Thrombotic microangiopathy-like hemolysis in vitamin B12 deficiency-related megaloblastic macrocytic anemia (original) (raw)
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Life-threatening Pseudo-thrombotic Microangiopathy Caused by Severe Vitamin B12 Deficiency
2021
Vitamin B12 deficiency is a common cause of macrocytic anemia. Life-threatening hematologic complications including immune and non-immune hemolytic anemia are present in about 10% of the cases. Pseudo-thrombotic microangiopathy is an extremely rare presentation found in around 2.5% of patients with vitamin B12 deficiency. We present a case of a 70-year-old male patient who presented with progressive fatigue and syncope. He was found to severe macrocytic anemia with hemoglobin of 4.1 g/dL. Further workup showed very low serum vitamin B12 level at 22 pg/mL (normal 180-914), methylmalonic acid of 93.23 umol/L (normal 0-0.4) and homocysteine of 93 umol/L (normal 4-12). Anti-parietal cell and intrinsic factor blocking antibodies were positive. He was noted as well to have thrombocytopenia, low haptoglobin, increased lactate dehydrogenase and increased serum creatinine. Peripheral blood smear showed schistocytes, hyper-segmented neutrophils, and marked dimorphic anemia. His presentation w...
Clinical Pharmacology: Advances and Applications
Pseudo-thrombotic microangiopathy (pseudo-TMA) is a recognized, yet uncommon, clinical presentation of vitamin B12 deficiency. Patients with pseudo-TMA present with microangiopathic hemolytic anemia (MAHA), thrombocytopenia and schistocytes. They are often misdiagnosed as thrombotic thrombocytopenia purpura (TTP) and receive unnecessary therapy. Here, we report a case of a 60-year-old male who presented with thrombocytopenia and normocytic normochromic anemia. Anemia work-up was remarkable for severe B12 deficiency (<60 pg/mL) and a positive non-immune hemolysis panel. Peripheral smear was reviewed and showed anisocytes, poikilocytes, schistocytes and hypersegmented neutrophils. Vitamin B12 replacement (1000 mcg IM daily) was started, ADAMTS13 activity was sent and daily plasmapheresis was initiated. Over the next 3 days, the patient's hemoglobin and platelets were stable and the hemolysis panel showed gradual improvement. On day 4, ADAMTS13 activity results came back normal at 61%. Accordingly, plasmapheresis was discontinued, parenteral B12 replacement was continued and that resulted in gradual improvement and eventually cessation of hemolysis and normalization of hemoglobin and platelets. In this patient, parietal cell autoantibodies were positive and so the diagnosis of pernicious anemia was made. Patients with severe vitamin B12 deficiency may present with features mimicking TTP such as MAHA, thrombocytopenia and schistocytosis. An early and accurate diagnosis of pseudo-TMA has a critical clinical impact with respect to administering the correct treatment with vitamin B12 replacement and avoiding, or shortening the duration of, unnecessary therapy with plasmapheresis.
Cureus, 2021
Hemolytic anemia with thrombocytopenia and organ damage raises suspicion for thrombotic microangiopathy (TMA), a pathology that results in thrombosis within the small vessels secondary to endothelial injury. While usually attributed to atypical hemolytic uremic syndrome (aHUS) or thrombotic thrombocytopenic purpura (TTP), an increasingly recognized and treatable entity is pseudo-thrombotic microangiopathic anemia (pseudo-TMA) secondary to severe vitamin B-12 deficiency. While TMA often requires expensive diagnostic testing and can lead to invasive treatment options such as plasma exchange, immunosuppression, and/or complement cascade blocking, pseudo-TMA requires only vitamin supplementation. Therefore, the prompt and accurate diagnosis of this entity is important for the clinician to recognize in order to avoid unnecessary health costs and institute appropriate treatment. We present the case of a 51-year-old male without any past medical history, who presented with generalized weak...
Cureus, 2022
Pernicious anemia (PA) is an autoimmune disease secondary to chronic atrophic gastritis leading to vitamin B12 deficiency. Rarely, some patients may develop advanced hematological complications that mimic those of thrombotic thrombocytopenic purpura (TTP). Differentiating these conditions is crucial because they require different management. We present a case of a 68-year-old male who presented with generalized weakness, fatigue, and shortness of breath. This patient had anemia, thrombocytopenia, and a markedly deficient serum level of vitamin B12. The symptoms initially mimicked those associated with TTP, but the activity of ADAMTS 13 was normal. A diagnosis of pseudo-TTP has been made due to vitamin B12 deficiency resulting from PA with reactive thrombocytosis. Ultimately, vitamin B12 deficiency pseudo-TTP should be considered a differential diagnosis for therapy refractory TTP because of its different management strategies ranging from parenteral Vitamin B12 in PA patients with highly favorable outcomes to more advanced treatment with less favorable outcomes in TTP patients.
Pseudothrombotic Microangiopathy as a Rare Presentation of Cobalamin Deficiency
Cureus, 2021
The hematological manifestations of cobalamin (vitamin B12) deficiency may range from asymptomatic to life-threatening forms. Pseudothrombotic microangiopathy is a rare but severe presentation, characterized by the presence of hemolysis and schistocytosis, that is completely reversible after vitamin supplementation. We present a challenging diagnostic approach of a 55-year-old man who presented with high hemolytic markers, pancytopenia, and schistocytes on the peripheral smear due to acquired cobalamin deficiency. Subsequent testing revealed positive anti-intrinsic factor and anti-parietal cell antibodies consistent with pernicious anemia. Cobalamin replacement led to the resolution of microangiopathic hemolysis and clinical improvement, thereby confirming the diagnosis. This case highlights the importance of early recognition of this syndrome, which is often misdiagnosed as true microangiopathic hemolytic anemia, confounding appropriate management.
Vitamin B12 deficiency from the perspective of a practicing hematologist
Blood, 2017
B12 deficiency is the leading cause of megaloblastic anemia, and although more common in the elderly, can occur at any age. Clinical disease caused by B12 deficiency usually connotes severe deficiency, resulting from a failure of the gastric or ileal phase of physiological B12 absorption, best exemplified by the autoimmune disease pernicious anemia. There are many other causes of B12 deficiency, which range from severe to mild. Mild deficiency usually results from failure to render food B12 bioavailable or from dietary inadequacy. Although rarely resulting in megaloblastic anemia, mild deficiency may be associated with neurocognitive and other consequences. B12 deficiency is best diagnosed using a combination of tests because none alone is completely reliable. The features of B12 deficiency are variable and may be atypical. Timely diagnosis is important, and treatment is gratifying. Failure to diagnose B12 deficiency can have dire consequences, usually neurological. This review is w...
A Case with Pancytopenia and Autoimmune Hemolytic Anemia due to Vitamin B12 Deficiency
Vitamin B12 deficiency anemia often leads to very different clinical findings, such as fatigue, dyspnea, loss of appetite, etc. The following case has been diagnosed with both vitamin B12 deficiency and autoimmune hemolytic anemia that have been presented with seriously hemolysis and pancytopenia. 35-year-old male patient who was Syria refugees and consulted with us due anemia and etiology of pancytopenia by internal medicine service has been admitted and examined by hematology ward. Vitamin B12 deficiency related megaloblastic anemia had been overlooked and after appropriate treatment had been administered clinical symptoms recovered. Both clinical cases should be kept in mind because AHA and vitamin B12 deficiency related megaloblastic anemia could be seen rarely.
IP Innovative Publication Pvt. Ltd., 2018
Aim: To determine the prevalence of vitamin B12 deficiency in subjects with anaemia and elevated mean corpuscular volume. Materials and Methods: Blood samples from 119 subjects were selected for the study based on the inclusion and exclusion criteria laid down. Blood samples were analysed on sysmex KX 21 haematology analyser. Morphological evaluation of blood cells was done on peripheral blood smear. Serum vitamin B 12 assays were done. Qualitative and quantitative variables were analysed by statistical methods. Results: Out of 119 subjects taken for study 70 (58.8 %) were male and 49 (41.2 %) were female with mean age ± SD 40.5± 18. Vitamin B 12 deficiency was noted in 82 (69 %) of which 12 (10.1%) were in Borderline levels of deficiency, 31 (26.1%) were in deficient levels and 39 (32.8%) were in severely deficient group. Red blood cell indices were categorized as per different groups of vitamin B 12 levels. Pancytopenia was noted in 67.1% (54) of B 12 deficient individuals. Significant negative correlation was found between vitamin B 12 levels and mean corpuscular volume (r = - 0.215 .p = 0.019). A positive correlation was found between vitamin B12 and platelet count. (p= < 0.001) and also with leukocyte counts (p value < 0.001) These correlations were found to be statistically significant. Peripheral blood film examination shows macrocytosis in 76 (64%) smears and hypersegmented neutrophils in 66 (56%) smears. Keywords: Vitamin B 12 deficiency, prevalence, Megaloblastic anaemia, Pancytopenia, Mean corpuscular volume.
Journal of Ayub Medical College, Abbottabad : JAMC
Folate and vitamin B12 deficiencies have been known to cause megaloblastic anaemia. Since the deficiencies of these two vitamins are very common in Pakistani population, it would be imperative to investigate their role in causing megaloblastic anaemia. The objective of this study was to find out the contribution of folate and vitamin B12 deficiencies in causing megaloblastic anaemia in our patient population. In this retrospective cohort study, clinical records of 220 patients (101 females and 119 males with an age range of 1-80 years) who presented themselves with macrocytic anaemia at the Aga Khan University Hospital were collected. Data pertaining to complete blood count and serum levels of folate and vitamin B12 were analysed. The mean haemoglobin (Hb) level was 6.8 +/- 0.2 gm/dl. Sixty-nine percent of the patients had severe anaemia (Hb < 8 gm/dl). Mean +/- SEM values of haemoglobin, serum folate and serum B12 were not significantly different between males and females (Hb 6....
Clinico-aetiologic profile of macrocytic anemias with special reference to megaloblastic anemia
Indian Journal of Hematology and Blood Transfusion, 2008
Purpose of study This study was conducted to study the clinical and laboratory parameters in patients with macrocytic anemia and to determine the etiology of macrocytic anemia with special reference to megaloblastic anemia. Materials and methods This study was a cross-sectional descriptive study carried over a period of 18 months on 60 adult patients (age ≥13 years) of macrocytic anemia. Macrocytic anemia was identifi ed when peripheral blood examination showed anemia with a mean red blood corpuscular volume of >95 fl. Result The most common cause of macrocytic anemia was megaloblastic anemia (38.4%). The major causes of nonmegaloblastic macrocytic anemia were primary bone marrow disorders (35%), liver diseases (15%) and hemolytic anemia (8.3%). There was a signifi cant male preponderance in the study (65%). The megaloblastic anemias observed were due to either vitamin B 12 defi ciency (78.3%) or combined B 12 and folate defi ciency (21.7%). A signifi cant proportion of non-vegetarians (73.9%) had megaloblastic anemia. Patients with an MCV of >110fl were more likely to have megaloblastic anemia (p value 0.0007). Three patients (mean age 55 years) with a megaloblastic marrow did not respond to vitamin replacement and were found to have myelodysplastic syndrome. Conclusion Megaloblastic anemia due to Vitamin B 12 or folate defi ciency remains the most important cause of macrocytic anemia. In settings with limited laboratory facilities, a therapeutic trial of vitamins B 12 or folic acid is useful in determining the specifi c vitamin defi ciency.