scholarly journals Acquired Thrombotic Thrombocytopenic Purpura in a Patient with Pernicious Anemia

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Ramesh Kumar Pandey ◽  
Sumit Dahal ◽  
Kamal Fadlalla El Jack Fadlalla ◽  
Shambhu Bhagat ◽  
Bikash Bhattarai
Keyword(s):  
Vitamin B12 ◽  
Pernicious Anemia ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hematological Parameters ◽  
Macrocytic Anemia ◽  
Intrinsic Factor ◽  
Serum Vitamin ◽  
Severe Thrombocytopenia ◽  
Thrombocytopenic Purpura ◽  
Intrinsic Factor Antibody

Introduction. Acquired thrombotic thrombocytopenic purpura (TTP) has been associated with different autoimmune disorders. However, its association with pernicious anemia is rarely reported.Case Report. A 46-year-old male presented with blood in sputum and urine for one day. The vitals were stable. The physical examination was significant for icterus. Lab tests’ results revealed leukocytosis, macrocytic anemia, severe thrombocytopenia, renal dysfunction, and unconjugated hyperbilirubinemia. He had an elevated LDH, low haptoglobin levels with many schistocytes, nucleated RBCs, and reticulocytes on peripheral smear. Low ADAMTS13 activity (<10%) with elevated ADAMTS13 antibody clinched the diagnosis of severe acquired TTP, and plasmapheresis was started. There was an initial improvement in his hematological markers, which were however not sustained on discontinuation of plasmapheresis. For his refractory TTP, he was resumed on daily plasmapheresis and Rituximab was started. Furthermore, the initial serum Vitamin B12 and reticulocyte index were low in the presence of anti-intrinsic factor antibody. So with the concomitant diagnosis of pernicious anemia, Vitamin B12 was supplemented. The rest of the immunological workups were negative. Subsequently, his symptoms resolved and his hematological parameters improved.Discussion. While pernicious anemia can masquerade as TTP, an actual association between the two can also occur and needs further evaluation and characterization.

scholarly journals Vitamin B12 Malabsorption Due to Intrinsic Factor Deficiency in Indian Subjects

Blood ◽  
1972 ◽  
Vol 40 (5) ◽  
pp. 747-753 ◽  
Author(s):  
H. G. Desai ◽  
F. P. Antia
Keyword(s):  
Vitamin B12 ◽  
Pernicious Anemia ◽  
Indian Population ◽  
Intrinsic Factor ◽  
Subacute Combined Degeneration ◽  
Factor Deficiency ◽  
Vitamin B12 Absorption ◽  
Intrinsic Factor Deficiency ◽  
Intrinsic Factor Antibody ◽  
Vitamin B12 Malabsorption

Abstract Sixteen patients (from Bombay) with severe vitamin B12 malabsorption due to intrinsic factor deficiency, presenting as subacute combined degeneration of the cord (7), tropical sprue (3), anemia (2), thyrotoxicosis (2), diabetes mellitus (1), and pain in the abdomen (1), are reported. The difficulties of establishing a definite diagnosis of pernicious anemia in Indian population are described. The lower incidence of circulating intrinsic factor antibody (IFA) in Indian patients with histamine-fast achlorhydria and poor vitamin B12 absorption is emphasized. The necessity of separating atrophic gastritis, with severely impaired vitamm B12 absorption, from pernicious anemia on the basis of absence or presence of IFA in serum and/or gastric juice cannot be overemphasized.

A Case Report of Pernicious Anemia and Recurrent Aphthous Stomatitis

2009 ◽  
Vol 10 (2) ◽  
pp. 83-89 ◽  
Author(s):  
Ricardo Alves Mesquita ◽  
Bruna Gonçalves Garcia ◽  
Marcelo Ferreira Pinto Cardoso ◽  
Omar de Faria ◽  
Ricardo Santiago Gomez
Keyword(s):  
Folic Acid ◽  
Case Report ◽  
Vitamin B12 ◽  
Pernicious Anemia ◽  
Systemic Disease ◽  
Intrinsic Factor ◽  
Serum Vitamin ◽  
Recurrent Aphthous Stomatitis ◽  
Aphthous Stomatitis ◽  
Vitamin B12 Malabsorption

Abstract Aim The aim of this report is to present the management of a patient with pernicious anemia afflicted with recurrent aphthous stomatitis (RAS). Background RAS is one of the most common lesions of the oral mucosa. Although the exact etiology of RAS is still unknown different hematinic deficiencies have been proposed. Case Report Painful recurrent ulcers covered with a grayish pseudomembrane surrounded by an erythematous margin were identified on the tongue and in the buccal mucosa of a 71-year-old woman. The patient also presented with depapilation of the tongue. The clinical diagnosis was RAS. Laboratory tests including a hemogram were ordered to determine existing levels of folic acid, iron, ferritin, and vitamins B2, B6, and B12. Levels of serum vitamin B12 and serum hemoglobin were low. The laboratory investigation also showed a medium corpuscular volume of 104.1 fl. A gastroduodenoscopy revealed no macroscopic abnormality. A gastric biopsy showed mucosal atrophy in the gastric corpus with evidence of intestinal metaplasia. Antibodies against an intrinsic factor were negative. The diagnosis pernicious anemia was made, with RAS caused by vitamin B12 malabsorption. Treatment consisted of the administration of 1.0 ml of hydroxocolabamin intramuscularly twice weekly over four weeks followed by 1.0 ml once weekly for four weeks. Clinical resolution was observed after two months. Summary The association of RAS with vitamin B12 malabsorption is a rare event. However, along with conventional RAS clinical management, iron, folic acid, vitamin B deficiencies, and nutritional intolerance must be considered. Evaluation of the predisposing factors is imperative in treating patients with RAS including vitamin B12 malabsorption. Clinical Significance Determination of the levels of vitamin B12 should be the basis for replacement therapy. Such therapy can be considered a benefit to the patients with RAS as its etiology remains unclear. Clinicians must be alert to the possibility this lesion could be a signal of systemic disease. Citation Garcia BG, Cardoso MFP, Faria O, Gomez RS, Mesquita RA. A Case Report of Pernicious Anemia and Recurrent Aphthous Stomatitis. J Contemp Dent Pract 2009 March; (10)2:083-089.

scholarly journals Metabolic Interrelations between Gastric Intrinsic Hematopoietic Factor and Vitamin B12

Blood ◽  
1954 ◽  
Vol 9 (12) ◽  
pp. 1127-1140 ◽  
Author(s):  
GEORGE B. JERZY GLASS ◽  
LOIS C. LILLICK ◽  
LINN J. BOYD ◽  
CARRIE GUNNIS ◽  
LUCY GORMAN CORTI
Keyword(s):  
Vitamin B12 ◽  
Pernicious Anemia ◽  
Total Gastrectomy ◽  
Macrocytic Anemia ◽  
Intrinsic Factor ◽  
The Body ◽  
Urinary Output ◽  
Blood Levels ◽  
Factor Concentrate ◽  
Hematopoietic Response

Abstract The concentration of vitamin B12 in blood serum and its excretion in the urine were assayed by the E. coli mutant technic in two patients after total gastrectomy and by use of the more sensitive Euglena gracilis method in four cases of pernicious anemia in relapse and 1 case of well controlled nutritional macrocytic anemia. Following parenteral administration of 100 µg. vitamin B12 intramuscularly to two patients with total gastrectomy, much higher blood levels were observed within the first few hours than those reported by others under similar circumstances in normals. Following oral administration of vitamin B12 together with a potent intrinsic factor concentrate from hog stomach, a rise in blood levels of vitamin B12 was observed in patients with pernicious anemia. The normal serum levels of vitamin B12 could not be maintained, however, for the entire duration of the oral treatment, in spite of the apparent hematologic and clinical remission. The urinary output of vitamin B12 was in all instances less than 0.5 per cent of the total dose of vitamin B12 ingested during the experimental period, in spite of an optimal or suboptimal hematopoietic response obtained. A similar situation existed in patients with total gastrectomy, as well as in the patient with controlled macrocytic nutritional anemia, when vitamin B12 was ingested alone or with intrinsic factor concentrate. The intensity of the obtained hematopoietic response in patients with pernicious anemia indicates the absorption of much larger amounts of vitamin B12 from the intestine than is indicated by the total urinary output of vitamin B12; therefore the anchorage and retention of a large part of vitamin B12 in the storage depots of the body after its absorption from the intestine must occur. A sharp increase in the urinary output of vitamin B12 was observed during or immediately following the hematopoietic response in all patients with pernicious anemia treated orally with vitamin B12 and intrinsic factor concentrate, i.e., coincident with or immediately following the reticulocyte peak in blood. Similar observation was reported by the authors in the previous series of investigations. Of many possible interpretations of this finding, the most plausible appears to be the metabolic release of free vitamin B12 from its complex binding in the body during hyperactivity of the hematopoietic organs and its escape through the kidneys.

JUVENILE PERNICIOUS ANEMIA

PEDIATRICS ◽  
1951 ◽  
Vol 8 (1) ◽  
pp. 88-106
Author(s):  
EDWARD H. REISNER ◽  
JAMES A. WOLFF ◽  
R. JAMES MCKAY ◽  
EUGENIA F. DOYLE
Keyword(s):  
Vitamin B12 ◽  
Pernicious Anemia ◽  
Gastric Juice ◽  
Liver Extract ◽  
Macrocytic Anemia ◽  
Intrinsic Factor ◽  
The Other ◽  
Free Hydrochloric Acid ◽  
Laboratory Findings ◽  
Gastric Contents

Histories have been presented of two pairs of sibling children with recurrent macrocytic anemia with megaloblastic bone marrow responding specifically to liver extract and vitamin B12. The clinical features and laboratory findings were similar to those of adult pernicious anemia in every respect except for the inconstant presence of histamine refractory achlorhydria. All the patients responded to vitamin B12 given parenterally, but in three to whom it was given by mouth no response was obtained. Two of these subsequently responded to oral B12 when it was accompanied by normal gastric juice. The other two developed histamine refractory achlorhydria while in hematologic remission. This is interpreted as proof that the anemia was due to deficient intrinsic factor in the gastric juice. Three patients showed evidence of disease of the spinal cord, in two of whom the symptoms were severe. These symptoms were compatible with a diagnosis of dorsolateral sclerosis, and improved with antipernicious anemia therapy. In one of these patients, treated with folic acid, the neurologic lesions were aggravated severely. Pernicious anemia due to the absence of gastric juice intrinsic factor occurs in children. The presence of free hydrochloric acid in the gastric contents does not preclude the possibility of the disease.

scholarly journals Evaluation of clinical, biochemical and hematological parameters in macrocytic anemia

2019 ◽  
Vol 6 (2) ◽  
pp. 489
Author(s):  
Nalina Thimmappa ◽  
Vijeth S. B. ◽  
Prashanth G. ◽  
Sreedevi B. K.
Keyword(s):  
Bone Marrow ◽  
Vitamin B12 ◽  
Biochemical Parameters ◽  
Reticulocyte Count ◽  
Megaloblastic Anemia ◽  
Hematological Parameters ◽  
Macrocytic Anemia ◽  
Serum Vitamin ◽  
Systematic Evaluation ◽  
Significant Difference

Background: Macrocytosis can be seen in many hematological and non-hematological disorders and more than one cause may co-exist in an individual. Serum vitamin B12 and folic acid tests are routinely ordered but they are limited by their low sensitivity and specificity. This study is done to analyze the clinical, hematological and biochemical parameters in macrocytic anemia and to study the difference between megaloblastic and non-megaloblastic anemia in these parameters.Methods: There were 100 patients presenting with macrocytosis were taken in to study. A detailed clinical history and physical examination was done in all cases. CBC, biochemical investigations, peripheral blood examination, Vitamin B12, folate levels, bone marrow aspiration and reticulocyte count was done in all cases.Results: Primary bone marrow disorders were the most common cause of macrocytosis (45%). The other causes in decreasing order of frequency were megaloblastic anemia (36%), alcoholism and liver disease (15%), drug induced (2%) and idiopathic thrombocytopenic purpura (1%). There was a significant difference in the mean values of MCV and serum LDH between megaloblastic and non-megaloblastic macrocytosis. When serum LDH >1124.5IU/L or MCV>120.5fl (criterion values of ROC curve) with reticulocyte count <2% was taken as criteria, the sensitivity was 94.4% and specificity was 93% for diagnosing megaloblastic anemia.Conclusions: Systematic evaluation of macrocytosis will help us to distinguish megaloblastic and non-megaloblastic macrocytosis. The blood and biochemical parameters especially CBC, RC, and serum LDH along with supporting clinical features help us in diagnosing megaloblastic anemia in a setup where vitamin and metabolite levels are difficult to obtain.

scholarly journals A Case of Autoimmune Polyglandular Syndrome Type 3b Initially Presenting as Generalized Weakness in an Elderly Patient

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A776-A776
Author(s):  
Hazem Ayesh ◽  
Cameron Burmeister ◽  
Ahmed Abdelrahman ◽  
Azizullah Beran ◽  
Pooja Suri
Keyword(s):  
Vitamin B12 ◽  
Pernicious Anemia ◽  
Female Patient ◽  
Hemolytic Anemia ◽  
Autoimmune Thyroiditis ◽  
Macrocytic Anemia ◽  
Intrinsic Factor ◽  
Coombs Test ◽  
Autoimmune Polyglandular Syndrome ◽  
History Of

Abstract Introduction: Autoimmune polyglandular syndrome (APS) is a multiorgan genetic autoimmune disease. APS-3B subtype is autoimmune thyroiditis with pernicious anemia. In this case, we will discuss an elderly female patient diagnosed with APS-3B. Case Presentation: A 69-year-old Caucasian female patient with a past medical history of autoimmune thyroiditis presented to the emergency department with a two-month history of generalized weakness and nausea. Associated symptoms included shortness of breath and diarrhea. Review of systems was otherwise unremarkable. Physical exam was positive for depigmented skin macules over the upper extremities. Lab results showed hemoglobin 8.2 [11.7 - 15.5 g/dL], MCV 121[80 - 100 fL], platelets 144,000 [150 - 450 X10E9/L], WBC 1.9 [4.0 - 11.0 X10E9/L], LDH 1153[100 - 235 U/L], TSH 0.28[0.49 - 4.67 uIU/mL], free T4 1.7 [0.61 - 1.60 ng/dL], direct Coombs test negative. Iron saturation 55%, vitamin B12 level &lt;50 [180 - 914 pg/mL], folate &gt;25[&gt;5.8 ng/mL], total bilirubin 2.3 [0.3 - 1.2 mg/dL], haptoglobin &lt;30 [32 - 228 mg/dL], AST 43 [0 - 41 U/L], reticulocyte 1.4%. Blood smear showed absolute neutropenia with flow cytometry unremarkable. Chest x-ray and urinalysis were negative. Immunofixation showed low IgM 44 [45 - 281 mg/dL], low IgG 619 [635 - 1,741 mg/dL]. Intrinsic factor antibodies (IF-Ab) were positive. Hematology reported that hemolytic anemia is less likely given Coombs test was negative. About 1.5% of Vitamin B12 deficiency present with a hemolytic picture due to ineffective erythropoiesis while Coombs test help to differentiate it from autoimmune hemolytic anemia. Diagnosis of pernicious anemia was made and the patient started on vitamin B12 injections. The combination of pernicious anemia, autoimmune thyroiditis, and vitiligo supported the diagnosis of autoimmune APS-3B. There was a normalization of vitamin B12 level and symptomatic improvement on a one-week follow-up. Discussion: The patient was diagnosed with autoimmune thyroiditis in 2014 with positive anti-TPO antibodies and elevated TSH; she required levothyroxine supplementation since diagnosis. Hypothyroidism causes macrocytic anemia, which may delay pernicious anemia diagnosis. APS-3B is associated with HLA-B8 and/or DR3 and DR5. Many studies reported that autoantibodies can be detected before developing symptoms of organ involvement. Thorough family history provides support for autoantibody testing to detect cases of APS-3B earlier. Active surveillance and early diagnosis will help minimize invasive testing such as bone marrow biopsy, so proper history taking is a key factor to early diagnose these conditions. Conclusion: APS-3B is a rare disorder. Diagnosis is difficult hypothyroidism causes macrocytic anemia. Early detection of APS-3B may help to prevent complications that increase the risk of mortality and morbidity, particularly in the elderly population.

scholarly journals The Value of the Serum Vitamin B12 Level in Diagnosing B12 Deficiency

Blood ◽  
1974 ◽  
Vol 43 (6) ◽  
pp. 915-921 ◽  
Author(s):  
H. Irving Pierce ◽  
Robert S. Hillman
Keyword(s):  
Vitamin B12 ◽  
Clinical Evidence ◽  
Macrocytic Anemia ◽  
Intrinsic Factor ◽  
Serum Vitamin ◽  
Clear Cut ◽  
Physician Performance ◽  
Factor Deficiency ◽  
B12 Deficiency ◽  
Intrinsic Factor Deficiency

Abstract Serum vitamin B12 levels of 2523 individuals were measured using the hemoglobin-coated charcoal assay. In one hospital, the test was performed on 1698 patients under hematologic evaluation for erythrocytic abnormalities of all types, while 825 patients from five other hospitals were studied because of suspected B12 deficiency. The incidence of low serum vitamin B12 was surprisingly low for both groups (2.3% and 3.5%, respectively). The value of the test as a diagnostic tool was further reduced by two characteristics of physician performance. First, with those patients who demonstrated a clear-cut macrocytic anemia and were suspects for intrinsic factor deficiency (pernicious anemia), extensive evaluations for B12 malabsorption were usually carried out prior to the receipt of the low B12 level. Second, when a deficiency state was not suspected, the return of a low value did not reliably stimulate physicians to begin evaluation or institute therapy. This was true despite the presence of clinical evidence of a possible gastrointestinal abnormality in many of the patients.

scholarly journals Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, and Management

2021 ◽  
Vol 10 (3) ◽  
pp. 536
Author(s):  
Senthil Sukumar ◽  
Bernhard Lämmle ◽  
Spero R. Cataland
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Initial Therapy ◽  
Fresh Frozen Plasma ◽  
Organ Injury ◽  
Severe Thrombocytopenia ◽  
Front Line ◽  
Thrombocytopenic Purpura ◽  
Fresh Frozen ◽  
Biallelic Mutations ◽  
Line Setting

Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and ischemic end organ injury due to microvascular platelet-rich thrombi. TTP results from a severe deficiency of the specific von Willebrand factor (VWF)-cleaving protease, ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). ADAMTS13 deficiency is most commonly acquired due to anti-ADAMTS13 autoantibodies. It can also be inherited in the congenital form as a result of biallelic mutations in the ADAMTS13 gene. In adults, the condition is most often immune-mediated (iTTP) whereas congenital TTP (cTTP) is often detected in childhood or during pregnancy. iTTP occurs more often in women and is potentially lethal without prompt recognition and treatment. Front-line therapy includes daily plasma exchange with fresh frozen plasma replacement and immunosuppression with corticosteroids. Immunosuppression targeting ADAMTS13 autoantibodies with the humanized anti-CD20 monoclonal antibody rituximab is frequently added to the initial therapy. If available, anti-VWF therapy with caplacizumab is also added to the front-line setting. While it is hypothesized that refractory TTP will be less common in the era of caplacizumab, in relapsed or refractory cases cyclosporine A, N-acetylcysteine, bortezomib, cyclophosphamide, vincristine, or splenectomy can be considered. Novel agents, such as recombinant ADAMTS13, are also currently under investigation and show promise for the treatment of TTP. Long-term follow-up after the acute episode is critical to monitor for relapse and to diagnose and manage chronic sequelae of this disease.

The occurrence of thrombotic thrombocytopenic purpura during treatment of HCV with direct-acting antiviral agents

2018 ◽  
Vol 1 (1) ◽  
pp. 1-9
Author(s):  
Amr Hanafy ◽  
◽  
Waseem Seleem ◽  
Salem Mohamed ◽  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Peripheral Blood Smear ◽  
Hcv Infection ◽  
Severe Thrombocytopenia ◽  
Direct Acting Antivirals ◽  
Thrombocytopenic Purpura ◽  
Direct Acting Antiviral ◽  
Chronic Hcv ◽  
Direct Acting

Background and aim Experts have reported thrombocytopenia linked to chronic liver disease in up to 70% in patients with advanced fibrosis and portal hypertension. Thrombotic thrombocytopenic purpura (TTP) occurrence with HCV infection is a rare and life-threatening event. We aimed to investigate the cause of disturbed conscious level, acute hemolytic anemia, and severe thrombocytopenia in a male patient with chronic HCV and under treatment with direct-acting antivirals. Case report: Development of severe thrombocytopenia, acute hemolytic anemia, neurological symptoms in the form of fits and coma in a 32- year- old man with chronic HCV infection after one week of treatment with direct-acting antivirals (sofosbuvir 400mg PO daily, and daclatasvir 60 mg PO daily). Brain CT was normal, with a negative Coombs test and the presence of schistocytes in the peripheral blood smear. The patient presentation was suggestive of thrombotic thrombocytopenic purpura (TTP). Conclusion: This is a case of TTP after one week of direct-acting antiviral drugs despite the safety profile of these medications. Studying the pathophysiology of TTP after DAAs needs more clarifications.

Sign in / Sign up

Export Citation Format

Share Document