</p><p class="elsevierStylePara">&#160;&#160;&#160;&#160;&#160;&#160;&#160;&#160;&#160;&#160;&#160; Two months after nephrectomy the serum creatinine was 3 mg&#47;dl and the erythrocyte sedimentation rate remained elevated to 141 mm&#47;h&#46; The total serum proteins were 8&#46;4 g&#47;dl&#44; albumin 4&#46;1 g&#47;dl&#44; and gammaglobulin 2&#46;4 g&#47;dl with a monoclonal peak&#46; The serum immunoglobulin levels were IgM 2970 mg&#47;ml&#44; IgG 865 mg&#47;ml&#44; and IgA 161 mg&#47;ml&#46; The patient was well and symptoms free&#44; and he refused any invasive exploration&#46; He received treatment with chlorambucil 5 mg&#47;week and despite of this the monoclonal peak &#40;IgM kappa&#41; persisted and the serum IgM level remained elevated&#46; The patient was followed by monthly routine blood examinations&#46; On April 2003&#44; an abdominal CT revealed a mass located in his solitary right kidney&#46; A fine needle aspiration biopsy of the kidney revealed a B clonal kappa&#44; CD19&#43;&#44; CD10&#43; and CD20&#43; cell infiltration&#46; A high-grade MALT lymphoma transformation was considered and the patient was treated simultaneously with CHOP &#40;cyclophosphamide&#44; doxorubicin&#44;<span class="elsevierStyleSup"> </span>vincristine and prednisone&#41; chemotherapy and a course of rituximab&#46; On May 26&#44; a second cycle of CHOP was initiated but he developed severe neutropenia and multiple complications&#44; including sepsis and acute renal failure necessitating hemodialysis&#46; On October 2003&#44; dissemination of the lymphoma occurred and the patient died with multi-organ failure&#46;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">&#160;</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">Discussion</span></p><p class="elsevierStylePara">Extranodal B-cell lymphomas of MALT arise at extranodal sites&#44; are usually associated with chronic inflammation as a result of an infection or autoimmune disorder&#44; and share histologic and immunophenotypic features&#46; At these anatomic sites&#44; MALT lymphomas are commonly associated with inflammatory conditions that predispose to lymphomagenesis&#46; This is best established in the stomach where MALT lymphomas are commonly associated with <span class="elsevierStyleItalic">H&#46;</span><span class="elsevierStyleItalic"> </span><span class="elsevierStyleItalic">pylori </span>infection &#91;1&#93;&#46; The absence of lymphoid tissue in the normal renal parenchyma and the inability to rule out absolutely the presence of microscopic foci of tumor elsewhere&#44; has led to controversy about the existence of primary renal lymphoma as a distinct disease&#46; However&#44; it has been suggested that lymphoma may arise from the renal hilum or from foci of inflammation that attract lymphocytes to the area&#44; such as chronic pyelonephritis &#91;8&#44; 20&#93;&#46; Other related factors contributing to the genesis of renal MALT lymphoma were Sj&#246;gren&#39;s syndrome &#91;4&#93;&#44; IgA nephropathy &#91;7&#93;&#44; membranoproliferative glomerulonephritis &#91;12&#93;&#44; Epstein-Barr virus &#91;10&#93;&#44; actinomycosis &#91;18&#93;&#44; sarcoidosis &#91;15&#93;&#44; systemic lupus erythematosus &#91;17&#93;&#44; and gastric <span class="elsevierStyleItalic">H&#46;</span><span class="elsevierStyleItalic"> </span><span class="elsevierStyleItalic">pylori </span>infection &#91;9&#44; 18&#93;&#46; The presence of concomitant renal cell carcinoma &#91;18&#93;&#44; transitional cell carcinoma &#91;18&#93; or colonic adenocarcinoma &#91;18&#93; no appears to be a contributing factor&#46;</p><p class="elsevierStylePara">To our knowledge&#44; only 30 unequivocal cases&#44; non including the one reported here&#44; of B-cell lymphomas of MALT involving the kidney have been reported in the English literature &#91;4-20&#93; &#40;Table I&#41;&#46; Except a recent report by Garcia et al&#46; &#91;18&#93; of a series of 10 cases the majority were case reports&#46; It is difficult in these patients to tell in which organ the lymphoma originated&#46; Thus&#44; whereas in 5 cases the primary tumor was localized in the salivary glands&#44; the orbit or the gastrointestinal tract and the kidney lesion represented dissemination &#91;6&#44; 7&#44; 8&#44; 18&#93;&#44; in other 2 cases the tumor was simultaneously diagnosed in the kidney and the parotid gland &#91;4&#44; 16&#93;&#44; and in other 3 cases MALT lymphoma involving the kidney was associated to enlarged lymph nodes &#91;10&#44; 17&#44; 18&#93;&#46; In all these cases secondary involvement of the kidney cannot be excluded&#46; Only in 18 cases the tumor could have been originated in the kidney &#91;5&#44; 9&#44; 11-15&#44; 18-20&#93;&#46;</p><p class="elsevierStylePara">MALT lymphomas are usually confined to the sites of origin when diagnosed and are slow to disseminate&#46; If disseminated&#44; have a tendency to involve other mucosal organs&#46;&#160; This explains the prolonged clinical course and the efficacy of surgical excision with no further therapy in some patients &#91;9&#44; 11&#44; 13-15&#44; 18-20&#93;&#46; Thus&#44; in 8 cases the neoplasm involving the kidney did not recur or disseminate following nephrectomy &#91;9&#44; 11&#44; 13-15&#44; 18-20&#93;&#46; In other case there was a remission following nephrectomy and irradiation &#91;5&#93;&#46; In one case with definitive resolution of the lymphoma following to chlorambucil therapy there was disappearance of serum M-component and improvement in symptomatology &#91;7&#93;&#46; In other case with renal actinomycosis there was a remission following to antibiotics &#91;18&#93;&#46;&#160; In other case there was a partial remission at 15 months following nephrectomy and chlorambucil administration &#91;4&#93;&#46; In some cases the tumor had indolent behavior and very slow clinical course&#46; In one case the lymphoma progressed very slowly and the patient remained alive without any treatment for 5 years &#91;8&#93;&#46; Nine patients were alive with no evidence of disease from 9 to 53 months &#91;18&#44; 19&#93;&#46; Three patients&#44; including our case&#44; died of disease from 3 to 13 years after initial diagnosis &#91;6&#44; 10&#93;&#44; 2 of who presented high-grade transformation and dissemination of the lymphoma&#46;</p><p class="elsevierStylePara">The lymphoma cells express monotypic surface immunoglobulins or&#44; to a lesser extent&#44; cytoplasmic immunoglobulins&#44; usually IgM &#91;15&#93;&#46; On the other hand&#44; monoclonal gammopathy is a common phenomenon in patients with MALT lymphoma&#44; most probably due to paraprotein production by the clonal lymphoplasmacytic cells &#91;21-23&#93;&#46; In a study the monoclonal gammopathy corresponded with the light chains detected on biopsy by immunohistochemical on the majority of cases &#91;22&#93;&#46; In 18 of 31 cases of B-cell MALT-type lymphoma of the kidney &#40;including the one reported here&#41; monotypic cytoplasmic immunoglobulins kappa or lambda were present&#46; However&#44; only 3 cases of monoclonal gammopathy IgM&#44; one with the light chain of the lambda type and 2 with kappa&#44; have been reported so far &#91;7&#44; 12&#93;&#46;</p><p class="elsevierStylePara">The pathogenesis of B-cell lymphoma in our case is unknown&#44; as there was no chronic inflammation identified in the kidney&#46; The case reported here is singular as it showed the morphologic&#44; immunologic&#44; and phenotypic features of a B-cell MALT-type lymphoma of the kidney occurring in a patient with a monoclonal gammopathy IgM kappa&#46; Thus&#44; in our case&#44; monoclonal gammopathy corresponded with the light chain detected on kidney by immunohistochemical&#46; On the other hand&#44; the demonstration of bone marrow cells of B-lineage expressing the same monoclonal protein as the tumor suggested bone marrow involvement&#44; even in the absence of identical morphology&#46; In the literature we identified only another case of MALT lymphoma involving the kidney and bone marrow &#91;18&#93;&#46; In addition&#44; at 2 months of the nephrectomy the persistence of the serum M-component was suggestive of incomplete remission&#46; Despite chemotherapy and rituximab treatment&#44; monoclonal gammopathy IgM kappa persisted&#46; Clinical follow-up showed right kidney extension with high-grade transformation&#44; and finally systemic dissemination&#46; Therefore&#44; the presence of monoclonal gammopathy was associated with more advanced disease&#44; with bone marrow involvement and high-grade transformation &#91;23&#93;&#46; Although MALT lymphoma seems to be a relatively benign disease in most patients&#44; the clinical course of our patient clearly demonstrated the malignant potential and the importance of prompt and aggressive treatment&#46; As occurred in our patient&#44; adverse prognosis factors may include presence of monoclonal gammopathy&#44; bone marrow involvement&#44; high tumor burden&#44; high-grade transformation&#44; or dissemination of disease &#91;24&#93;&#46;</p><p class="elsevierStylePara">In summary&#44; this case illustrates that the kidney is among the sites that may be involved by MALT B-cell lymphomas in a primary or secondary fashion&#44; and the need for expanded investigation of the possible relation with systemic involvement&#46; These lymphomas&#44; like other extranodal MALT-type lymphomas&#44; have indolent behaviour and slow clinical course as demonstrated by the available literature&#46; However&#44; in some cases high-grade transformation and&#47;or dissemination of disease occur&#46; The treatment choice should be patient-tailored&#44; taking into account the site&#44; the stage and the clinical characteristics of the individual patient&#46;</p><p class="elsevierStylePara"><a href="grande&#47;10428&#95;18107&#95;6545&#95;en&#95;10428&#95;18107&#95;1935&#95;es&#95;10309&#95;332&#95;1935&#95;en&#95;figure11&#46;jpg" class="elsevierStyleCrossRefs"><img src="10428_18107_6545_en_10428_18107_1935_es_10309_332_1935_en_figure11.jpg" alt="CT showing tumoral growth through the renal cortex of the left kidney&#46;"></img></a></p><p class="elsevierStylePara">Figure 1&#46; CT showing tumoral growth through the renal cortex of the left kidney&#46;</p><p class="elsevierStylePara"><a href="grande&#47;10428&#95;18107&#95;6546&#95;en&#95;10309&#95;332&#95;1936&#95;en&#95;composicion&#46;jpg" class="elsevierStyleCrossRefs"><img src="10428_18107_6546_en_10309_332_1936_en_composicion.jpg"></img></a></p><p class="elsevierStylePara">Figure 2&#46; </p><p class="elsevierStylePara"><a href="10428&#95;18107&#95;6542&#95;en&#95;10428&#95;table&#95;1&#46;doc" class="elsevierStyleCrossRefs">10428&#95;18107&#95;6542&#95;en&#95;10428&#95;table&#95;1&#46;doc</a></p><p class="elsevierStylePara">Table 1&#46; Summary of clinical and pathologic features of B-cell lymphoma of MALT involving the kidney</p>"
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        "resumen" => "<p class="elsevierStylePara">We report a case of low-grade B-cell lymphoma of mucosa-associated lymphoid tissue &#40;MALT&#41; involving the left kidney and simultaneous onset of a monoclonal gammopathy IgM kappa&#46; No predisposing local inflammatory condition was identified&#46; Following left nephrectomy&#44; the renal specimen showed the centrocyte like cells and lymphoid cells in the lymphoepithelial lesions were positive for CD20 and CD79&#945;&#46; The neoplastic cells expressed monotypic cytoplasmic IgM kappa&#46; The demonstration of bone marrow cells of B-lineage expressing the same monoclonal protein as the tumor suggested bone marrow involvement&#44; even in the absence of identical morphology&#46; Despite chemotherapy and rituximab treatment&#44; clinical follow-up showed right kidney extension with high-grade transformation&#44; and finally systemic dissemination&#46; This case illustrates that the kidney is among the sites that may be involved by MALT B-cell lymphomas in a primary or secondary fashion&#44; and the need for expanded investigation of the possible dissemination&#46; We review the literature on this unusual extranodal lymphoma&#46;</p>"
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        "resumen" => "<p class="elsevierStylePara">Se presenta un caso de linfoma de c&#233;lulas B de bajo grado del tejido linfoide asociado a mucosas &#40;MALT&#41;&#44; afectando al ri&#241;&#243;n izquierdo&#44; y comienzo simult&#225;neo de una gammapat&#237;a monoclonal IgM kappa&#46; En este paciente no pudo identificarse ning&#250;n proceso inflamatorio predisponente local&#46; Tras la nefrectom&#237;a izquierda&#44; el esp&#233;cimen renal mostr&#243; c&#233;lulas centrocito-<span class="elsevierStyleItalic">like</span> y c&#233;lulas linfoides en las lesiones linfoepiteliales que fueron positivas para CD20 y CD79 alfa&#46; Las c&#233;lulas neopl&#225;sicas expresaron IgM kappa monot&#237;pica citopl&#225;smica&#46; La demostraci&#243;n de c&#233;lulas de estirpe B de la m&#233;dula &#243;sea expresando la misma prote&#237;na monoclonal que el tumor sugiri&#243; la afectaci&#243;n de la m&#233;dula &#243;sea incluso en ausencia de id&#233;ntica morfolog&#237;a&#46; A pesar del tratamiento con quimioterapia y rituximab&#44; el seguimiento cl&#237;nico demostr&#243; extensi&#243;n al ri&#241;&#243;n derecho&#44; con transformaci&#243;n a linfoma de alto grado y&#44; finalmente&#44; diseminaci&#243;n sist&#233;mica&#46; Este caso ilustra que el ri&#241;&#243;n se encuentra entre las localizaciones que pueden verse afectadas por los linfomas de c&#233;lulas B de tipo MALT&#44; de forma primaria o secundaria&#44; y explica&#160;la necesidad de extender la investigaci&#243;n para detectar su posible diseminaci&#243;n&#46; Se revis&#243; la literatura sobre este infrecuente linfoma extranodal&#46;</p>"
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Nefrología (English Edition)
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¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?