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)

Spontaneous Remission of Pernicious Anemia / 대한내과학회지

Pernicious anemia is a macrocytic anemia that is caused by vitamin B12 deficiency, itself a result of the absence of intrinsic factors due to autoimmune destruction of parietal cells. We report here the case of a 43-year-old female with spontaneous remission of pernicious anemia. The patient presented with fatigue. Her serum vitamin B12 level was low, hemoglobin level was 7.6 g/dL, and serologic tests for anti-intrinsic factor and anti-parietal cell antibodies were positive. We diagnosed her with pernicious anemia, but did not administer vitamin B12 because her hemoglobin level increased spontaneously. Since then, the patient's hemoglobin and serum vitamin B12 levels have been within the normal range.

Polyglandular Syndrome Type III and Severe Peripheral Neuropathy: An Unusual Association.

INTRODUCTION: Polyglandular syndrome is characterized by the association of autoimmune, organ-specific, endocrine and non-endocrine diseases. OBJECTIVE: To present a case of polyglandular syndrome type III (b) accompanied by pernicious anemia and autoimmune thyroiditis. METHOD: Report the clinical case of a young patient that developed progressive and disabling peripheral neuropathy framework, triggered by vitamin B12 deficiency. DISCUSSION: It was proven that atrophic gastritis with positive intrinsic anti-factor was responsible for the framework of pernicious anemia, which in turn dangerously reduced the serum levels of vitamin B12, leading to myelopathy. There was a progressive neurological improvement after parenteral cyanocobalamin replacement, keeping the patient at ambulatory, under maintenance therapy. CONCLUSION: The importance of suspecting on polyglandular syndrome in the presence of autoimmune frameworks is important, especially those involving diabetes, thyroiditis, atrophic gastritis, pernicious anemia, vitiligo, among others.

INTRODUÇÃO: A Síndrome Poliglandular é caracterizada pela associação de doenças autoimune órgão-específicas, endócrinas e não endócrinas. OBJETIVO: Apresentar um caso de Síndrome Poliglandular do tipo III (b) acompanhada de anemia perniciosa e tireoidite auto-imune. MÉTODO: Relata-se o caso clínico de uma paciente jovem que desenvolveu quadro de neuropatia periférica progressiva e incapacitante, desencadeada pela deficiência de vitamina B12. DISCUSSÃO: Ficou comprovado que a gastrite atrófica com anticorpo anti-fator intrínseco positivo foi responsável pelo quadro de anemia perniciosa, que por sua vez reduziu perigosamente os níveis séricos da vitamina B12. Houve melhora progressiva do quadro neurológico após reposição parenteral da cianocobalamina, mantendo-se a paciente, em seguimento ambulatorial, sob terapia de manutenção. CONCLUSÃO: Ressalta-se a importância de se suspeitar da síndrome poliglandular na vigência de quadros auto-imunes, especialmente aqueles envolvendo diabetes, tireoidites, gastrite atrófica, anemia perniciosa, vitiligo, entre outros.

A multicenter retrospective analysis of the clinical features of pernicious anemia in a Korean population.

To determine the approximate incidence and clinical features of pernicious anemia in a Korean population, we retrospectively analyzed clinical data for patients with pernicious anemia who were diagnosed between 1995 and 2010 at five hospitals in Chungnam province. Ninety-seven patients were enrolled, who accounted for 24% of patients with vitamin B(12) deficiency anemia. The approximate annual incidence of pernicious anemia was 0.3 per 100,000. The median age was 66 (range, 32-98) yr, and the male/female ratio was 1.25. Anemia-associated discomfort was the most common symptom (79.4%), followed by gastrointestinal and neurological symptoms (78.4% and 38.1%, respectively). Pancytopenia was found in 36 patients (37.1%), and autoimmune disorders were found in 15 patients (15.5%). Antibody to intrinsic factor was detected in 62 (77.5%) of 80 patients examined, and antibody to parietal cells was detected in 35 (43.2%) of 81 patients examined. Of the 34 patients who underwent tests for Helicobacter pylori, 7 (12.5%) were positive. The anemia-associated and gastrointestinal symptoms resolved completely in all patients after intramuscular injection of cobalamin, whereas neurological symptoms remained in some. In conclusion, pernicious anemia is less frequent in Koreans than in Western populations; however, the clinical features of this disorder in Koreans do not differ from those of Western cases.

A Multicenter Retrospective Analysis of the Clinical Features of Pernicious Anemia in a Korean Population

To determine the approximate incidence and clinical features of pernicious anemia in a Korean population, we retrospectively analyzed clinical data for patients with pernicious anemia who were diagnosed between 1995 and 2010 at five hospitals in Chungnam province. Ninety-seven patients were enrolled, who accounted for 24% of patients with vitamin B12 deficiency anemia. The approximate annual incidence of pernicious anemia was 0.3 per 100,000. The median age was 66 (range, 32-98) yr, and the male/female ratio was 1.25. Anemia-associated discomfort was the most common symptom (79.4%), followed by gastrointestinal and neurological symptoms (78.4% and 38.1%, respectively). Pancytopenia was found in 36 patients (37.1%), and autoimmune disorders were found in 15 patients (15.5%). Antibody to intrinsic factor was detected in 62 (77.5%) of 80 patients examined, and antibody to parietal cells was detected in 35 (43.2%) of 81 patients examined. Of the 34 patients who underwent tests for Helicobacter pylori, 7 (12.5%) were positive. The anemia-associated and gastrointestinal symptoms resolved completely in all patients after intramuscular injection of cobalamin, whereas neurological symptoms remained in some. In conclusion, pernicious anemia is less frequent in Koreans than in Western populations; however, the clinical features of this disorder in Koreans do not differ from those of Western cases.

The Treatment of Pernicious Anemia in Children with Graves' Disease

Organ specific autoimmune diseases may occur in the same individual. Type III polyglandular autoimmune disease is defined by the occurrence in the same individual of two or more of the following: autoimmune thyroid disease, pernicious anemia, insulin dependent diabetes, and other organ specific autoimmune diseases not falling into class I or class II categories. A 16-year-old girl developed pernicious anemia during the treatment of Graves' disease. She was diagnosed with Graves' disease 5 years ago and had received methimazole 20 mg/day, but the medication was not well tolerated. Bone marrow findings were compatible with pernicious anemia as macrocytic normochromic red blood cell (RBC) and increased megakaryocyte. The serum value of vitamin B12 was low, and the serum titer of antibody to gastric parietal cell was high. After diagnosis of pernicious anemia, she had treatment by monthly intramuscular vitamin B12 and methimazole (20 mg/day). The values of hemoglobin and RBC indices as well as thyroid function were normalized after 2 months. Vitamin B12 therapy was maintained for 1 year with normal RBC indices. At present, she dose not receive treatment with vitamin B12 but she does receive methimazole treatment. We report a case of treatment of pernicious anemia in a 16 year-old girl during treatment for Graves disease.

The Treatment of Pernicious Anemia in Children with Graves' Disease

Organ specific autoimmune diseases may occur in the same individual. Type III polyglandular autoimmune disease is defined by the occurrence in the same individual of two or more of the following: autoimmune thyroid disease, pernicious anemia, insulin dependent diabetes, and other organ specific autoimmune diseases not falling into class I or class II categories. A 16-year-old girl developed pernicious anemia during the treatment of Graves' disease. She was diagnosed with Graves' disease 5 years ago and had received methimazole 20 mg/day, but the medication was not well tolerated. Bone marrow findings were compatible with pernicious anemia as macrocytic normochromic red blood cell (RBC) and increased megakaryocyte. The serum value of vitamin B12 was low, and the serum titer of antibody to gastric parietal cell was high. After diagnosis of pernicious anemia, she had treatment by monthly intramuscular vitamin B12 and methimazole (20 mg/day). The values of hemoglobin and RBC indices as well as thyroid function were normalized after 2 months. Vitamin B12 therapy was maintained for 1 year with normal RBC indices. At present, she dose not receive treatment with vitamin B12 but she does receive methimazole treatment. We report a case of treatment of pernicious anemia in a 16 year-old girl during treatment for Graves disease.

Quantitative Estimation of Parietal Cell Secretory Function in Man.

With measurement of volume, acid, and intrinsic factor after maximal histamine stimulation the gastric secretory patterns in healthy persons and patients with duodenal ulcer, atrophic gastritis, and pernicious anaemia are described. In duodenal ulcer patients a higher than normal secretion of acid and intrinsic factor was found, but the secretory patterns in duodenal ulcer patients and healthy persons were identical, the essential difference being a higher secretory volume in duodenal ulcer patients corresponding to a greater but normally functioning parietal cell mass. In atrophic gastritis the gastric secretion of acid and intrinsic factor was decreased compared to that of healthy persons. The acid secretion was more reduced than the secretion of intrinsic factor, and the findings may indicate a dissociation between these parietal cell secretory components in hyposecretory states. In patients with pernicious anaemia an abolished parietal cell function was found.