Patients can develop malignancies due to various reasons including genetic factors, chemical carcinogens, radiation, and defects in their immune system. The immune system is postulated to carry out routine surveillance for malignancy. Patients who have defective immune responses may be susceptible to malignancies due to complicated underlying mechanisms. These include defective immune response to cancer-causing bacteria, transforming viruses, and concomitant molecular, cellular and immunoregulatory defects. Common variable immune deficiency (CVID) is characterized by hypogammaglobulinemia, impaired antibody responses and an increased susceptibility to infections. A disorderly immune response, or immune dysregulation, may also lead to autoimmune complications and possibly to malignancy. The treatment of CVID involves infusion of replacement doses of immunoglobulin, either intravenously (IGIV) or subcutaneously (SCIG). However, it is unclear whether adequate replacement of immunoglobulins is sufficient to prevent the increased risk of malignancy seen in this disease. We present two cases of unusual solid tumors complicating CVID treated with adequate doses of intravenous immunoglobulins. In this study we review the occurrence of malignancy in patients with CVID and postulate mechanisms that may be involved indigent to this disease. We will also review the role of replacement immunoglobulin and discuss cancer screening in these high risk individuals.

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior / This doctoral thesis involved the study of diseases in pigs, including the description of a case of granulocytic sarcoma and outbreaks of eosinophilic salt poisoning. The study of the characteristics and frequency of disease in swine population in the region covered by the Laboratório de Patologia Veterinária (LPV), Universidade Federal de Santa Maria (UFSM) was conducted through a retrospective study of all autopsy diagnoses. Five hundre and sity four with conclusive diagnoses were set apart. The pig population in the region covered by the study is predominantly family and diagnosed diseases reflect this reality. The infectious and parasitic diseases were the most prevalent [384 (68.1%)], followed, in descending order, nutritional and metabolic diseases [64 (11.,3%)], poisoning and toxi-infections [33(5,8%)] and developmental disorders [17(3.1%)]. Other changes to various etiologies, especially trauma, were cause of death in just over 11% of the protocols examined. Bacterial diseases were responsible for more than half of the causes of death or reason for euthanasia of animals studied, pointing to the influence of management, environmental and nutritional regional husbundry . The main disease diagnosed in pigs in the region covered by the study is the edema disease and along with other forms of infection by Escherichia coli is responsible for 23% of deaths of viral diseases and cancer are not major causes of death in pigs in the region studied. Nutritional hepatose and aflatoxicosis are important diseases in the region and and there cuasation are mainly linked to nutritional management. For description of eosinophilic granulocytic sarcoma, a set of techniques (cytology, histology, histochemistry and immunohistochemistry)were used and which proved to be essential for the confirmation of macroscopic tentive diagnosis. Salt poisoning is a condition recognized in pigs as far back as the first half of the last century and still remains as an important cause of mortality; the large amount of salt accumulated in the body in cases of poisoning can be demonstrated by determination of sodium ion in the liver, muscle, CSF, serum and aqueous humor, and these are acillary data to confirm the diagnosis. Pigs affected by salt poisoning have eosinopenia due to severe meningoencephalitis and infiltration of eosinophils in tissues, acute neuronal necrosis which develops into deeplaminar cortical necrosis as the clinical progresses. / Esta tese envolveu o estudo de doenças de suínos, incluindo a descrição de um sarcoma granulocítico eosinofílico e surtos de intoxicação por sal. O estudo das características e frequência das doenças na população suína na região de abrangência do Laboratório de Patologia Veterinária (LPV) da Universidade Federal de Santa Maria (UFSM) foi realizado através de um estudo retrospectivo de todos os diagnósticos de necropsias. Foram separados 564 casos com diagnósticos conclusivos. A população suína na região de abrangência do estudo é predominantemente familiar e as doenças diagnosticadas, refletem esta realidade. As doenças infecciosas e parasitárias foram as mais prevalentes [384 (68,1%)], seguidas, em ordem decrescente, das doenças metabólicas e nutricionais [64 (11,3%)], intoxicações e toxiinfecções [33 (5,8%)], e distúrbios do desenvolvimento [17 (3,1%)]. Outras alterações de diversas etiologias, sobretudo de trauma, foram causa de morte em pouco mais de 11% dos protocolos examinados. Doenças bacterianas são responsáveis por mais da metade das causas de morte ou razão para eutanásia dos suínos estudados, revelando a influência de fatores de manejo, ambientais e nutricionais do modo de vida local. A principal doença diagnosticada em suínos na região de abrangência do estudo é a doença do edema e juntamente com outras formas de infecção por Escherichia coli é responsável por 23% das mortes Doenças de etiologia viral e neoplasias não são importantes causas de morte em suínos na região estudada. Hepatose nutricional e aflatoxicose são importantes doenças da região e se devem, sobretudo, a fatores de manejo nutricional. Para descrição do sarcoma granulocítico eosinofílico, foi utilizado um conjunto de técnicas (citologia, histologia, histoquímica e imuno-histoquímica), indispensáveis para a confirmação da suspeita macroscópica. Intoxicação por sal é uma condição reconhecida em suínos desde a primeira metade do século passado e continua uma importante causa de mortalidade e a grande quantidade de sal acumulada em casos de intoxicação pode ser comprovada pelas dosagens do íon sódio no fígado, músculo, líquor, soro e humor aquoso, e esses dados servem como forma de diagnóstico. Suínos intoxicados por sal apresentam eosinopenia devido a grave infiltração de eosinófilos e a meningoencefalite eosinofílica evolui para uma necrose neuronal laminar, à medida que se desenvolve o curso clínico da doença.