[Systemic AA amyloidosis induced by benign neoplasms]. / Amiloidosis secundaria (AA) asociada a tumoraciones benignas.

Amyloidosis is a systemic disorder characterized by the extracellular tissue deposition of insoluble, toxic aggregates in bundles of beta-sheet fibrillar proteins. These deposits are typically identified on the bases of their apple-green birrefringence under a polarized light microscope after staining with Congo red, and by the presence of rigid, nonbranching fibrils 8 to 10 nm in diameter on electron microscopy. The type of amyloid fibril unit can be further defined by immunohistology or by immunoelectron microscopy. It has been described at least 25 different human protein precursors of amyloid fibrils, which will describe its corresponding amyloid disease. The most common types of amyloidosis are AL (primary) and AA (secondary) types; the former, is the most frequent and is due to deposition of proteins derived from immunoglobulin light chain fragments, occurring alone or in association with multiple myeloma. The later (AA), is caused by deposition of fibrils composed of fragments of the acute phase reactant serum amyloid A (SAA) and complicates chronic diseases with ongoing or recurring inflammation, namely; rheumatoid arthritis (RA), juvenile chronic polyarthritis, ankylosing spondylitis, familial periodic fever syndromes (Familial Mediterranean Fever), chronic infections and furthermore, some neoplasms (mainly renal cell carcinoma and Hodgkin's disease). Despite its less frequent association, some benign neoplasms can subsequently complicate to AA amyloidosis, therefore, an early diagnose and successful treatment may lead indeed, to regression of the amyloid disease. Herein, we present two cases of AA amyloidosis, both of them caused by 2 different benign neoplasms: 1. A 34 year-old woman, after chronic oral contraceptive use, developed an hepatic adenoma (fig. 1) which finally lead to AA amyloidosis with primary kidney presentation (pure nephrotic syndrome) (table 1). Post-surgical complications yield to acute renal failure from which unfortunately could not be recovered. After being on hemodialysis therapy during 10 months she received a first renal allograft without any complication. 2. A 20 year old woman, was diagnosed of AA amyloidosis after a renal biopsy (fig. 2) because of nephrotic syndrome (table 1). Further investigation lead to the finding of a hialyne-vascular type Castleman's disease located in the retroperitoneum (fig. 2). Despite surgical resection and medical treatment (colchicine) she developed progressive renal failure requiring initialization of hemodialysis therapy. After 6 years being on hemodialysis, she received a first renal allograft which is currently functioning after one year of follow- up. Although other chronic inflammatory diseases complicate more frequently to AA amyloidosis, benign tumors have to be taken into account as a potential ethiological cause for secondary amyloidosis.

Amiloidosis secundaria (AA) asociada a tumoraciones benignas / Systemic AA amyloidosis induced by benign neoplasms

La amiloidosis se caracteriza por el depósito de proteínas de características ultraestructurales fibrilares, con plegamiento Beta en capas e insolubles, que se depositan mayoritariamente a nivel de los espacios extracelulares de órganos y tejidos. Se clasifica típicamente según la naturaleza bioquímica de la proteína fibrilar, y según su distribución en el organismo podrá ser sistémica o localizada. La amiloidosis sistémica más frecuente en la práctica clínica es la denominada AL (idiopática primaria o asociada a mieloma múltiple) cuyas fibrillas están formadas por cadenas ligeras. En cambio, la amiloidosis AA (secundaria, reactiva o adquirida) es aquella que se desarrolla típicamente como complicación de una enfermedad inflamatoria crónica, destacando entre las más habituales; enfermedades de origen reumatológico (artritis reumatoide, espondilitis anquilopoyética, artritis psoriásica), la fiebre mediterránea familiar, la enfermedad inflamatoria intestinal, así como infecciones cronificadas (tuberculosis, osteomielitis). No obstante, otras causas responsables de su desarrollo y en muchas ocasiones infravaloradas, son las tumoraciones benignas. Algunas de estas entidades, también tendrán capacidad de actuar como estímulo responsable de la formación de estas proteínas, que finalmente se depositarán en diferentes tejidos del organismo. Es importante resaltar, que el diagnóstico precoz así como el tratamiento eficaz de la enfermedad subyacente ha permitido disminuir su incidencia, así como en algunos casos incluso revertirla. Aquí, presentamos dos casos clínicos paradigmáticos de tumoraciones benignas, adenoma hepático y Enfermedad de Castlemann, que desarrollaron posteriormente amiloidosis AA con afectación renal principalmente en forma de síndrome Nefrótico (AU)

Amyolidosis is a systemic disorder characterized by the extracellular tissue deposition of insoluble, toxic aggregates in bundles of Beta-sheet fibrillar proteins. These deposits are typically identified on the bases of their apple-green birrefringence under a polarized light microscope after staining with Congo red, and by the presence of rigid, non branching fibrils 8 to 10 nm in diameter on electron microscopy. The type of amyloid fibril unit can be further defined by immunohistology or by immunoelectron microscopy. It has been described at least 25 different human protein precursors of amyloid fibrils, which will describe its corresponding amyloid disease. The most common types of amyloidosis are AL (primary) and AA (secondary) types; the former, is the most frequent and is due to deposition of proteins derived from immunoglobulin light chain fragments, occurring alone or in association with multiple myeloma. The later (AA), is caused by deposition of fibrils composed of fragments of the acute phase reactant serum amyloid A (SAA) and complicates chronic diseases with ongoing or recurring inflammation, namely; rheumatoid arthritis (RA), juvenile chronic polyarthritis, ankylosing spondylitis, familial periodic fever syndromes (Familial Mediterranean Fever), chronic infections and furthermore, some neoplasms (mainly renal cell carcinoma and Hodking¿s disease). Despite its less frequent association, some benign neoplasms can subsequently complicate to AA amyloidosis, therefore, an early diagnose and successful treatment may lead indeed, to regression of the amyloid disease. Herein, we present two cases of AA amyloidosis, both of them caused by 2 different benign neoplasms: 1. A 34 year-old woman, after chronic oral contraceptive use, developed an hepatic adenoma (fig. 1) which finally lead to AA amyloidosis with primary kidney presentation (pure nephrotic syndrome) (table 1). Post-surgical complications yield to acute renal failure from which unfortunately could not be recovered. After being on hemodialysis therapy during 10 months she received a first renal allograft without any complication. 2. A 20 yearold woman, was diagnosed of AA amyloidosis after a renal biopsy (fig. 2) because of nephrotic syndrome (table 1). Further investigation lead to the finding of a hialyne-vascular type Castleman¿s disease located in the retroperotoneum (fig. 2). Despite surgical resection and medical treatment (colchicine) she developed progressive renal failure requiring initialization of hemodialysis therapy. After 6 years being on hemodialysis, she received a first renal allograft which is currently functioning after one year of follow- up. Although other chronic inflamatory diseases complicate more frequently to AA amyloidosis, benign tumors have to be taken into account as a potential ethiological cause for secondary amyloidosis (AU)

[An immunohistochemical study of chronic otitis media]. / Estudio inmunohistoquímico de las otitis medias crónicas.

We use immunohistochemical procedures (OKT6) to detect LCs. We have studied tissue samples from surgical operations in cases of chronic otitis media. The samples were from skin of external ear canal, both the superior wall (close to cholesteatoma) and the inferior wall; we took also samples from the cholesteatoma matrix (epidermal and subepidermal stratum). We found LCs in the skin of external ear canal in similar proportion if the samples were of superior or inferior wall. In cholesteatoma tissue (17 cases) we observed LCs in the epidermal stratum in greater proportion than in skin. Sometimes the LCs form aggregates or "clusters".

[Uremic hemolytic syndrome in the adult. A study of four cases (author's transl)]. / Síndrome hemolítico urémico del adulto. Estudio de cuatro casos.

Four cases of the uremic-hemolytic syndrome in the adult are presented. One of them was associated with the presence of the Australia antigen in the plasma. Another had recurrent and familial characteristics associated with the ingestion of oral contraceptives and exhibited a persistent activation of the C3 fraction and the presence of C3NeF (nephritic factor). The two remaining cases were associated with the post partum. Three patients have received sodium heparin continuously, two of which (post partum uremic-hemolytic syndrome) recovered the renal function. A third case, with recurrent uremic-hemolytic syndrome, presented an improvement of the renal function under heparin treatment after the first episode of the disease, but not after the second. Two patients are included in a program of periodic hemodyalisis. The mechanisms by which infections, ingestion of oral contraceptives or post partum can produce an intravascular coagulation syndrome or immunitary disorders are analyzed, both phenomena being interrelated and apparently playing an important role in the pathogenesis of the uremic-hemolytic syndrome.

PIP: 4 cases of the uremic-hemolytic syndrome in the adult are presented. 1 of them was associated with the presence of the Australia antigen in the plasma. Another had recurrent and familial characteristics associated with the ingestion of oral contraceptives (OCs) and exhibited a persistent activation of the C3 fraction and the presence of C3NeF (nephritic factor). The 2 remaining cases were associated with the post partum. 3 patients have received sodium heparin continuously, 2 of which (post partum uremic-hemolytic syndrome) recovered the renal function. A 3rd case, with recurrent uremic-hemolytic syndrome, presented an improvement of the renal function under heparin treatment after the 1st episode of the disease, but not after the 2nd. 2 patients are included in a program of periodic hemodyalisis. The mechanisms by which infections, ingestion of OCs or post partum can produce an intravascular coagulation syndrome or immunitary disorders are analyzed, both phenomena being interrelated and apparently playing an important role in the pathogenesis of the uremic-hemolytic syndrome. (Author's modified)

[Zollinger-Ellison syndrome type II due to diffuse hyperplasia of the pancreatic islet cells (author's transl)]. / Síndrome de Zollinger-Ellison tipo II por hiperplasia difusa de los isoltes pancreáticos.

A 58-year-old patient with hypergastrinemia (basal and after stimulation by means of protein food, calcium, glucagon, and secretin), acid hypersecretion, recurrent anastomotic ulcer, gastrocolonic fistula, steatorrhea, and malabsortion (hypocalcemia, hypocholesterolemia and a rather elevated 5-HIAA) is reported. The definite preoperative diagnosis of Zollinger-Ellison syndrome was established after the intravenous secretin test (75 U) which produced a significant stimulation peak 5 minutes after being injected. The possible existence of a multiple endocrine adenomatosis syndrome type I was discarded. During the operation no pancreatic or extrapancreatic macroscopic tumor was found. A total gastrectomy, transverse colectomy, splenectomy, and subtotal pancreatic resection were performed; Rosanow's techniques was used to re-established the gastrointestinal continuity. The morphological study of the excised pancreatic tissue showed a diffuse hyperplasia of the Langerhans islet cells; indirect immunofluorescence in the presence of antigastrin antibodies was faintly positive and difficult to evaluate. However, gastrin levels clearly decrease after the operation may be because the inhibitory effect of total gastrectomy or because of the partial pancreatectomy. Furthermore, the inhibitory effect of tyrocalcitonine onthe pre- and postoperative gastrin levels measured by radioimmunoassay could be verified. For the moment the importance of this test in the diagnosis of Zollinger-Ellison syndrome, and especially in the diagnosis of ZES-type II, is not known.