Pancitopenia em pacientes com anemia perniciosa. Estudo descritivo de 33 casos. / Pancytopenia in patients with pernicious anemia. Descriptive study of 33 cases.

Objetivo: Discriminar as variáveis de sexo e idade nos pacientes com anemia perniciosa; estudar seu perfil hematimétrico; verificar a prevalência de outras doenças autoimunes e anemia perniciosa; analisar a incidência da pancitopenia e sua relação com alterações laboratoriais comum na doença; e avaliar a frequência dos autoanticorpos anticélulas parietais e antifator intrínseco. Métodos: Estudo transversal descritivo, de base clínica e laboratorial, de 33 prontuários de pacientes com anemia perniciosa, diagnosticados em um ambulatório de hospital terciário de atenção à saúde, no período de junho de 2009 a junho de 2014. Para analisar a relação da presença e da ausência de pancitopenia com os níveis da enzima lactato desidrogenase e vitamina B12, foi utilizado o teste qui quadrado. O programa utilizado foi o software Epi Info, versão 7. Resultados: Na amostra, 63,6% eram mulheres, e a idade média geral foi de 47,3 anos. Doenças autoimunes estavam associadas em 30,3% dos pacientes. A pancitopenia esteve presente em 39,4% dos pacientes. Houve significância estatística na relação da pancitopenia com os níveis de enzima lactato desidrogenase (p<0,05). A prevalência do antifator intrínseco foi de 69,7% e dos autoanticorpos anticélulas parietais foi de 72,7%. Conclusão: A pancitopenia mostrou-se um achado significante na população com anemia perniciosa, assim como níveis elevado de LDH, acrescentando a anemia perniciosa como um diagnóstico diferencial de tais alterações laboratoriais.(AU)

Objective: To discriminate the gender and age variables in patients with pernicious anemia; to study erythrocyte profile; to check the prevalence of other autoimmune diseases and pernicious anemia; to analyze the incidence of pancytopenia and its relationship with common laboratory abnormalities in the disease; to evaluate the frequency of anti-gastric parietal cell antibodies, and anti-intrinsic factor antibodies. Methods: Descriptive, cross-sectional study of clinical and laboratorybased medical records of 33 patients with pernicious anemia diagnosed in an outpatient's department of a tertiary healthcare center, in the period between June 2009 and June 2014. To analyze the relationship between the presence and absence of pancytopenia with levels of lactate dehydrogenase enzyme and levels of Vitamin B12 we used the chi-squared test. The software used was Epininfo version 7. Results: The sample showed 63.6% women and 36.4% men with a mean age of 47.3 years. Autoimmune diseases were associated in 30.3% of the patients. Pancytopenia was present in 39.4% of patients. There was statistically significant relationship of pancytopenia with lactate dehydrogenase enzyme levels (p <0.05). The frequency of anti-intrinsic factor antibodies was 69.7%, and the antigastric parietal cell antibodies was 72.7%. Conclusion: Pancytopenia proved to be a significant finding among the population with pernicious anemia and high levels of LDH, which includes pernicious anemia as a differential diagnosis of such laboratory alterations.(AU)

Caso clínico: síndrome poliglandular autoinmune tipo II, forma de presentación en un paciente adulto / Clinical case: autoimmune polyglandular syndrome type II, presentation in adult patient

Autoimmune Polyglandular Syndrome (APS) type II or Schmidt`s Syndrome is diagnosed when a patient has adrenocortical deficiency with type 1 diabetes mellitus, chronic lymphocyticthyroiditis, or Graves’ disease. The disease commonly manifests in the third or fourth decade. We present the case of a 45 yo male patient who manifested simultaneously at the moment of diagnosis, clinical and laboratory features of hypothyroidism, pernicious anemia and Addison disease. We discuss etiologic, clinical and biochemical aspects in type II APS.

Tableau neuro-psychiatrique revelant une maladie de Biermer en l'absence d'anemie. A propos de 3 observations

In addition to an anemic syndrome, pernicious disease can also include neuropsychiatric manifestations. We report here three cases diagnosed as pernicious disease revealed by neuropsychiatric symptoms and even without anemia. Our patients were made of a man and tow women with a mean age of 55 years. They consulted for progressively worsening troubles of step which were related to a combined degeneration of the cord. This degeneration was associated to a cerebellar syndrome in one case. An isolated macrocytosis with a mean MGV at 109 ft, was noticed allowing thus to evoke the Biermer pernicious disease. This diagnosis was confirmed by the marrow puncture which showed a medullar megaloblastosis in two cases and an erythroblastic nucleocytoplosmic maturation's asynchronism. Treated by vitamin B12 the evolution was favorable in two cases with a total neurological recovery after six months in two patients. One patient died after six days of treatment Following an acute myocardial infarction. If faced to symptoms made of a combined degeneration of the spinal card, a periphery neuropathy and/or psychiatric troubles, pernicious disease is a diagnosis that we must evoke even in absence of anemia

Pernicious anemia in a young Bahraini female.

Pernicious anemia (PA) is an autoimmune disorder associated with atrophic gastritis, presence of antibodies to gastric parietal cells and intrinsic factor (IF) and vitamin B2 malabsorption leading to megaloblastic anemia. It has a comparatively higher prevalence in people of North European origin, is uncommon in Arabs and usually affects the elderly. This report, the first from Bahrain, describes a rare case of PA in young female. The presenting symptoms, clinical and laboratory features were similar to those described in classical elderly Caucasian patients. No association with any other autoimmune disease was detected.

Anemia peerniciosa associada a anemia hemolitica auto-imune - relato de um caso / Pernicious anaemia associated to auotimmune haemolytic

Relatamos o caso de uma paciente de 60 anos com anemia perniciosa que, 10 meses apos seu diagnostico, apresentou episodio de anemia hemolitica, com teste de Coombs direto positivo. A anemia perniciosa foi diagnosticada por aspirado e biopsia de medula ossea, biopsia de fundo gastrico e teste terapeutico. Nesta ocasiao, o teste de Coombs direto foi negativo. O episodio de anemia hemolitica foi tratado com corticoide, com subsequente melhora clinica e laboratorial. Um segundo episodio, 4 meses apos o primeiro, tambem foi controlado com corticoterapia. E discutida a associacao entre anemia perniciosa e fenomenos auto imunes. Como evento raro , torna-se de interesse o relato de mais um caso de anemia perniciosa associada a anemia hemolitica auto-imune

Anemias nutricionais / Nutritional anemias

A falta de matéria prima para a biossíntese dos glóbulos vermelhos do sangue leva anemias, destacando-se principalmente a anemia ferropriva e a anemia perniciosa, ambas curáveis pela suplementaçäo alimentar

Mala absorción tropical en Bogotá / Tropical malabsorption in Bogota

Se presentan 75 casos de mala absorción tropical, 65 adultos y 10 niños, estudiados desde el año de 1968 en el Hospital San Juan de Dios de Bogotá. La mayoría de los pacientes provenían de Cundinamarca y del Distrito Especial de Bogotá. En todos hubo mala absorción de la D-xilosa y esteatorrea. La biopsia peroral de la mucosa intestinal practicada en 64, mostró atrofia de 1+ en 16, de 2+ en 30, de 3+ en 18 y en ninguno se observó atrofia de 4+. Se encontró anemia en 58 pacientes, la cual fue de tipo megaloblástico en 45 (60 por ciento), ocasionada a su vez por deficiencia de ácido fólico, en 4, de Vitamina B12 en 9 y de ácido fólico y Vitamina B12 en 32. Fueron hallazgos frecuentes además del peso bajo, la glositis, la carotinemia baja (<40ug/dl) la colesterolemia inferior a 150mg/dl y la albuminemia por debajo de 3.5 g/dl. Fueron tratados exitosamente con dieta balanceada, ácido fólico, Vitamina B12, tetraciclina o sulfas, en forma aislada o empleando combinaciones de estos medicamentos. En presencia de megaloblastosis la terapia ideal es el tratamiento "triconjugado" de Vitamina B12, ácido fólico y tetraciclina; solamente falleció un paciente, por tromboembolismo pulmonar. El diagnóstico diferencial debe hacerse principalmente con la enteropatía por Gluten y con la anemia perniciosa