Genetic diversity and phylogeographic analysis of human herpesvirus type 8 (HHV-8) in two distant regions of Argentina: Association with the genetic ancestry of the population.

BACKGROUND: The genetic diversity of persistent infectious agents, such as HHV-8, correlates closely with the migration of modern humans out of East Africa which makes them useful to trace human migrations. However, there is scarce data about the evolutionary history of HHV-8 particularly in multiethnic Latin American populations. OBJECTIVES: The aims of this study were to characterize the genetic diversity and the phylogeography of HHV-8 in two distant geographic regions of Argentina, and to establish potential associations with pathogenic conditions and the genetic ancestry of the population. STUDY DESIGN: A total of 101 HIV-1 infected subjects, 93 Kaposi's Sarcoma (KS) patients and 411 blood donors were recruited in the metropolitan (MET) and north-western regions of Argentina (NWA). HHV-8 DNA was detected by ORF-26 PCR in whole blood, saliva and FFPE tissues. Then, ORF-26 and ORF-K1 were analyzed for subtype assignment. Mitochondrial DNA and Y chromosome haplogroups, as well as autosomal ancestry markers were evaluated in samples in which subtypes could be assigned. Phylogeographic analysis was performed in the ORF-K1 sequences from this study combined with 388 GenBank sequences. RESULTS: HHV-8 was detected in 50.7%, 59.2% and 8% of samples from HIV-1 infected subjects, KS patients and blood donors, respectively. ORF-K1 phylogenetic analyses showed that subtypes A (A1-A5), B1, C (C1-C3) and F were present in 46.9%, 6.25%, 43.75% and 3.1% of cases, respectively. Analyses of ORF-26 fragment revealed that 81.95% of strains were subtypes A/C followed by J, B2, R, and K. The prevalence of subtype J was more commonly observed among KS patients when compared to the other groups. Among KS patients, subtype A/C was more commonly detected in MET whereas subtype J was the most frequent in NWA. Subtypes A/C was significantly associated with Native American maternal haplogroups (p = 0.004), whereas subtype J was related to non-Native American haplogroups (p < 0.0001). Sub-Saharan Africa, Europe and Latin America were the most probable locations from where HHV-8 was introduced to Argentina. CONCLUSIONS: These results give evidence of the geographic circulation of HHV-8 in Argentina, suggest the association of ORF-26 subtype J with KS development and provide new insights about its relationship with ancient and modern human migrations and identify the possible origins of this virus in Argentina.

Evolución inusual de un carcinoma urotelial variante plasmocitoide / Unusual evolution of a plasmacytoid urothelial carcinoma of the bladder

Se comunica el caso de un hombre de 63 años que acudió a la consulta debido a un episodio de hematuria, por lo que se realizó una resección vesical transuretral, con diagnóstico de carcinoma infiltrante. Se llevó a cabo una cistectomía radical. En la pieza se identificó un tumor ulcerovegetante en la base de la vejiga. La microscopia mostró una infiltración de células malignas de aspecto plasmocitoide, positivas para CK7 y negativas para CK20 y CD138. El diagnóstico fue de carcinoma urotelial plasmocitoide. Tres meses después el paciente reingresó por taponamiento cardíaco. El examen citológico del líquido pericárdico demostró células similares a las de su carcinoma primario. El paciente murió 6 meses después del diagnóstico. El carcinoma urotelial representa el 90% de los carcinomas vesicales. Teniendo en cuenta sus variantes histopatológicas la forma plasmocitoide es inusual. Sus signos y síntomas no se presentan hasta que la enfermedad ha progresado a una etapa avanzada, lo que conlleva un mal pronóstico y curso agresivo. La histología es característica: las células presentan aspecto plasmocitoide y son por lo general CK7 y CK20 positivas. Hasta un 94% pueden ser positivas para CD138. Los principales diagnósticos diferenciales son plasmocitoma y linfoma con diferenciación plasmocitoide. La afectación cardiovascular es infrecuente. El carcinoma urotelial plasmocitoide es una entidad histopatológica que es difícil de diagnosticar debido su baja frecuencia, requiere el diagnóstico diferencial con otras enfermedades y su comportamiento es agresivo y atípico (AU)

A 63 year old man presented with hematuria and underwent a transurethral resection of the bladder. A diagnosis of muscle invasive carcinoma was made and a radical cystectomy was performed. An ulcerating vegetative tumour was present in the base of the bladder. Microscopy revealed infiltration by atypical cells with plasmacytoid appearance and which were positive for CK7 and negative for CK20 and CD138. The final diagnosis was plasmacytoid urothelial carcinoma. Three months later the patient was readmitted with a cardiac tamponade. Cytology of the pericardial fluid revealed cells similar to those of the primary carcinoma. He died six months after initial diagnosis. Urothelial carcinoma represents 90% of all bladder carcinomas and the plasmacytoid form is an unusual variant which only becomes clinically manifest when it reaches an advanced stage and behaves aggressively with a poor prognosis. It has a characteristic histology with cells with plasmacytoid appearance which are usually positive for CK7 and CK20. Up to 94% can be positive for CD138. The main differential diagnoses are plasmacytoma and plasmacytoid lymphoma. Cardiovascular involvement is unusual. Plasmacytoid urothelial carcinoma poses diagnostic problems due to its rarity. Careful differential diagnosis should be made. Its behavior is aggressive and atypical (AU)

Expression of VEGF-A, HIF-1 A, CD34 and Ki67 in clear cell renal cell carcinomas and their relationship with conventional prognostic markers.

Clear cell renal carcinoma is the most frequent type of renal carcinoma. Recently, attention has been focused in the expression of angiogenic factors by these tumors, which would justify in part their capacity to grow, invade and disseminate, stating a worse evolution of those patients with an unfavorable angiogenic profile. 83 samples of nephrectomy with a diagnosis of clear cell renal cell carcinoma were studied. Clinical and pathological data were collected. Tumors were studied to assess immunohistochemical expression of the following markers: VEGF-A, HIF-1α, CD34 and Ki67. Results indicated a direct linear relationship between expressions of these four markers. Besides, the expression of HIF-1α was directly related to Furhman grade, invasion of the renal vein and tumor stage. Likewise, tumor proliferation index, assessed with Ki67, was directly related to the presence of necrosis, capsular invasion and advanced tumor stage. Regarding the expression of CD34, vascular density was inversely related to tumor necrosis and overall survival. These findings are controversial compared with the available literature. Then, a research scenery would be open, where the importance of generating prospective and more standardized studies are highlighted to determine the role of these angiogenic factors in tumor evolution and prognostic evaluation of these tumors.

El carcinoma renal de células claras es la variante más frecuente de carcinoma renal. En los últimos años, la atención se ha enfocado en la expresión de factores angiogénicos por estos tumores, lo que justificaría en parte su capacidad de crecer, invadir y diseminarse, determinando una peor evolución de aquellos pacientes con un perfil angiogénico desfavorable. Se estudiaron 83 piezas de nefrectomía con diagnóstico de carcinoma renal de células claras. Se recolectaron datos clínicos y patológicos. Los tumores fueron estudiados para evaluar la expresión inmunohistoquímica de los siguientes marcadores: VEGF-A, HIF-1α, CD34 y Ki67. Los resultados indicaron una relación lineal directa entre la expresión de estos cuatro marcadores. Además, la expresión de HIF-1α se encontraba directamente relacionada con el grado de Furhman, la invasión de la vena renal y el estadio tumoral. Asimismo, el índice de proliferación tumoral, evaluado con Ki67, se hallaba directamente relacionado con la presencia de necrosis, la invasión capsular y el estadio tumoral avanzado. Con respecto a la expresión de CD34, mientras mayor es la densidad vascular, menor es la necrosis tumoral y menor la sobrevida global. Los hallazgos resultan controvertidos en comparación con la literatura disponible. Se abriría, entonces, un escenario de investigación donde se destaca la importancia de generar estudios prospectivos y más estandarizados para determinar el rol que cumplen estos factores angiogénicos en la evolución tumoral y la posibilidad de estandarizar resultados que permitan un mejor estudio diagnóstico y pronóstico de estos tumores.

Pancreatic plasmacytoma with biliary obstruction as a manifestation of multiple myeloma relapse

No disponible

Clinicopathological features of aggressive B-cell lymphomas including B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell and Burkitt lymphomas: a study of 44 patients from Argentina.

Aggressive B-cell lymphomas incorporate a wide spectrum of lymphomas that pose challenges in diagnosis as well as treatment. We evaluated the clinicopathological features of 44 patients with aggressive B-cell lymphomas which were classified into 3 groups based on the World Health Organization 2008 classification as follows: including 30 cases of diffuse large B-cell lymphoma (DLBCL), 8 cases of Burkitt lymphoma (BL) and 6 cases of B-cell lymphoma, unclassifiable, with features intermediate between Burkitt lymphoma and diffuse large B-cell lymphoma (BCLU). Male predominance was observed in BL and BCLU groups and the mean age varied from 29 years in BL, 61 years in DLBCL and 70 years in BCLU. Patients with BCLU presented at more advanced stages and had a higher international prognostic index. By immunohistochemistry, they shared characteristics of both BL (including more frequent expression of SOX11) and DLBCL. FISH analyses showed three cases with more than one rearrangement: one MYC/BCL2 and two BCL2/BCL6, in addition to which one case with BCL2/IGH translocation and another with MYC rearrangement were also detected. The mean follow-up survival time of BCLU was 6.6 months, which was significantly shorter in comparison to DLBCL (31 months) and BL (30 months), respectively. The importance of recognizing this BCLU group relies on its different clinical course, poor prognosis and shorter survival than DLBCL and BL. An accurate diagnosis is critical for risk stratification and to improve therapeutic approaches and outcomes.

[Unusual presentation of plasma cell myeloma]. / Morfología dual de células de mieloma: inusual presentación de un caso.

A 41 year-old woman consulted because of facial pain. A magnetic resonance imaging showed a mass in the right petrous apex. A biopsy revealed a diffuse proliferation of large atypical cells with plasmablastic appearance, positive for CD138, BCL6, CD56 and p53. The proliferation factor was 80%. Monoclonal kappa light chain expression was observed. Because the unusual clinicopathological features the patient was studied to rule out systemic plasma cell myeloma. Bone scan disclosed multiple cranium osteolytic lesions; proteinogram showed hypogammaglobulinemia and immunofixation in serum and urine were negative. Afterwards, bone marrow biopsy was performed and it presented a 30% infiltration of the bone cylinder by mature plasma cells. These were monoclonal for kappa light chain with focal expression of p53 and without expression of CD56. These findings suggested the diagnosis of multiple myeloma. This case proposes a morphological spectrum of plasma cell neoplasms, showing a continuous clonal evolution of tumor cells, with an acquired plasticity of dedifferentiate, become immature and infiltrate extramedullary tissues, a fact possibly determined by accumulation of multiple genetic alterations. These findings confirm the difficulty of the differential diagnosis from histopathology study between plasmablastic lymphoma and plasmablastic transformation of plasma cell myeloma because of the nearly identical immunohistochemical profiles.

Morfología dual de células de mieloma: inusual presentación de un caso / Unusual presentation of plasma cell myeloma

Una mujer de 41 años consultó por dolor facial. En una resonancia magnética nuclear se observó una masa en el ápex del peñasco derecho. La biopsia mostró una infiltración difusa por células grandes atípicas con morfología plasmablástica, positivas para CD138, BCL6, CD56 y p53, con expresión monoclonal de cadena liviana kappa y factor de proliferación del 80%, planteando el diagnóstico diferencial entre linfoma plasmablástico versus plasmocitoma plasmablástico. Un mapeo óseo evidenció múltiples lesiones osteolíticas en cráneo; el proteinograma reveló hipogamaglobulinemia y la inmunofijación en suero y orina fueron negativas. Se realizó biopsia de médula ósea donde se observó infiltración en un 30% del cilindro óseo por células plasmáticas maduras monoclonales para kappa, con expresión focal de p53 y negativas para CD56. Estos hallazgos confirmaron el diagnóstico de mieloma múltiple. Este caso pone de manifiesto la existencia de un espectro morfológico de las neoplasias de células plasmáticas, mostrando una evolución clonal continua con una plasticidad adquirida para desdiferenciarse, volverse inmaduras e infiltrar tejidos extramedulares, posiblemente debido a acumulación de alteraciones moleculares. Por lo tanto, se evidencia la dificultad del diagnóstico diferencial histopatológico entre linfoma plasmablástico y transformación plasmablástica de mieloma múltiple, debido a sus perfiles inmunohistoquímicos casi idénticos.

A 41 year-old woman consulted because of facial pain. A magnetic resonance imaging showed a mass in the right petrous apex. A biopsy revealed a diffuse proliferation of large atypical cells with plasmablastic appearance, positive for CD138, BCL6, CD56 and p53. The proliferation factor was 80%. Monoclonal kappa light chain expression was observed. Because the unusual clinicopathological features the patient was studied to rule out systemic plasma cell myeloma. Bone scan disclosed multiple cranium osteolytic lesions; proteinogram showed hypogammaglobulinemia and immunofixation in serum and urine were negative. Afterwards, bone marrow biopsy was performed and it presented a 30% infiltration of the bone cylinder by mature plasma cells. These were monoclonal for kappa light chain with focal expression of p53 and without expression of CD56. These findings suggested the diagnosis of multiple myeloma. This case proposes a morphological spectrum of plasma cell neoplasms, showing a continuous clonal evolution of tumor cells, with an acquired plasticity of dedifferentiate, become immature and infiltrate extramedullary tissues, a fact possibly determined by accumulation of multiple genetic alterations. These findings confirm the difficulty of the differential diagnosis from histopathology study between plasmablastic lymphoma and plasmablastic transformation of plasma cell myeloma because of the nearly identical immunohistochemical profiles.

[Metastatic cardiac tamponade as initial manifestation of papillary thyroid carcinoma]. / Taponamiento cardíaco secundario a carcinoma papilar esclerosante difuso de tiroides.

Papillary carcinoma, diffuse sclerosing variant corresponds to 2% of all papillary thyroid carcinomas. It is usually diffuse and bilateral, affecting the entire gland. At the time of diagnosis, patients present lymph node and lung metastasis. It affects mainly young women. This case report describes a cardiac tamponade as the initial manifestation of an unusual variant of papillary thyroid carcinoma. A 32 year-old woman was attended at the emergency room with epigastric pain and dry cough. Physical examination revealed hypotension, tachycardia and decreased heart sounds. An echocardiogram confirmed severe pericardial effusion. Pericardial fluid cytology was positive for malignancy. The patient evolved with recurrent pericardial effusion and a pleuropericardial window was performed. At this procedure, a subpleural nodular lesion was found, which histology corresponded to metastases of papillary carcinoma, probably from thyroid origin. Total thyroidectomy was performed. The final diagnosis was papillary carcinoma, diffuse sclerosing variant. This variant infiltrates the connective tissue of the interfollicular spaces, mimicking thyroiditis and it is associated with early vascular permeation. This tumor, compared to the classic variants of thyroid carcinoma, is more aggressive and it has higher risk of recurrence. Papillary thyroid carcinoma should be considered as differential diagnosis in our population, in all metastatic papillary lesions, and even more in young female patients.

Taponamiento cardíaco secundario a carcinoma papilar esclerosante difuso de tiroides / Metastatic cardiac tamponade as initial manifestation of papillary thyroid carcinoma

El carcinoma papilar, variante esclerosante difusa, corresponde al 2% de todos los carcinomas papilares de la tiroides. Se caracteriza por comprometer de manera difusa y bilateral a la glándula tiroides. Clínicamente se manifiesta con metástasis ganglionares y pulmonares, afectando predominantemente a mujeres jóvenes. Se describe un caso de taponamiento cardíaco como presentación inicial de un carcinoma papilar de tiroides variante esclerosante difusa. Una mujer de 32 años concurrió al servicio de emergencias médicas refiriendo epigastralgia y tos seca. Durante el examen físico se constató hipotensión arterial, taquicardia y ruidos cardíacos disminuidos. Se realizó un ecocardiograma, observándose derrame pericárdico. Por medio de una pericardiocentesis se obtuvo líquido pericárdico, cuyo análisis mostró células neoplásicas. Durante la evolución la paciente presentó recurrencia del derrame pericárdico por lo que se realizó una ventana pleuropericárdica, detectándose durante la cirugía una lesión nodular subpleural, la cual fue biopsiada e informada posteriormente como una metástasis de carcinoma papilar vinculable a origen tiroideo. Se realizó una tiroidectomía total con linfadenectomía cervical bilateral. El diagnóstico final fue carcinoma papilar, variante esclerosante difusa. Esta variante infiltra el tejido conectivo de los espacios interfoliculares, simulando una tiroiditis y se caracteriza por una permeación vascular temprana. En oposición a la variante clásica, la esclerosante difusa presenta mayor agresividad y mayor tasa de recurrencia. El carcinoma papilar de tiroides debe tenerse presente como diagnóstico diferencial en nuestro medio, en todas aquellas lesiones neoplásicas papilares metastásicas, más aún si se trata de mujeres jóvenes.

Papillary carcinoma, diffuse sclerosing variant corresponds to 2% of all papillary thyroid carcinomas. It is usually diffuse and bilateral, affecting the entire gland. At the time of diagnosis, patients present lymph node and lung metastasis. It affects mainly young women. This case report describes a cardiac tamponade as the initial manifestation of an unusual variant of papillary thyroid carcinoma. A 32 year-old woman was attended at the emergency room with epigastric pain and dry cough. Physical examination revealed hypotension, tachycardia and decreased heart sounds. An echocardiogram confirmed severe pericardial effusion. Pericardial fluid cytology was positive for malignancy. The patient evolved with recurrent pericardial effusion and a pleuropericardial window was performed. At this procedure, a subpleural nodular lesion was found, which histology corresponded to metastases of papillary carcinoma, probably from thyroid origin. Total thyroidectomy was performed. The final diagnosis was papillary carcinoma, diffuse sclerosing variant. This variant infiltrates the connective tissue of the interfollicular spaces, mimicking thyroiditis and it is associated with early vascular permeation. This tumor, compared to the classic variants of thyroid carcinoma, is more aggressive and it has higher risk of recurrence. Papillary thyroid carcinoma should be considered as differential diagnosis in our population, in all metastatic papillary lesions, and even more in young female patients.

Relación de la biopsia temporal en el diagnóstico de arteritis de células gigantes y sus manifestaciones clínicas / Relation of the temporal artery biopsy in the diagnosis of gigant cell arteritis and its clinic manifestations

Objetivo: determinar el papel de la biopsia de arteria temporal en el diagnóstico de arteritis temporal así como evaluar la efectividad de los criterios del ACR en el diagnóstico de esta entidad. Materiales y métodos: se realizó un estudio retrospectivo observacional descriptivo. Se revisaron informes de 40 biopsias de arteria temporal recibidas en el servicio de patología del Hospital Privado de Córdoba, Argentina entre 2000 y 2008. El total de biopsias se correlacionó con los hallazgos clínicos y de laboratorio. Resultados: 43% de las biopsias resultaron positivas para arteritis de células gigantes, mientras que 57% restante no cumplió los criterios histológicos. Al aplicar los criterios del Colegio Americano de Reumatología, 65% de los pacientes cumplió los criterios necesarios. De estos 26 individuos, 61% presentó biopsias positivas. De los pacientes que no reunían los criterios americanos, solo uno presentó positividad en la biopsia. Al tomar dichos criterios como parámetro de diagnóstico de la enfermedad y compararlos con la biopsia, cuentan con una sensibilidad de 94% y una especificidad de 56%, un valor predictivo positivo de 61% y un valor predictivo negativo de 93%. Los principales predictores de positividad en la biopsia fueron la claudicación mandibular (OR:6,76), las alteraciones visuales (OR:1,98) y las anomalías en el examen físico de las arterias temporales (OR:2,77). Discusión: el diagnóstico de arteritis de células gigantes surge a partir de la sospecha clínica y no siempre es confirmado por la histopatología. Es importante llegar al mismo debido al riesgo, sobretodo visual, que reviste no iniciar tratamiento con esteroides lo antes posible...

Objectives: to determine the role of temporal artery biopsies in the diagnosis of temporal arteritis and to assess the efficacy of ACR criteria in the recognition of this disease. Material and methods: a retrospective, descriptive, observational study was performed. A total of 40 reports of temporal artery biopsies were reviewed at the Pathology service of our institution between 2000 and 2008. These results were correlated with clinical and laboratory findings. Results: 43% of biopsies were positive for giant cell arteritis, while 57% did not meet histological criteria for giant cell arteritis. By applying the diagnostic criteria of the American College of Rheumatology, 65% of patients met the criteria for giant cell arteritis. Of these 26 individuals, 61% had positive biopsies, while the rest had negative results. From the patients who did not meet American criteria, only one had a positive biopsy. If we take the parameters of the American College of Rheumatology criteria for diagnosing the disease and compared them with the biopsy, we see that they have a sensitivity of 94% and a specificity of 56%; a positive predictive value of 61% and a negative predictive value of 93%. The main predictors of positive biopsy were jaw claudication (OR:6.76), visual disorders (OR:1.98) and abnormalities on physical examination of temporal arteries (OR:2.77). Discusion: giant cell arteritisdiagnosis mainly arises from the clinical suspicion and is not always confirmed by histopathology. It is important to reach its diagnosis because of the risk, primarily visual, that lies in not starting the steroid treatment as early as possible...

[Discrepancies between clinical diagnoses and autopsy findings]. / Discrepancias entre diagnósticos clínicos y hallazgos de autopsia.

The main objective of this study was to identify the clinical-pathological discrepancies in autopsies performed in the institution. We reviewed autopsies of clinical cases in 53 adults in the period between January 2005 and June 2009, carried out in the Pathology Service at the Hospital Privado de Córdoba, Argentina. Six autopsies were excluded due to insufficient information. The Goldman et al. classification was applied to establish clinico-pathologic discrepancies between clinical diagnoses and autopsy findings. Frequently clinical diagnoses were respiratory infections and acute pulmonary embolism. The most frequently reported postmortem diagnoses were respiratory infections and acute myocardial infarction. There were 17 major discrepancies and 30 coincidences. No any minor discrepancy was detected. Respiratory infections were the main cause of error, followed by myocardial infarction. We conclude that in 17/47 (37%) of cases the pre-mortem and postmortem diagnoses were different, and that respiratory infections were the main cause of error. We suggest to adopt educational and informative strategies, to revalue the importance of autopsy, and traditional clinical practices.

Discrepancias entre diagnósticos clínicos y hallazgos de autopsia / Discrepancies between clinical diagnoses and autopsy findings

El objetivo principal del presente trabajo fue identificar las discrepancias clínico-patológicas en las autopsias realizadas en la institución. Se revisaron 53 autopsias de casos clínicos en adultos en el período comprendido entre enero de 2005 y junio de 2009, realizadas en el servicio de Anatomía Patológica del Hospital Privado de Córdoba, Argentina. Se excluyeron seis debido a información insuficiente. Se aplicó la clasificación de Goldman y col. para establecer las discrepancias clínico-patológicas entre los diagnósticos pre y post-mortem. Los diagnósticos clínicos más frecuentes fueron las infecciones respiratorias y el tromboembolismo pulmonar agudo. Los hallazgos de autopsia más frecuentes fueron las infecciones respiratorias y el infarto agudo de miocardio. Se detectaron 17 discrepancias mayores y 30 concordancias, no se detectaron discrepancias menores. Las infecciones respiratorias fueron la principal causa de error, seguidas por el infarto agudo de miocardio. Concluimos que en 17/47 (37%) de los casos se realizó un diagnóstico clínico diferente al post-mortem, y que las infecciones respiratorias constituyen el principal tipo de error. Se sugiere adoptar estrategias informativas y educativas, revalorizar la autopsia y las prácticas clínicas tradicionales.

The main objective of this study was to identify the clinical-pathological discrepancies in autopsies performed in the institution. We reviewed autopsies of clinical cases in 53 adults in the period between January 2005 and June 2009, carried out in the Pathology Service at the Hospital Privado de Córdoba, Argentina. Six autopsies were excluded due to insufficient information. The Goldman et al. classification was applied to establish clinico-pathologic discrepancies between clinical diagnoses and autopsy findings. Frequently clinical diagnoses were respiratory infections and acute pulmonary embolism. The most frequently reported postmortem diagnoses were respiratory infections and acute myocardial infarction. There were 17 major discrepancies and 30 coincidences. No any minor discrepancy was detected. Respiratory infections were the main cause of error, followed by myocardial infarction. We conclude that in 17/47 (37%) of cases the pre-mortem and postmortem diagnoses were different, and that respiratory infections were the main cause of error. We suggest to adopt educational and informative strategies, to revalue the importance of autopsy, and traditional clinical practices.

Anemia hemolítica autoinmune como manifestación inicial de linfoma Hodgkin / Auto inmune hemolytic anemia how initial manifestation of Hodgkin lymphoma

Se presenta el caso de una paciente de 66 años de edad, a la que se le diagnóstica anemia hemolítica, la cual fue refractaria al tratamiento y requirió esplenectomía. Además presenta adenomegalias inguinales, cuya biopsia determina infiltración parcial por células linfoides B CD20+, con atipia, y CD30+, con factor de proliferación alto; en médula ósea se constata incremento de linfocitos T. Cuatro meses después, consulta por la aparición de adenopatías inguinales y axilares, de las cuales la biopsia reveló enfermedad de Hodgkin variante esclerosis nodular, y en médula ósea se evidenció infiltración por la enfermedad linfoproliferativa. Si bien es infrecuente la asociación entre anemia hemolítica y linfoma Hodgkin, debe tenerse en cuenta para llegar a la búsqueda oportuna de su causa y al diagnóstico de un probable proceso linfoproliferativo subyacente.

In this work we report and study a case of 66 year old woman, whit diagnosis of hemolytic anemia, which was refractory to treatment and she required splenectomy. The patient presented inguinal lymphadenopathy which biopsy has determined a partial infiltration of B-cells CD20+ and CD30+ with atypia and high growth factor. The bone marrow biopsy informed an increased number of T lymphocytes. Four month later, the patient complained due to the appearance of inguinal and axillaries lymph nodes, which biopsy revealed a nodular sclerosis Hodgkin lymphoma. The bone marrow biopsy showed infiltration by lymphoproliferative disease. Although the association between hemolytic anemia and Hodgkin lymphoma is less frequent, this fact should be taken into account in searching its cause and reaching the diagnosis of a probable underlying lymphoproliferative process.

[Diffuse plane xanthomatosis associated with haematologic disorder and solid tumor. Findings of an autopsy]. / Xantomatosis plana difusa asociada a tumor hematológico y neoplasia sólida. Hallazgos de una autopsia.

Diffuse plane xanthomatosis associated with haematologic disorder and solid tumor. Findings of an autopsy. Diffuse plane xanthomatosis is an uncommon subtype of non-Langerhans histiocytosis. We report a case of a 78 year-old woman with a history of monoclonal gammopathy of undetermined significance and diffuse plane xanthomatosis since 1995. She consulted for malaise and died four days after admission. Autopsy was requested. Findings were diffuse plane xanthomatosis with extensive involvement of internal organs, lymphoplasmacytic lymphoma and cecal adenocarcinoma. Final event was acute bronchopneumonia. This entity is a subtype of histiocytosis. It affects skin and involves mucous membranes in 40% of the cases. Extracutaneous extension has been reported. It is usually associated with haematologic disorders. In this case, the patient had a lymphoplasmacytic lymphoma and colonic adenocarcinoma. We highlight the importance of recognition its association with monoclonal gammopathies and solid tumors in order to make a proper follow up of patients.

[Colonic metastases of breast infiltrating lobular carcinoma: atypical presentation of a clinical case]. / Carcinomatosis colónica por carcinoma lobulillar de mama: presentación inusual de un caso.

Gastrointestinal metastases are rare. May occur years after initial diagnosis and its symptoms are nonspecific, delaying its correct diagnosis and aggravating its prognosis. The most common histological subtype is lobular breast carcinoma. We present a 75-year-old woman with history of left mastectomy six years ago by infiltrating lobular carcinoma. She was treated with tamoxifen for five years. At present, there was no evidence of disease. She attended the hospital for intestinal subocclusion, being admitted for study. A barium enema revealed multiple strictures of the large bowel and a colonoscopy revealed an impassable stricture in the rectum-sigma. Due to the severity of symptoms, underwent total colectomy. The suspected diagnosis was Crohn's disease. The surgical specimen showed multiple stenosis of the light, with thickened wall and mucosa with granulations. Microscopic examination showed transmural infiltration of colonic wall by malignant cells CK7 positive and ER positive. Breast infiltrating lobular carcinoma has more special tendency to affect the digestive tract, even many years after the diagnosis of the primary tumor. In front of a patient with history of breast cancer and gastrointestinal symptoms, its mandatory to consider gastrointestinal metastases, making differential diagnosis with inflammatory bowel disease, infections or primary tumors, as the therapeutic actions are different.