Recurrent membranoproliferative glomerulonephritis after second renal graft treated with plasmapheresis and rituximab.

We present a case of a 45-year-old man who suffered from idiopatic membranoproliferative glomerulonephritis (MPGN) in the native kidney that relapsed after his first and second renal grafts. The patient was diagnosed in 1990 with lobular MPGN type I, receiving his first renal graft in 1996. In 2001, a biopsy showed recurrence of MPGN type I (rMPGN). He underwent a second renal graft in 2008. In January 2010, he experienced increased proteinuria and creatinine. Upon electron microscopy of a renal graft biopsy we diagnosed a new rMPGN. At the time of the biopsy, complement levels were normal, although C3 and C4 decreased further. We administered 12 plasmapheresis (PP) sessions and four doses of rituximab. Due to persistent renal impairment, we performed a new biopsy 3 months later, showing less severity of the acute lessions. He received a new cycle of treatment (PP+rituximab). One year later, his renal function was stable with a creatinine ranging between 2 and 2.5 mg/dL and a protein/creatinine ratio less than 1 mg/mg. We concluded that the treatment stopped the disease progression.

Marcadores de desdiferenciación podocitaria en un paciente con glomerulonefritis colapsante / No disponible

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[Therapeutic embolization of uterine leiomyomas: cases requiring surgery]. / Embolización terapéutica de leiomiomas uterinos; casos que requirieron cirugía.

OBJECTIVE: To retrospectively analyze the clinical, radiolgical, and histological findings in patients with uterine leiomyomas (LU) that required surgical intervention after embolization. MATERIAL AND METHODS: Between July 1999 and January 2006, we embolized 182 patients with LU. Eight of these patients subsequently required surgical resection of the tumor. We reviewed clinical data, imaging findings, embolization technique, and reasons for surgery, histological findings in the resected specimens, identification and location of the embolizing material, presence and type of necrosis in the LU, and associated pathology in adjacent organs. RESULTS: The 8 patients that required surgery represented 4.3% of all patients embolized for LU. Surgery was necessary due to technical failure in two patients (1.02%), complications in three (1.64%) and failed treatment in the remaining three 3 (1.64%). The mean maximum diameter of the LU was 9.8 cm (range: 4-17 cm). The mean volume of the LU was 491.88 ml (range: 30-1.365 ml) The mean age of the patients was 37.7 years (range: 28-48 years). Global necrosis was evident in 6 LU; necrosis was hyaline type in 3 and inflammatory in the remaining 3. The embolizing material was detected in the LU in one case, in the uterine myometrium in two cases, and in the ovary in one. CONCLUSION: Less than 5% of cases of LU required surgery after embolization; the risk of surgery after embolization was greater in large lesions. When the embolization technique was adequate, histological study confirmed global necrosis of the tumors, although this was not accompanied by clinical improvement.

Embolización terapéutica de leiomiomas uterinos; casos que requirieron cirugía / Therapeutic embolization of uterine leiomyomas: cases requiring surgery

Objetivo. Análisis retrospectivo clínico, radiológico y patológico de pacientes con leiomiomas uterinos (LU) que requirieron intervención quirúrgica tras el tratamiento con embolización. Material y métodos. Desde julio de 1999 hasta enero de 2006 se embolizaron 182 pacientes con LU. Del total de ellas, 8 precisaron intervención quirúrgica con extirpación del tumor. Revisamos retrospectivamente, en estas 8 pacientes, datos clínicos, hallazgos de pruebas por imagen, técnica de embolización y causas de la cirugía, hallazgos histológicos en las piezas extirpadas, identificación y localización del material embolizante (ME), presencia y tipo de necrosis en los LU y patología asociada en órganos adyacentes. Resultados. Las 8 pacientes intervenidas representan un 4,3% del total de pacientes embolizadas. La causa fue por fallo técnico en dos (1,02%), complicaciones en tres (1,64%) y fallo en el tratamiento en otras tres (1,64%). La media de los diámetros máximos de los LU fue de 9,8 cm (rango: 4-17 cm). El volumen medio de los LU fue de 491,88 ml (rango: 30-1,365 ml). La edad media de las pacientes fue de 37,7 años (rango: 28-48 años). Histológicamente se evidenció necrosis global en 6 de los LU, siendo de tipo hialino en 3 e inflamatorio en los otros 3. El ME se detectó en el LU (un caso), en el miometrio uterino (dos casos) y en un ovario (un caso). Conclusión. Los casos de LU que requieren cirugía posembolización son inferiores al 5%, existiendo mayor riesgo en los de gran tamaño. Cuando la técnica de embolización fue adecuada, el estudio histológico confirmó la necrosis global de los tumores, aunque este hecho no se acompañó de mejoría clínica (AU)

Objective. To retrospectively analyze the clinical, radiolgical, and histological findings in patients with uterine leiomyomas (LU) that required surgical intervention after embolization. Material and methods. Between July 1999 and January 2006, we embolized 182 patients with LU. Eight of these patients subsequently required surgical resection of the tumor. We reviewed clinical data, imaging findings, embolization technique, and reasons for surgery, histological findings in the resected specimens, identification and location of the embolizing material, presence and type of necrosis in the LU, and associated pathology in adjacent organs. Results. The 8 patients that required surgery represented 4.3% of all patients embolized for LU. Surgery was necessary due to technical failure in two patients (1.02%), complications in three (1.64%) and failed treatment in the remaining three 3 (1.64%). The mean maximum diameter of the LU was 9.8 cm (range: 4-17 cm). The mean volume of the LU was 491.88 ml (range: 30-1.365 ml) The mean age of the patients was 37.7 years (range: 28-48 years). Global necrosis was evident in 6 LU; necrosis was hyaline type in 3 and inflammatory in the remaining 3. The embolizing material was detected in the LU in one case, in the uterine myometrium in two cases, and in the ovary in one. Conclusion. Less than 5% of cases of LU required surgery after embolization; the risk of surgery after embolization was greater in large lesions. When the embolization technique was adequate, histological study confirmed global necrosis of the tumors, although this was not accompanied by clinical improvement (AU)

Granulomatous slack skin disease in a child: the outcome.

Granulomatous slack skin syndrome is a rare clinical and pathologic disorder. Only 42 patients have been reported, one of whom we described in 1997--the only child so far reported. We now describe the evolution of this patient and the transformation of the disease into a peripheral T-cell lymphoma, and the complications resulting in the child's death.

Primary primitive neuroectodermal tumour of the urinary bladder: a clinico-pathological study emphasising immunohistochemical, ultrastructural and molecular analyses.

Primary primitive neuroectodermal tumours (PNETs) of the bladder are extremely rare and aggressive neoplasms, and only six examples have been reported in the literature. The case of a 21-year-old woman, who remains disease free 3 years after tumour resection, is reported here. Morphological features were found to correspond to a small round blue cell tumour without rosette formation and with extensive areas of necrosis. Strong expression of CD99, vimentin and CD117 (c-kit), and focal reactivity to cytokeratin and S-100 protein was observed in tumour cells. Ultrastructurally, sparse neurosecretory granules were observed. Diagnosis of PNET was supported by molecular genetic analysis, showing the EWS-FLI-1 fusion transcript type 2 by RT-PCR and EWS gene rearrangement by fluorescence in situ hybridisation. A normal genetically balanced genotype was shown by comparative genomic hybridisation, which, together with the expression of c-kit, a known therapeutic target for imatinib, may have prognostic and therapeutic implications.

Large B-cell lymphoma with Hodgkin's features.

AIMS: To describe the features of a series of nine cases of diffuse large B-cell lymphoma (DLBCL) showing morphological and immunophenotypic features that are intermediate with Hodgkin's lymphoma (HL). METHODS AND RESULTS: Most cases (6/9) presented as mediastinal tumours affecting young males, while the other three cases arose in extramediastinal locations. Histopathologically, tumours showed diffuse large cell areas in a polymorphous background, with pleomorphic cytology and the common presence of Hodgkin's and Reed-Sternberg cells. Immunophenotypically, tumours shared features of DLBCL and classical HL, with expression of CD30, CD15 (6/9), and a full B-cell profile including CD45RB, CD20, CD79a and OCT2. Epstein-Barr virus-latent membrane protein expression was found in 2/9 cases. The majority of tumours had immunohistochemical features consistent with activation of the NF-(kappa)B pathway, including nuclear location of the c-REL/p65 subunit, overexpression of phosphorylated I(kappa)B(alpha), and overexpression of NF-(kappa)B targets. Finally, 2/9 cases showed 3q27 (BCL6) rearrangement, and 1/9 had p53 gene mutations, both of which are rarely detected in classical HL. CONCLUSIONS: These findings suggest that DLBCLs with HL features constitute a distinctive subgroup of aggressive lymphomas whose neoplastic growth and peculiar characteristics could be facilitated by a particular microenvironment found in the mediastinum.

Effect of PGE1 on TNF-alpha status and hepatic D-galactosamine-induced apoptosis in rats.

Prostaglandin E1 has hepatoprotective properties in several clinical and experimental models of liver dysfunction. Hepatotoxicity induced by D-galactosamine (D-GalN) is a suitable animal model of human acute hepatic failure. The aim of the study was to investigate if prostaglandin E1 (PGE1) protection against hepatic D-GalN-induced apoptosis was related to tumour necrosis factor-alpha (TNF-alpha) content in serum. This cytokine is associated with in vitro apoptosis and general inflammatory disorders. In this study, PGE1 was administered 30 min before D-GalN to rats. In other experiments, several doses of TNF-alpha were administered 15min after PGE1 to D-Ga1N-treated rats. Several parameters related to apoptosis and necrosis were measured by flow cytometry, gel electrophoresis, biochemical analysis, and optical and electron microscopy. Tumour necrosis factor-alpha was quantified by competitive enzyme-linked immunosorbent assay (ELISA). PGE1 by itself did not modify the cell cycle of hepatocytes and liver toxicity, but increased TNF-alpha in serum in comparison with the control group. D-Galactosamine increased the percentage of hepatocytes in apoptosis and in the S phase of the cell cycle, and decreased those in G0/G1. Such an increase of hepatocytes in apoptosis was correlated with a higher number of apoptotic bodies and DNA fragmentation in liver than control samples. Also, D-GalN increased alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase and TNF-alpha in serum compared with the control group. Pre-administration of PGE1 to D-GalN-treated rats reduced all the parameters of apoptosis and necrosis in liver, and increased additionallyTNF-alpha content in serum. In those experiments where low doses of TNF-alpha were administered to PGE1 and D-GalN-treated rats an inverse relationship appeared between TNF-alpha and ALT content in serum. In conclusion, the protective effects of PGE1 on D-GalN-induced apoptosis may be linked to its capacity to modulate cell division and/or its immunomodulatory activity. In this sense, our experimental results suggest that TNF-alpha could be involved in protection or exacerbation of liver damage in relation to the pathophysiological status of the liver.

Submandibular swelling as the first manifestation of giant cell arteritis. Report of a case.

Giant cell arteritis is a systemic disease with a broad range of clinical signs and symptoms. Although the most frequently involved vessel is the superficial temporal artery, other arteries can be affected. Vasculitic changes in the facial artery usually present as jaw claudication. A report of a case of giant cell arteritis is presented in which facial artery involvement first manifested itself clinically as a submandibular mass. This is a very rare and atypical form of clinical presentation.

[Renal cystic carcinoma: report of five cases]. / Carcinoma renal quístico: presentación de cinco casos.

Of 204 renal adenocarcinomas seen in the Urology Service at the Hospital "12 de Octubre", from January 1974 to December 1992, we found five cases (2.5%) of cystic renal carcinoma in patients with an average age of 51.8 years (range 35-69), with a female vs male ratio of 3:2, and a right-sided vs left sided ratio of 4:1. The article describes the signs and symptoms, diagnostic methods used, treatment and subsequent evolution of our series, and includes a revision of the published literature.

Biochemical basis of a novel T lymphocyte receptor immunodeficiency by immunohistochemistry. A possible CD3 gamma abnormality.

Necropsic lymphoid tissues obtained from an infant with a novel type of immunodeficiency consisting of a peripheral blood T lymphocyte antigen receptor (TCR) surface expression defect, were analyzed by immunohistochemistry for the expression of various TCR-associated epitopes. The work was aimed to characterize the biochemical basis of this kind of disorder and confirm the defect in different lymphoid tissues. Within an assessed lymphoid depletion, the patient's tissues showed a normal expression of several TCR epitopes (those associated to CD3 epsilon, CD3 delta and the clonotypic -Ti- alpha and beta chains). In contrast, the expression of the epitopes recognized by the monoclonals OKT3, WT31, and BMA031 was severely diminished. Our results therefore support that CD3 epsilon, CD3 delta, Ti alpha and Ti beta are probably not involved in this type of immunodeficiency, and strongly suggest that CD3 gamma (forming part of the epitope recognized by OKT3) may rather be the affected chain giving rise to the defective surface T cell phenotype; however, alternative interpretations are not ruled out. The disrupted TCR thus formed, containing Ti alpha beta heterodimers and CD3 epsilon and CD3 delta subunits, but lacking normal CD3 gamma, would in this scheme lack the conformational framework determinants recognized by WT31 and BMA031.

Endometriosis in an inguinal crural hernia. Diagnosis by fine needle aspiration biopsy.

The fine needle aspiration (FNA) biopsy findings of endometriosis is an inguinal crural hernia in a 40-year-old woman are presented. The cytologic findings were similar to those previously reported in aspirates of solid endometriosis in other sites: nonatypical, small, epithelial groups in an inflammatory and proteinaceous background. The cytologic diagnosis of a benign epithelial lesion, possibly endometriosis, was confirmed by histologic study of the extirpated mass. This case shows that endometriosis must be included in the differential diagnosis of FNA samples of palpable lesions of the groin in women of reproductive age.

[Paratesticular liposarcoma of the spermatic cord]. / Liposarcoma paratesticular del cordón espermático.

Funiculus liposarcoma is a very uncommon tumor. Most cases have a low degree of malignancy. Inguinal orchiectomy with a wide local excision is the appropriate treatment. Liposarcoma presurgical diagnosis is very infrequent and ultrasonography is the method that can provide some help to locate it. Currently, chemotherapy indication is unclear. Due to recurrence, specially of local foci, being a possibility a periodical follow-up is necessary in these patients. Nowadays, local recurrences are treated with repeated ample local excisions and radiotherapy. This work reviews the literature and presents a new case of round cells liposarcoma of the funiculus.

[Neoplasm invasion of the renal vein by transitional cell carcinoma of the kidney pelvis]. / Invasión neoplásica de la vena renal por Ca. de células transicionales de pelvis renal.

We present two cases of renal pelvis transitional cell carcinoma with invasion of the renal vein. We emphasize the importance of preoperative diagnosis and review the diagnostic methods available for revealing this uncommon eventuality. Owing to the prognostic importance and subsequent surgical treatment, this possibility should be evaluated in those cases of renal pelvis transitional cell carcinoma with extensive parenchymatous invasion or in those cases in which kidney function cancellation occurs in the excretory urograph.