Síndrome de hiperviscosidade em paciente com mieloma múltiplo / Hyperviscosity syndrome in multiple myeloma patients

O mieloma múltiplo é uma neoplasia progressiva e incurável de células B, caracterizado pela proliferação desregulada e clonal de plasmócitos na medula óssea. A síndrome de hiperviscosidade é uma das complicações relacionadas às gamopatias monoclonais, sendo considerada emergência oncológica. O objetivo deste estudo foi descrever o quadro clínico de um paciente diagnosticado com mieloma múltiplo que apresentou síndrome de hiperviscosidade, avaliando a prevalência de sinais e sintomas, bem como características fisiopatológicas dessa entidade clínica. Foi revisado o prontuário de um paciente internado na enfermaria da Clínica Médica do Hospital Regional do Cariri (CE) no período de junho a julho de 2018. Além disso, foi realizada revisão de literatura em base de dados (PubMed®) direcionada ao tema proposto. O diagnóstico de mieloma múltiplo foi comprovado por mielograma, sendo prontamente iniciada a corticoterapia e avaliada a resposta clínica após essa terapêutica. Apesar de incomum e menos frequentemente relacionada ao mieloma múltiplo, a síndrome de hiperviscosidade está relacionada a uma grande taxa de mortalidade quando apresenta diagnóstico tardio. A terapia de primeira linha indicada para a síndrome de hiperviscosidade foi a plasmaferese, no entanto, as condições clínicas (instabilidade hemodinâmica) impossibilitaram sua realização. O desfecho deste caso foi o óbito do paciente. Concluiu-se que o diagnóstico precoce e a intervenção terapêutica estão diretamente relacionados à ocorrência de menor incidência de complicações relacionadas ao mieloma múltiplo e à síndrome de hiperviscosidade.

Multiple myeloma is a progressive and incurable B-cell neoplasm characterized by unregulated and clonal proliferation of plasmocytes in the bone marrow. Hyperviscosity syndrome is one of the complications related to monoclonal gammopathies and is considered an oncological emergency. The aim of this study was to describe the clinical condition of a patient diagnosed with multiple myeloma who presented hyperviscosity syndrome, evaluating the prevalence of symptoms and signs, as well as the pathophysiological characteristics of this clinical entity. The medical records of a patient admitted to the Internal Medicine ward of the Hospital Regional do Cariri (CE) from June to July of 2018 were reviewed. In addition, we conducted a literature review in a database (PubMed®) directed to the theme proposed. The diagnosis of multiple myeloma was confirmed by myelogram, and corticosteroid therapy was promptly initiated and the clinical response was evaluated after this therapy. Although uncommon and less frequently related to multiple myeoloma, hyperviscosity syndrome is related to a high mortality rate when diagnosed late. The first line therapy indicated to hyperviscosity syndrome was plasmapheresis; however, the clinical conditions (hemodynamic instability) precluded its performance. The outcome of this case was the patient's death. Thus, it was concluded that early diagnosis and therapeutic intervention are directly related to the occurrence of lower incidence of complications related to multiple myeloma and hyperviscosity syndrome.

Características inmunológicas de los niños infectados por vía vertical con el VIH: estudio de casos y controles / Immunological characteristic of HIV infected children by vertical way: cases and controls study

HIV infection in children conditions a serious immunodeficiency with special characteristic that distinguish it from the adult, causing a global immune deficit. This constitutes a cases and controls study between Cuban paediatric patients infected with HIV by vertical transmission and a control group of supposedly healthy children. The both groups were characterized from the clinical point of view and markers were used for evaluated the immunologic and virologycal state. Clinically 75% of patients present a pattern of precocious progression, from total only two stay asymptomatic. All HIV infected children receive antirretroviral treatment and three of them present values of viral load bigger than 100,000 cp/mL. The immune alterations found in the HIV infected children compared with healthy children were: a cellular immune depletion with diminish counts of lymphocytes subsets of T CD4+, CD16+/CD56 + and CD19+, an increase in subsets of CD3+, CD8+, CD8+/CD38+, CD3+/ CD95+ and a hipergammaglobulinemia to prevalence of immunoglobulin gamma IgG (p < 0.05). On the other hand, they were not significantly differences in the serum levels of both C3 and C4, as well as in the haemolytic activity of the roads classic and it alternates of the complement system. This finding allowed us to a better attention and treatment of paediatric HIV patients.

La infección por el virus de la inmunodeficiencia humana (VIH) en niños condiciona una grave inmunodeficiencia con características especiales que la distinguen del adulto, ocasionando un déficit inmune global. Se realizó un estudio de casos y controles de los pacientes pediátricos cubanos infectados por transmisión vertical con el VIH comparado con niños supuestamente sanos. Ambos grupos se caracterizaron desde el punto de vista clínico y se emplearon marcadores que evaluaron el estado inmunológico y virológico. Clínicamente 75% de los pacientes infectados por VIH presentan un patrón de progresión precoz, y dos se mantienen asintomáticos. A todos los niños infectados se les suministró tratamiento antirretroviral y tres presentan valores de carga viral mayores de 100,000 cp/mL. Las alteraciones inmunes encontradas en los pacientes VIH+ fueron: una inmunodepresión celular con conteos de subpoblaciones linfoides T CD4+, CD16+/CD56 + y CD 19+ disminuidas significativamente con respecto al grupo control (p < 0.05). Además, se encontró un aumento de linfocitos CD3+, CD8+, CD8+/CD38+, CD3+/CD95+ y una hipergammaglobulinemia a predominio de inmunoglobulina gamma IgG en la comparación estadística (p < 0.05). Por otra parte, no se encontraron diferencias significativas en los niveles séricos de C3 y C4, así como en la actividad hemolítica de las vías clásica y alterna del sistema del complemento. Este conocimiento nos permitió sentar pautas para contribuir al manejo y tratamiento de los pacientes pediátricos infectados por VIH.

Significance of serum immunoglobulin levels in conglomeration with trans-sternal phlebography in the phlebography management of breast cancer.

The study was conducted on 63 patients with breast lump and twenty normal healthy females. In benign breast disease, a significant (P < 0.001) rise in serum IgA, significant (P < 0.001) decrease in IgG and no change in IgM levels was seen before operation. A significant decrease (P < 0.001) in serum IgA and significant increase in IgG and IgM was observed post operatively. In carcinoma breast, a significant (P < 0.001) elevation in IgA, IgG and IgM levels was found pre-operatively with a concomitant decrease in serum IgA and IgG and increase in serum IgM levels, post operatively. Trans-sternal phlebography (TSP) carried out with 95.23% success has revealed significant (P < 0.001) change in the staging of carcinoma breast. The increased levels of serum immunoglobulins associated with the patients of carcinoma breast with metastasis has led to conclude that these levels, if punctuated with TSP findings can lead to better assessment of the staging of carcinoma breast and thereby its management.

Elevada concentracion de inmunoglobulinas séricas en distintos estadios clínicos de infección por HIV-1 / Concentration elevated of serum immunoglobulins in different clinical stages of infection for HIV-1

La concentración de IgG, IgA e IgM fue determinada por inmunodifusión radial el suero de 127 pacientes, 77 infectados por HIV-1 (Grupo A), agrupados en distintos estadios clínicos, y 50 no infectados por HIV-1 (Grupo B). Se encontraron diferencias estadísticamente significativas entre los niveles séricos de IgG e IgM (Grupo A), y los del Grupo B. En el contraste, discriminando por estadios clínicos, la concentración de IgG Estadio III y IV (Grupo A), fue mayor que IgG Grupo B, al igual que para IgM Estadio IV fue mayor que IgM Grupo B. Las medias de los niveles de inmunoglobulinas entre estadios clínicos, arrojaron diferencias significativas para las distintas inmunoglobulinas, y, especialmente, para IgA Estadio IV, mayor que IgA Estadio III. Resultó notable el aumento de IgM en el estadio IV, el de mayor compromiso clínico e inmunológico; esto podría indicar una respuesta paradógica remanente a nuevos antígenos. Las concentraciones elevadas de inmunoglobulinas séricas estarían dadas por una respuesta inmune inusual, que no es exclusiva de los linfocitos-B, y que ocurre desde los primeros estadios de infección por HIV-1.

Larva migrans visceral (Toxocariase): relato de um caso com intensa hipergamaglobulinemia / Visceral larva migrans (Toxocariases): report of one case with intense hypergammaglobulinemia

Os autores relatam um caso de paciente com 1 ano e 7 meses, natural e procedente de Säo Paulo, com quadro clínico-laboratorial e anátomopatológico de Larva Migrans Visceral, apresentando intensa hipergamaglobulenemia (até 8,54 g%). Os autores apresentam ainda quadro comparativo clínico-laboratorial entre o presente caso e casos de autores nacionais e um autor chileno