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Ralph Carmel - One of the best experts on this subject based on the ideXlab platform.
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failures of cobalamin assays in Pernicious Anemia
The New England Journal of Medicine, 2012Co-Authors: Ralph Carmel, Y P AgrawalAbstract:The authors show that commercial tests for cobalamin levels provide false normal values in 22 to 35% of cases of Pernicious Anemia, the main disease they were designed to detect. The manufacturers need to solve the problem of interference of the assay by antibodies to intrinsic factor.
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autoimmune cytopenias in Pernicious Anemia a report of four cases and review of the literature
European Journal of Haematology, 2009Co-Authors: Arthur P Rabinowitz, Yale Sacks, Ralph CarmelAbstract:Abstract: Pernicious Anemia appears to be autoimmune in origin and is associated with immune disorders of several organ systems. We report 4 patients with Pernicious Anemia and immune cytopenias, an association that may sometimes pose diagnostic problems unless specifically considered. Pernicious Anemia coexisted with or was closely followed by idiopathic thrombocytopenic purpura in 3 patients and by autoimmune hemolytic Anemia in a 4th patient. In addition to cobalamin therapy, all patients required corticosteroids (2 also received danazol), while 1 also required splenectomy. All 4 patients were women. The 3 patients with idiopathic thrombocytopenic purpura were also blood group O and were iron-deficient. Autoimmune cytopenias may occur in patients with treated or untreated Pernicious Anemia and require specific therapy.
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prevalence of undiagnosed Pernicious Anemia in the elderly
JAMA Internal Medicine, 1996Co-Authors: Ralph CarmelAbstract:Background: Existing information about the prevalence of Pernicious Anemia is largely based on older surveys that favored florid manifestations, tended to be retrospective analyses of previously diagnosed disease, and usually studied homogeneous European populations. The lack of current data in the United States has, among other things, hampered discussions of the proposal to increase folate intake by the general population. Objective: To estimate the prevalence of undiagnosed and untreated Pernicious Anemia among the elderly. Methods: A prospective survey of cobalamin levels and anti—intrinsic factor antibody was done in the elderly. Blood testing was done in 729 people aged 60 years or older and follow-up assessment with the Schilling test and other tests was offered when results were abnormal. Results: Seventeen subjects were found to have Pernicious Anemia, usually with only minimal clinical manifestations of cobalamin deficiency. Although cobalamin deficiency had been suspected by the physicians of three subjects, they had been treated inadequately and still had evidence of deficiency. Excluding these three partially treated subjects from the analysis, 1.9% of the survey population had unrecognized and untreated Pernicious Anemia. The prevalence was 2.7% in women and 1.4% in men; 4.3% of the black women and 4.0% of the white women had Pernicious Anemia. Conclusions: Undiagnosed Pernicious Anemia is a common finding in the elderly, especially among black and white women. If these findings can be extrapolated, almost 800000 elderly people in the United States have undiagnosed and untreated Pernicious Anemia, and, thus, would be at possible risk for masked cobalamin deficiency if exposed to large amounts of folate. This number does not include those elderly with cobalamin deficiency caused by other disorders or the still unknown number of younger people with unrecognized Pernicious Anemia and other causes of deficiency. ( Arch Intern Med. 1996;156:1097-1100 )
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prevalence of undiagnosed Pernicious Anemia in the elderly
American Society of Hematology Annual Meeting, 1996Co-Authors: Ralph CarmelAbstract:Background : Existing information about the prevalence of Pernicious Anemia is largely based on older surveys that favored florid manifestations, tended to be retrospective analyses of previously diagnosed disease, and usually studied homogeneous European populations. The lack of current data in the United States has, among other things, hampered discussions of the proposal to increase folate intake by the general population. Objective : To estimate the prevalence of undiagnosed and untreated Pernicious Anemia among the elderly. Methods : A prospective survey of cobalamin levels and anti-intrinsic factor antibody was done in the elderly. Blood testing was done in 729 people aged 60 years or older and follow-up assessment with the Schilling test and other tests was offered when results were abnormal. Resuts : Seventeen subjects were found to have Pernicious Anemia, usually with only minimal clinical manifestations of cobalamin deficiency. Although cobalamin deficiency had been suspected by the physicians of three subjects, they had been treated inadequately and still had evidence of deficiency. Excluding these three partially treated subjects from the analysis, 1.9% of the survey population had unrecognized and untreated Pernicious Anemia. The prevalence was 2. 7% in women and 1.4% in men ; 4.3% of the black women and 4.0% of the white women had Pernicious Anemia. Conclusions : Undiagnosed Pernicious Anemia is a common finding in the elderly, especially among black and white women. If these findings can be extrapolated, almost 800 000 elderly people in the United States have undiagnosed and untreated Pernicious Anemia, and, thus, would be at possible risk for masked cobalamin deficiency if exposed to large amounts of folate. This number does not include those elderly with cobalamin deficiency caused by other disorders or the still unknown number of younger people with unrecognized Pernicious Anemia and other causes of deficiency.
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helicobacter pylori infection in Pernicious Anemia a prospective controlled study
Gastroenterology, 1991Co-Authors: Ralph Carmel, Tseling Fong, Cornelius P Dooley, Margarita Dehesa, Hartley CohenAbstract:Although some authors believe that Helicobacter pylori is the etiologic agent in chronic nonspecific gastritis, it has also been suggested that the bacterium colonizes inflamed mucosa as a secondary event. This study documents the prevalence of H. pylori in 28 patients with Pernicious Anemia and compares the findings with those of a group of 28 age-, race-, and sex-matched asymptomatic control subjects. All subjects underwent endoscopy with biopsy of the gastric antrum and corpus. A sample of serum was obtained before endoscopy for determination of antibodies (immunoglobulin A and immunoglobulin G) to H. pylori. The prevalence of H. pylori (by biopsy) in patients with Pernicious Anemia was significantly less than that in controls (11% vs. 71%, P less than 0.0001). All patients with Pernicious Anemia had abnormalities of corpus histology (inflammation and/or atrophy). In addition, 50% of patients with Pernicious Anemia had a lymphocytic infiltration of the antrum. All controls with H. pylori had gastritis, 50% having active chronic gastritis. Atrophic changes of the corpus were more commonly found in patients with Pernicious Anemia (75% vs. 7%, P less than 0.0001). Serology and biopsy results correlated poorly in the patients with Pernicious Anemia: all 5 patients with positive serology results had negative biopsy results, whereas all 3 patients with positive cultures on biopsy had negative serological studies. In conclusion, patients with Pernicious Anemia are protected from infection with H. pylori, and H. pylori does not passively colonize mucosa inflamed by an unrelated process.
Siddesh Besur - One of the best experts on this subject based on the ideXlab platform.
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Pseudo-thrombotic thrombocytopenic purpura: A rare presentation of Pernicious Anemia.
North American journal of medical sciences, 2011Co-Authors: Ashvin Tadakamalla, Siva K. Talluri, Siddesh BesurAbstract:Context: Schistocytes are fragmented red blood cells due to the flow of blood through damaged capillaries and indicate endothelial injury. They are typical of microangiopathic hemolytic Anemia seen in life threatening conditions like disseminated intravascular coagulation or thrombotic thrombocytopenic purpura/hemolytic uremic syndrome .We report a rare sub-acute presentation of Pernicious Anemia with hemolysis, thrombocytopenia and numerous schistocytes that was initially diagnosed as a more serious thrombotic thrombocytopenic purpura. Case Report : A 31-year-old Caucasian woman presented with fatigue and paresthesia of both feet for 1 week. Past medical history included hypertension and gastro-esophageal reflux disease. Examination revealed scleral icterus and pallor. Examination of the abdomen did not show hepatosplenomegaly. Initial laboratory tests showed severe Anemia, and low platelets. Indirect bilirubin and serum Lactate De Hydrogenase were elevated. Prothrombin time, partial thromboplastin time, serum fibrinogen, and serum fibrin degradation product levels were normal. Peripheral smear revealed numerous schistocytes, anisocytosis and macro-ovalocytes. Thrombotic thrombocytopenic purpura (TTP) was suspected due to the constellation of sub-acute onset of fatigue and paresthesia along with thrombocytopenia, schistocytes and an elevated LDH. Plasmapheresis was initiated for possible TTP. However, platelet count worsened despite plasmapheresis for 4 days. On re-evaluation, vitamin B 12 was found to be low. Treatment with intra-muscular vitamin B 12 led to symptomatic and hematologic improvement. Pernicious Anemia was confirmed by the presence of anti-intrinsic factor antibodies, elevated serum gastrin level and atrophic gastritis. Conclusion : Clinicians must be aware of unusual clinical presentation of vitamin B 12 deficiency with schistocytes as the management is simple and effective.
Hsinming Chen - One of the best experts on this subject based on the ideXlab platform.
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do all the patients with vitamin b12 deficiency have Pernicious Anemia
Journal of Oral Pathology & Medicine, 2016Co-Authors: Andy Sun, Shihjung Cheng, Julia Yu Fong Chang, Hsinming Chen, Yiping Wang, Chunpin ChiangAbstract:Background Vitamin B12 deficiency may result in Pernicious Anemia (PA). This study evaluated whether all the patients with vitamin B12 deficiency had PA. Methods The blood hemoglobin (Hb), iron, vitamin B12, folic acid, and homocysteine concentrations and mean corpuscular volume (MCV) in 90 vitamin B12-deficient patients were measured and compared with the corresponding data in 180 age- and sex-matched healthy control subjects. PA was defined by World Health Organization (WHO) as having an Hb concentration <13 g/dl for men and <12 g/dl for women, an MCV ≧ 100 fl, a serum vitamin B12 level <200 pg/ml, and serum gastric parietal cell antibody (GPCA) positivity. Results We found that 35 (38.9%) and 20 (22.2%) patients with vitamin B12 deficiency had deficiencies of Hb (men 12.7 μM) and high MCV (≧100 fl), respectively. In addition, 43 (47.8%) vitamin B12-deficient patients with had GPCA positivity. Patients with vitamin B12 deficiency had a significantly higher frequency of Hb or iron deficiency, of abnormally elevated blood homocysteine level or high MCV, and of GPCA positivity than healthy control subjects (all P-values < 0.001). However, only 17 (18.9%) of 90 vitamin B12-deficient patients were diagnosed as having PA by the WHO definition. Conclusion Only 18.9% of patients with vitamin B12 deficiency are discovered to have PA by the WHO definition.
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hematinic deficiencies and Pernicious Anemia in oral mucosal disease patients with macrocytosis
Journal of the Formosan Medical Association, 2015Co-Authors: Julia Yu Fong Chang, Shihjung Cheng, Yang Che Wu, Yiping Wang, Hsinming ChenAbstract:Background/purpose Macrocytosis is defined as having the mean corpuscular volume (MCV) ≥ 100 fL. This study assessed hematinic deficiencies and Pernicious Anemia (PA) in oral mucosal disease patients with macrocytosis. Methods The blood hemoglobin (Hb), iron, vitamin B12, folic acid, and homocysteine concentrations and MCV in 60 oral mucosal disease patients with macrocytosis were measured and compared with the corresponding data in 120 age- and sex-matched healthy control participants. PA was defined by the World Health Organization (WHO) as having an Hb concentration Results We found that 30 (50.0%), 7 (11.7%), 24 (40.0%), and three (5.0%) oral mucosal disease patients with macrocytosis had deficiencies of Hb (men 12.3 μM) and serum GPCA positivity, respectively. Macrocytosis patients had a significantly higher frequency of Hb, iron, or vitamin B12 deficiency, of abnormally elevated blood homocysteine level, and of GPCA positivity than healthy control participants ( p Conclusion Only 16.7% of oral mucosal disease patients with macrocytosis are discovered to have PA by the WHO definition.
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Hematinic deficiencies and Pernicious Anemia in oral mucosal disease patients with macrocytosis
Elsevier, 2015Co-Authors: Julia Yu Fong Chang, Shihjung Cheng, Hsinming Chen, Yiping Wang, Andy SunAbstract:Macrocytosis is defined as having the mean corpuscular volume (MCV) ≥ 100 fL. This study assessed hematinic deficiencies and Pernicious Anemia (PA) in oral mucosal disease patients with macrocytosis. Methods: The blood hemoglobin (Hb), iron, vitamin B12, folic acid, and homocysteine concentrations and MCV in 60 oral mucosal disease patients with macrocytosis were measured and compared with the corresponding data in 120 age- and sex-matched healthy control participants. PA was defined by the World Health Organization (WHO) as having an Hb concentration < 13 g/dL for men and < 12 g/dL for women, an MCV ≥ 100 fL, a serum vitamin B12 level < 200 pg/mL, and serum gastric parietal cell antibody (GPCA) positivity. Results: We found that 30 (50.0%), 7 (11.7%), 24 (40.0%), and three (5.0%) oral mucosal disease patients with macrocytosis had deficiencies of Hb (men
Emmanuel Andres - One of the best experts on this subject based on the ideXlab platform.
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association Pernicious Anemia and autoimmune polyendocrinopathy a retrospective study
Journal of medicine and life, 2017Co-Authors: Abrarahmad Zulfiqar, Emmanuel AndresAbstract:Objective To investigate the association between Pernicious Anemia and other autoimmune diseases. Methods This retrospective and bicentric study was conducted at Reims and Strasbourg University Hospitals and involved 188 patients with Pernicious Anemia examined between 2000 and 2010 in order to search for other autoimmune diseases and to evaluate the role of Pernicious Anemia in autoimmune polyglandular syndrome. Results A total of 74 patients with a combination of Pernicious Anemia and other autoimmune diseases were included in the study. Our study revealed the privileged association of Pernicious Anemia with autoimmune thyroiditis. The association of Pernicious Anemia and autoimmune thyroiditis are a part of the autoimmune polyglandular syndrome type 3b. Conclusion We suggest undertaking a systematic clinical examination and laboratory investigations in search of autoimmune thyroiditis in patient(s) with the diagnosis of Pernicious Anemia. The association of Pernicious Anemia and autoimmune thyroiditis is frequent and a part of autoimmune polyglandular 3b.
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optimal management of Pernicious Anemia
Journal of Blood Medicine, 2012Co-Authors: Emmanuel Andres, Khalid SerrajAbstract:Pernicious Anemia (also known as Biermer's disease) is an autoimmune atrophic gastritis, predominantly of the fundus, and is responsible for a deficiency in vitamin B12 (cobalamin) due to its malabsorption. Its prevalence is 0.1% in the general population and 1.9% in subjects over the age of 60 years. Pernicious Anemia represents 20%-50% of the causes of vitamin B12 deficiency in adults. Given its polymorphism and broad spectrum of clinical manifestations, Pernicious Anemia is a great pretender. Its diagnosis must therefore be evoked and considered in the presence of neurological and hematological manifestations of undetermined origin. Biologically, it is characterized by the presence of anti-intrinsic factor antibodies. Treatment is based on the administration of parenteral vitamin B12, although other routes of administration (eg, oral) are currently under study. In the present update, these various aspects are discussed with special emphasis on data of interest to the clinician.
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effects of oral crystalline cyanocobalamin 1000 μg d in the treatment of Pernicious Anemia an open label prospective study in ten patients
Current Therapeutic Research-clinical and Experimental, 2005Co-Authors: Emmanuel Andres, Noureddine Henoun Loukili, E Noel, Frederic Maloisel, S Vinzio, Georges Kaltenbach, Florence Carosampara, J F BlickleAbstract:Background: Standard treatment of cobalamin (vitamin B12) deficiency involvesregular (1000 μg/mo) IM cyanocobalamin administration. It has been suggested that high-dose (>2000 μg/d) oral cyanocobalamin may be effective in patients with Pernicious Anemia.
Ban Hock Toh - One of the best experts on this subject based on the ideXlab platform.
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Pathophysiology and laboratory diagnosis of Pernicious Anemia
Immunologic Research, 2017Co-Authors: Ban Hock TohAbstract:Pernicious Anemia is the hematologic manifestation of chronic atrophic gastritis affecting the corpus of the stomach that denudes the gastric mucosa of gastric parietal cells. Asymptomatic autoimmune gastritis, a chronic inflammatory disease of the gastric mucosa, precedes the onset of corpus atrophy by 10-20 years. The gastritis arises from activation of pathologic Th1 CD4 T cells to gastric H/K ATPase that is normally resident on gastric mucosal secretory membranes. The onset of autoimmune gastritis is marked by circulating parietal cell antibody to gastric H/K ATPase. Gastric parietal cells produce two essential biologics: intrinsic factor and HCl acid. Pernicious Anemia is a consequence of intrinsic factor loss and neutralizing intrinsic factor antibody that impairs cobalamin absorption. Acid loss leads to iron deficiency Anemia that precedes cobalamin-deficient Pernicious Anemia by 20 years. Laboratory diagnosis rests on parietal cell antibody with or without intrinsic factor antibody, cobalamin-deficient megaloblastic Anemia and elevated serum gastrin from loss of acid secretion. Autoimmune gastritis is associated with autoimmune thyroiditis and type 1 diabetes mellitus.
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animal models of human disease experimental autoimmune gastritis a model for autoimmune gastritis and Pernicious Anemia
Clinical Immunology, 2002Co-Authors: Frank Alderuccio, John W Sentry, Aiden C J Marshall, Mark Biondo, Ban Hock TohAbstract:Human autoimmune gastritis is an organ-specific autoimmune disease of the stomach. It is characterized by the development of disease-specific autoantibodies and a pathology that specifically targets specialized cells within the gastric environment. The autoantigens associated with this disease have been defined as the gastric H+/K+ ATPase and intrinsic factor. The development of experimental disease models has been pivotal in our contemporary understanding of autoimmunity. Here we review mouse models of autoimmune gastritis and their relevance to human autoimmune gastritis associated with Pernicious Anemia. We appraise some historical as well as recent studies of experimental autoimmune gastritis (EAG), highlighting key findings that have formed the basis of our current understanding of the etiology and mechanism(s) associated with autoimmune gastritis. A precise understanding of the pathogenesis of autoimmune gastritis will permit the design of innovative and rational therapeutic strategies to prevent, arrest, ameliorate or reverse the disease.