[Systemic mastocytosis: systematic review]. / Mastocitosis sistémica: Revisión sistemática.

Mastocytosis is a hematologic malignance characterized by an abnormal proliferation of mastocytes. In a consensus classification in 2001, it was distinguished between matters limited to skin and systemic matters (70% of osseous involvement and 50% of hepatomegaly). The most typical symptoms are skin lesions and systemic manifestations due to mediators secreted by tumoral cells. They are useful chemotherapy to reduce the tumoral burden and antihistaminic to control systemic manifestations. Interferon is useful in most of systemic and local manifestations, and it is recommended to use prednisona before the use of this medication.

Multiple bone lesions resembling a metastatic origin. An unexpected diagnosis.

Lytic and blastic lesions have been associated to malignant tumours, such as solid cancer (breast cancer, renal cancer, prostate cancer, malignant melanoma or thyroid tumours). Although a mixed pattern with lytic and blastic lesions is due to metastatic tumour, this is not the only possible origin. The following case shows a systematic. This case report shows the number of tests that were made in order to discover the origin of osteolytic and osteoblastic lesions and it is notable that there is not an occult neoplasia on every occasion.

Multiple bone lesions resembling a metastatic origin. An unexpected diagnosis

Lytic and blastic lesions have been associated to malignant tumours, such as solid cancer (breast cancer, renal cancer, prostate cancer, malignant melanoma or thyroid tumours). Although a mixed pattern with lytic and blastic lesions is due to metastatic tumour, this is not the only possible origin. The following case shows a systematic. This case report shows the number of tests that were made in order to discover the origin of osteolytic and osteoblastic lesions and it is notable that there is not an occult neoplasia on every occasion (AU)

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Mastocitosis sistémica. Revisión sistemática / Systemic mastocitosis. Systematic review

La mastocitosis es una enfermedad definida por un crecimiento anormal y por la acumulación de mastocitos. En la clasificación de consenso de la OMS del 2001, se distinguió entre procesos bien limitados a piel (mastocitosis cutánea) o bien acumulados a nivel de otros tejidos: médula ósea y/u otros órganos extracutáneos (mastocitosis sistémica) como huesos, hígado, bazo o ganglios linfáticos (70% afectación ósea, con patrón osteolítico u osteoblástico, seguida de 50% de hepatoesplenomegalia). La sintomatología más común en estos enfermos es la afectación de la piel por urticaria pigmentosa (más frecuente en la infancia) o telangiectasiamacularis pertans (más frecuente en adultos) donde los mastocitos pueden estar recluidos mucho tiempo, con clínica que proviene de sus mediadores, siendo los niveles de triptasa el reflejo de la carga tumoral. El manejo de esta enfermedad se basa en la administración de tratamiento sintomático con antagonistas de histaminas H1 y H2, así como cromoglicato disódico, necesitando terapia citorreductora sólo en las variantes agresivas de mastocitos sistémica (asociadas a mutación del receptor detirosin kinasa 816V) o asociadas a SMP (proliferación de mastocitos e hipereosinofilias asociado a la expresión del gen de fusión FIP1L1-PDGFRA). El interferón tiene un efecto beneficioso sobre los síntomas dermatológicos, hematológicos, gastrointestinales y sistémicos, así como en los esqueléticos, debido a su capacidad de aumentar la densidad ósea y reducirlos episodios dolorosos, siendo beneficioso el tratamiento inicial con prednisona

Mastocitosis is a hematologic malignance characterized by an anormal proliferation of mastocites. In a consess classification in 2001, it was distinguished between matters limited to skin and systemic matters (70% of osseous involvement and 50% of hepatomegaly). The most typical symptoms are skin lesions and systemic manifestations due to mediators secreted by tumoral cells. They are useful chemotherapy to reduce the tumoral burden and antyhystaminic to controlsystemic manifestations. Interpheron is useful in most of systemic and local manifestations, andit is recommended to use prednisona before the use of this medication

Quimioterapia adyuvante en ancianas con cáncer de mama / No disponible

El cáncer de mama es el primer tumor por incidencia en mujeres,y en países desarrollados se asocia cada vez más al envejecimiento.No existe unanimidad en cuanto a los criterios a seguiren el tratamiento de este grupo de la población, y no se disponede evidencia suficiente como para elaborar unas guías estrictas alrespecto. En esta revisión se exponen las tendencias actuales enel tratamiento adyuvante con quimioterapia en ancianas con cáncerde mama, y los problemas que derivan del infratratamiento ode la administración de quimioterapia en pacientes frágiles(AU)

Breast cancer is the most frequent tumor in women. In affluentnations, breast cancer is a disease associated with aging.There is not consensus to administed chemotherapy to oldwomen and there is not enough evidence to make suitableguidelines. In this review they have been explained the actualtrends in adjuvant treatment of breast cancer in older womenand negative consequences derived from an inadequate treatmentin this group of population and the serious toxicity ofchemotherapy when administered in fragile women(AU)

Deposition-associated diseases related with a monoclonal compound.

Up to 3% of adults over 50 years of age show a monoclonal peak values in blood or urine. Findings and prognosis will be distinct in view of the nature of this factor. In B-cell neoplasias (multiple myeloma, Waldeström macroglobulinaemia, chronic myeloid leukaemia and non-Hodgkin lymphoma) the clinical pattern is dominated by the systemic effects produced by the expansion of the malign clone; the monoclonal protein may result in hyperviscosity syndrome or renal damage. On the other hand, there are other less frequent processes called diseases associated to monoclonal components, where the main clinical manifestations and prognosis depend of the biological effects of the monoclonal protein. With reference to this last group, which is the objective of this revision, no bone lesions, anaemia or a greater tendency to infections usually occur when compared with the first group. Even so, there are some cases of interposition between both groups: for instance, type IgM immunoglobulin present in Waldeström macroglobulinaemia may have cold agglutinin activity, and in the case of multiple myeloma, the clone may secrete amyloidogenic light chains.

Deposition-associated diseases related with a monoclonal compound

Up to 3% of adults over 50 years of age show a monoclonal peak values in blood or urine. Findings and prognosis will be distinct in view of the nature of this factor. In B-cell neoplasias (multiple myeloma, Waldeström macroglobulinaemia, chronic myeloid leukaemia and non-Hodgkin lymphoma) the clinical pattern is dominated by the systemic effects produced by the expansion of the malign clone; the monoclonal protein may result in hyperviscosity syndrome or renal damage. On the other hand, there are other less frequent processes called diseases associated to monoclonal components, where the main clinical manifestations and prognosis depend of the biological effects of the monoclonal protein. With reference to this last group, which is the objective of this revision, no bone lesions, anaemia or a greater tendency to infections usually occur when compared with the first group. Even so, there are some cases of interposition between both groups: for instance, type IgM immunoglobulin present in Waldeström macroglobulinaemia may have cold agglutinin activity, and in the case of multiple myeloma, the clone may secrete amyloidogenic light chains (AU)

Síndrome pluriglandular autoinmune. Revisión / Pluriangular autoimmune syndrome. Systematic review

Se han descrito múltiples casos de síndromes autoinmunes que cursan con hipofunción glandular asociada a otras enfermedades, no siempre endocrinológicas, que, en ocasiones, presentan agregación familiar. Se conoce como síndromes poliglandulares autoinmunes (SPA) aquellos en los que coexisten al menos dos insuficiencias de glándulas endocrinas como consecuencia de un mecanismo autoinmune basado en la acción de los autoanticuerpos o los linfocitos T activados frente a distintos antígenos de los órganos diana o de las glándulas endocrinas, o en el bloqueo de la función hormonal. A continuación, se presenta una revisión de los principales síndromes autoinmunes, los criterios diagnósticos de los mismos y los procesos involucrados

There are a lot of autoimmune syndromes with glandular disfunction which are associated to another diseases. Sometimes, these processes are associated to similar cases in the same family. Autoimmune polyglandular syndromes are characterized by the coexistence of two or more endocrine insufficiencies due to an autoimmune mechanism: the activity of auto antibodies or T activated lymphocytes against organs or endocrine glands. In this report, they have been described the main autoimmune syndromes, the diagnostic methods and the mechanisms which take arole in their origin

[Pluriglandular autoimmune syndrome. Systematic review]. / Síndrome pluriglandular autoinmune. Revisión.

There are a lot of autoimmune syndromes with glandular disfunction which are associated to another diseases. Sometimes, these processes are associated to similar cases in the same family. Autoimmune polyglandular syndromes are characterized by the coexistence of two or more endocrine insufficiencies due to an autoimmune mechanism: the activity of autoantibodies or T activated lymphocytes against organs or endocrine glands. In this report, they have been described the main autoimmune syndromes, the diagnostic methods and the mechanisms which take a role in their origin.

Tumor neuroendocrino de mama / No disponible

El tumor neuroendocrino de mama es poco frecuente, mástípico de mujeres con edad avanzada y asociado a un componentemucinoso. No se han publicado series acerca de tratamientoadyuvante en este grupo de pacientes, por lo que noexiste unanimidad en cuanto al empleo de quimioterapia o elesquema a utilizar. Se presenta un caso clínico en una mujerjoven con tumor neuroendocrino de mama que fue tratadacon cirugía, quimioterapia y hormonoterapia adyuvantes, y sehace una revisión de la literatura existente(AU)

Neuroendocrine breast tumor is an unusual breast cancerusually related to advanced age and a mucinous tumoral component.There are no series of adjuvant treatment of this kindof tumor, so that there is no a consensus about the best chemotherapyschedule to use in them. We present a case of ayoung woman with neuroendocrine breast cancer who wastreated with surgery, adjuvant chemoterapy and hormonetherapy.A literature review is made(AU)

Diagnóstico diferencial de las gammapatías monoclonales / Monoclonal gammopathies differential diagnosis

Las aparición de un pico monoclonal en sangre o en orina, es un hallazgo que aparece con bastante frecuencia en la práctica clínica habitual. Son múltiples las patologías que pueden ser responsables de tal dato, y conseguir llegar al diagnóstico definitivo determinará que el tratamiento sea el adecuado y que se modifique el pronóstico del paciente. Asimismo, el descartar una etiología maligna de la gammapatía monoclonal, permitirá evitar pruebas cruentas innecesarias a este grupo de pacientes. En el siguiente manuscrito, se ofrece una descripción de las principales patologías asociadas a picos monoclonales, así como datos que orientarán al diagnóstico definitivo

The presence of a monoclonal pike in blood is very habitual in our daily medical activity. There are a lot of causes of that. If we know which kind of pathology had originated the monoclonal pike, we could be able to bring a suitable treatment and to change the prognosis of the patient. If we are able to exclude a malignant disease, the patient will not suffer unnecessary aggressive tests. In this report, we describe the main diseases which are associated to monoclonal pike and the clues to get a definitive diagnosis

Diagnosis and current treatment of neurological paraneoplastic syndromes

Neurological paraneoplastic syndromes (NPS) affect only 0.01% of cancer patients, chiefly those affected by lung, breast, ovarian and stomach cancer. They frequently cause major disability and produce limitations in patients' daily activities; the character of the disease is irreversible. Clinical suspicion is fundamental for an early diagnosis and it must be backed up by the specification of certain antibodies both present in blood and in cerebrospinal fluid (CSF). Conventional treatments are very inefficient at the time of treating these disorders; at present, the administration of immunoglobulins, immunosuppressors, chemotherapy agents and corticoids are under study, but so far results are not promising. The aim of this review is to analyze the variety of NPS and describe the findings concerning autoimmunity and treatments used at present (AU)

[Monoclonal gammopathies differential diagnosis]. / Diagnóstico diferencial de las gammapatías monoclonales.

The presence of a monoclonal pike in blood is very habitual in our daily medical activity. There are a lot of causes of that. If we know which kind of pathology had originated the monoclonal pike, we could be able to bring a suitable treatment and to change the prognosis of the patient. If we are able to exclude a malignant disease, the patient will not suffer unnecessary aggressive tests. In this report, we describe the main diseases which are associated to monoclonal pike and the clues to get a definitive diagnosis.