Retrospective cross-sectional study of 34 cases of pernicious anemia at Mohammed V Military Training Hospital, Morocco.

Introduction: pernicious anemia is an autoimmune disease characterized by atrophic gastritis due to malabsorption of vitamin B12. Certain oral manifestations, such as Hunter´s glossitis and burning mouth syndrome, may precede the onset of this anemia. The aim of this study is to describe the clinical presentation, para-clinical aspects, the treatment, and the evolution of the pernicious anemia (PA) after treatment. Methods: retrospective study conducted at the Department of Haematology and Internal Medicine B of the Mohammed V Military Training Hospital in Rabat between January 2009 and December 2018. Thirty-four patients were enrolled with vitamin B12 deficiency, non-regenerative macrocytic anemia, a positive anti-intrinsic factor antibody and anti-parietal cell antibody and a histological diagnosis of atrophic gastritis in the presence or not of Helicobacter pylori. The qualitative variables were expressed in numbers and percentages, and the quantitative variables in mean and standard deviation. Multivariate analysis used the Fischer test; it was considered significant for a p < 0.05 value. Results: thirty-four cases were studied; the population study consists of 56% (n=19) of men and 44% (n=15) of women. The average age was 54.88± 9.14. The clinical manifestations of pernicious anemia are dominated by megaloblastic anemia 85.3% (n=29), followed by digestive 58.8%(n=20) and oral 55.9% (n=19) signs. Neurological manifestations were rarely found in 41% (n=14). Hunter´s glossitis 37% (n=7), stomatodynia 11% (n=2) were the most common oral manifestations accompanying pernicious anemia. The evolution was favorable in 79.4% (n=27) patients under substitution therapy with vitamin B12. Conclusion: dentists´ involvement in the diagnosis of pernicious anemia is based on changes in oral mucous membranes, which have been reported in 55.9% of all patients. These oral changes may occur in the absence of symptomatic anemia.

Subacute combined degeneration of the spinal cord with concomitant autoimmune disease: report of 2 cases

The etiology of subacute combined degeneration (SCD) of the spinal cord is closely associated with vitamin B12 (VitB12) deficiency. The clinical manifestations of SCD are complex and vary substantially. Due to some SCD patients with atypical manifestations and concomitant autoimmune disorders, the probability of misdiagnosis and missed diagnosis is still relatively high in the early stage. We report the cases of two patients who were missed or misdiagnosed at another hospital because of the normal initial VitB12 level and partial overlap of clinical manifestations, finally diagnosed as SCD with atypical manifestations and concomitant autoimmune disorders, pharyngeal-cervical-brachial Guillain-Barre syndrome in Case 1 and SCD with autoimmune thyroiditis in Case 2. After undergoing corresponding treatment, death was reported in Case 1 and improvement in Case 2. Analysis of the clinical manifestations and investigation of the underlying pathogenesis in such patients could help improve the rate of early diagnosis and allow timely treatment of SCD, thereby preventing disease progression and poor clinical outcomes.

Prevalence of Autoimmune Gastritis in Individuals Undergoing Medical Checkups in Japan.

Objective Based on both endoscopic findings and serum auto-antibody levels, we determined the prevalence of autoimmune gastritis (AIG), which has not been previously reported, in individuals who underwent health checkup examinations in Japan. Methods At total of 6,739 subjects (4,288 males, 2,451 females; mean age 52.1 years) underwent an upper gastrointestinal endoscopic examination as part of an annual medical checkup. Those suspected to have AIG based on endoscopic evidence of proximal-predominant gastric mucosal atrophy were further examined for the presence of anti-parietal cells and anti-intrinsic factor antibodies, with a final diagnosis of AIG made in cases found to be positive for either or both of those factors. Results Of the 6,739 examined subjects, 46 were suspected to have AIG based on the endoscopic findings, of whom 33 were finally diagnosed with AIG, for an overall prevalence 0.49% (females 0.65%, males 0.40%). Seven with AIG also had thyroid disease, including Hashimoto's and Basedow disease, while none with AIG showed anemia in blood test findings. The prevalence of AIG was not different regardless of the H. pylori infection status (negative, positive, post-eradicated). Conclusion In individuals who underwent an upper gastrointestinal endoscopic examination as part of an annual checkup in Japan, the prevalence of AIG was 0.49%. We concluded that it is not uncommon for asymptomatic and healthy individuals to have AIG, and propose that additional studies are needed to clarify its prevalence as well as to establish the criteria used for diagnosis.

West Syndrome in an Infant With Vitamin B12 Deficiency Born to Autoantibodies Positive Mother.

Infantile vitamin B12 deficiency, a rare nutritional disorder in developed countries, is characterized by megaloblastic anemia and non-specific symptoms, including failure to thrive, hypotonia, and seizure. Symptoms usually develop at 6 months of age. Exclusively breast-fed infants of vegan-diet mothers are particularly at risk. We report the case of a 7-month-old boy with West syndrome born to a mother with subclinical vitamin B12 deficiency due to autoantibodies. Electroencephalography revealed the characteristic hypsarrhythmia pattern, whereas cranial magnetic resonance imaging revealed cerebral atrophy and hypomyelination. Biochemical analysis revealed elevated urinary methylmalonic acid and homocysteine and reduced plasma methionine. Serum vitamin B12 levels were extremely low in both the child and his mother. The mother tested positive for both anti-intrinsic factor and anti-parietal cell antibodies. Low-dose adrenocorticotropic hormone was effective for seizure control. Contrary to previous reports, age-appropriate neurodevelopment was not achieved despite rapid normalization of metabolic profile with vitamin B12 supplementation. Further investigations failed to detect any causative mutations in the genes associated with developmental and epileptic encephalopathy as well as metabolic and other identifiable disorders known to cause West syndrome. To the best of our knowledge, this is the first reported case in which maternal anti-intrinsic factor antibody was considered to be the reason for infantile vitamin B12 deficiency with West syndrome. Differential diagnosis of West syndrome should include vitamin B12 deficiency due to its treatable nature, and early diagnosis is essential to prevent permanent neurological consequences.

A case of asymptomatic pancytopenia with clinical features of hemolysis as a presentation of pernicious anemia.

Pernicious anemia is an autoimmune disease with a variety of clinical presentations. We describe a case of pernicious anemia presenting with pancytopenia with hemolytic features. Further workup revealed very low vitamin B12 levels and elevated methylmalonic acid. It is important for a general internist to identify pernicious anemia as one of the cause of pancytopenia and hemolytic anemia to avoid extensive workup. Pernicious anemia can present strictly with hematological abnormalities without neurological problems or vice versa as in our case.

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.

Pernicious Anemia: A Retrospective Analysis of 22 Cases

BACKGROUND: Pernicious anemia is the most common cause of vitamin B12 deficiency in western populations, but to date, only case reports or small series dealing with this malady have been reported in Korea. This study describes the clinical characteristics of pernicious anemia in Koreans. METHODS: We retrospectively analyzed the clinical data for twenty-two Korean patients with pernicious anemia who were diagnosed during the period from 1995 to 2004 at Chungnam National University Hospital. RESULTS: Only two patients were diagnosed before 2000. The median age of the patients was 66 years and the male/female ratio was 1.8. Anemia-associated discomfort was the most common symptom (95.5%); this was followed by gastrointestinal and neurological symptoms (77.2% and 50.0%, respectively). Autoimmune disorders were found in five patients (22.7%). The median hemoglobin level was 7.0g/dL (range: 3.1~11.8g/dL) and pancytopenia was found in 12 patients (54.5%). The median serum vitamin B12 Level was 26pg/mL (range: 12~189pg/mL). Fifteen (78.9%) and eight (42.1%) of the 19 patients who underwent tests for antibodies were positive for anti-intrinsic factor and anti-parietal cell antibody, respectively. Nineteen of 21 patients who were treated with intramuscular cobalamin recovered from their cytopenia within 3 months. The gastrointestinal symptoms resolved completely for all the patients, while neurological symptoms remained for some of the patients. CONCLUSION: This study shows that the clinical features of pernicious anemia in Koreans are not different from those of the western cases. In addition, pernicious anemia is not a rare finding in Korea.