Your Guide to Anemia
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Predictors of Autoimmune Hemolytic Anemia in Beta-Thalassemia
Blood Cells, Molecules and Diseases 79 (2019) 102342 Contents lists available at ScienceDirect Blood Cells, Molecules and Diseases journal homepage: www.elsevier.com/locate/bcmd Predictors of autoimmune hemolytic anemia in beta-thalassemia patients with underlying red blood cells autoantibodies T ⁎ Monia Ben Khaleda,b, , Monia Ouedernia,b, Nessrine Sahlia,b, Nawel Dhouibb, Ahmed Ben Abdelazizc, Samia Rekayaa,b, Ridha Koukia,b, Houda Kaabid, Hmida Slamad, Fethi Melloulia,b, Mohamed Bejaouia,b a Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia b Pediatric Immuno-Hematology Unit, Bone Marrow Transplantation Center Tunis, Tunis, Tunisia c Information System Directions, Sahloul University Hospital, Sousse, Tunisia d National Center of Blood Transfusion, Tunis, Tunisia ARTICLE INFO ABSTRACT Editor: Mohandas Narla In beta-thalassemia patients, erythrocyte autoantibodies can remain silent or lead to Autoimmune Hemolytic Keywords: Anemia (AIHA).The aim of this study was to identify predictors of AIHA in beta-thalassemia patients with Autoimmune hemolytic anemia positive Direct Antiglobulin Test (DAT), in Tunisia. Thalassemia This longitudinal prognosis study was carried out on beta-thalassemia patients with a positive confirmed Transfusion DAT. Predictors of AIHA were identified the Kaplan-Meier method. A Cox model analysis was used to identify Autoantibodies independent predictors. Red blood cells Among 385 beta thalassemia patients, 87 developed positive DAT (22.6%). Autoimmune hemolytic anemia Direct antiglobulin test was occurred in 25 patients. Multivariate analysis showed that AIHA was independently associated with beta- thalassemia intermedia and similar family history of AIHA. Splenectomy in patients with positive DAT was independently associated with an increased risk of AIHA (HR = 6.175, CI: 2.049–18.612, p < 0.001). -
Section 8: Hematology CHAPTER 47: ANEMIA
Section 8: Hematology CHAPTER 47: ANEMIA Q.1. A 56-year-old man presents with symptoms of severe dyspnea on exertion and fatigue. His laboratory values are as follows: Hemoglobin 6.0 g/dL (normal: 12–15 g/dL) Hematocrit 18% (normal: 36%–46%) RBC count 2 million/L (normal: 4–5.2 million/L) Reticulocyte count 3% (normal: 0.5%–1.5%) Which of the following caused this man’s anemia? A. Decreased red cell production B. Increased red cell destruction C. Acute blood loss (hemorrhage) D. There is insufficient information to make a determination Answer: A. This man presents with anemia and an elevated reticulocyte count which seems to suggest a hemolytic process. His reticulocyte count, however, has not been corrected for the degree of anemia he displays. This can be done by calculating his corrected reticulocyte count ([3% × (18%/45%)] = 1.2%), which is less than 2 and thus suggestive of a hypoproliferative process (decreased red cell production). Q.2. A 25-year-old man with pancytopenia undergoes bone marrow aspiration and biopsy, which reveals profound hypocellularity and virtual absence of hematopoietic cells. Cytogenetic analysis of the bone marrow does not reveal any abnormalities. Despite red blood cell and platelet transfusions, his pancytopenia worsens. Histocompatibility testing of his only sister fails to reveal a match. What would be the most appropriate course of therapy? A. Antithymocyte globulin, cyclosporine, and prednisone B. Prednisone alone C. Supportive therapy with chronic blood and platelet transfusions only D. Methotrexate and prednisone E. Bone marrow transplant Answer: A. Although supportive care with transfusions is necessary for treating this patient with aplastic anemia, most cases are not self-limited. -
A Distant Gene Deletion Affects Beta-Globin Gene Function in an Atypical Gamma Delta Beta-Thalassemia
A distant gene deletion affects beta-globin gene function in an atypical gamma delta beta-thalassemia. P Curtin, … , A D Stephens, H Lehmann J Clin Invest. 1985;76(4):1554-1558. https://doi.org/10.1172/JCI112136. Research Article We describe an English family with an atypical gamma delta beta-thalassemia syndrome. Heterozygosity results in a beta-thalassemia phenotype with normal hemoglobin A2. However, unlike previously described cases, no history of neonatal hemolytic anemia requiring blood transfusion was obtained. Gene mapping showed a deletion that extended from the third exon of the G gamma-globin gene upstream for approximately 100 kilobases (kb). The A gamma-globin, psi beta-, delta-, and beta-globin genes in cis remained intact. The malfunction of the beta-globin gene on a chromosome in which the deletion is located 25 kb away suggests that chromatin structure and conformation are important for globin gene expression. Find the latest version: https://jci.me/112136/pdf A Distant Gene Deletion Affects ,8-Globin Gene Function in an Atypical '6y5-Thalassemia Peter Curtin, Mario Pirastu, and Yuet Wai Kan Howard Hughes Medical Institute and Department ofMedicine, University of California, San Francisco, California 94143 John Anderson Gobert-Jones Department ofPathology, West Suffolk County Hospital, Bury St. Edmunds IP33-2QZ, Suffolk, England Adrian David Stephens Department ofHaematology, St. Bartholomew's Hospital, London ECIA-7BE, England Herman Lehmann Department ofBiochemistry, University ofCambridge, Cambridge CB2-lQW, England Abstract tologic picture of f3-thalassemia minor in adult life. Globin syn- thetic studies reveal a ,3 to a ratio of -0.5, but unlike the usual We describe an English family with an atypical 'yS6-thalassemia fl-thalassemia heterozygote, the levels of HbA2 (and HbF) are syndrome. -
Paroxysmal Nocturnal Haemoglobinuria: a Case Series from Oman Arwa Z
Paroxysmal Nocturnal Haemoglobinuria: A Case Series from Oman Arwa Z. Al-Riyami1*, Yahya Al-Kindi2, Jamal Al-Qassabi1, Sahimah Al-Mamari1, Naglaa Fawaz1, Murtadha Al-Khabori1 , Mohammed Al-Huneini1 and Salam AlKindi3 1Department of Hematology, Sultan Qaboos University Hospital, Muscat, Oman 2College of Medicine and Health Sciences, Sultan Qaboos University, Muscat, Oman 3Department of Hematology, College of Medicine and Health Sciences, Sultan Qaboos University, Muscat, Oman Received: 17 August 2020 Accepted: 23 December 2020 *Corresponding author: [email protected] DOI 10.5001/omj.2022.13 Abstract Introduction Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired stem cell disorder that manifests by hemolytic anemia, thrombosis and cytopenia. There are no data on PNH among Omani patients. Methods We performed a retrospective review of all patients tested for PNH by flow cytometry at the Sultan Qaboos University Hospital between 2012 and 2019. Manifestations, treatment modalities and outcomes were assessed. Results Total of 10 patients were diagnosed or were on follow up for PNH (median age 22.5 years). Clinical manifestations included fatigue (80%) and anemia (70%). There were six patients who had classical PNH with evidence of hemolysis, three patient had PNH in the context of aplastic anemia, and one patient with subclinical PNH. The median reported total type II+III clone size was 95.5 (range 1.54-97) in neutrophils (FLAER/CD24) and 91.6 (range 0.036-99) in monocytes (FLAER/CD14). There were four patients who were found to have a clone size > 50% at time of diagnosis. The median follow up of the patients were 62 months (range: 8-204). -
Anemia in Heart Failure - from Guidelines to Controversies and Challenges
52 Education Anemia in heart failure - from guidelines to controversies and challenges Oana Sîrbu1,*, Mariana Floria1,*, Petru Dascalita*, Alexandra Stoica1,*, Paula Adascalitei, Victorita Sorodoc1,*, Laurentiu Sorodoc1,* *Grigore T. Popa University of Medicine and Pharmacy; Iasi-Romania 1Sf. Spiridon Emergency Hospital; Iasi-Romania ABSTRACT Anemia associated with heart failure is a frequent condition, which may lead to heart function deterioration by the activation of neuro-hormonal mechanisms. Therefore, a vicious circle is present in the relationship of heart failure and anemia. The consequence is reflected upon the pa- tients’ survival, quality of life, and hospital readmissions. Anemia and iron deficiency should be correctly diagnosed and treated in patients with heart failure. The etiology is multifactorial but certainly not fully understood. There is data suggesting that the following factors can cause ane- mia alone or in combination: iron deficiency, inflammation, erythropoietin levels, prescribed medication, hemodilution, and medullar dysfunc- tion. There is data suggesting the association among iron deficiency, inflammation, erythropoietin levels, prescribed medication, hemodilution, and medullar dysfunction. The main pathophysiologic mechanisms, with the strongest evidence-based medicine data, are iron deficiency and inflammation. In clinical practice, the etiology of anemia needs thorough evaluation for determining the best possible therapeutic course. In this context, we must correctly treat the patients’ diseases; according with the current guidelines we have now only one intravenous iron drug. This paper is focused on data about anemia in heart failure, from prevalence to optimal treatment, controversies, and challenges. (Anatol J Cardiol 2018; 20: 52-9) Keywords: anemia, heart failure, intravenous iron, ferric carboxymaltose, quality of life Introduction g/dL in men) (2). -
Severe Aplastic Anemia
Severe AlAplas tic AiAnemia Monica S. Thakar, MD Pediatric BMT Medical College of Wisconsin Outline of Talk 1. Clinical description of aplastic anemia 2. Data collection forms And a couple of quizzes in between… CLINICAL DESCRIPTIONS What is aplastic anemia? • More than just anemia – Involves low counts in 2 of 3 cell lines: red blood cells (RBC), white blood cells (WBC), pltltlatelets • Should NOT involve dysplasia – EiException: RBC can sometimes be dlidysplastic • Should have NORMAL cytogenetics • If dysplasia (beyond RBCs) or abnormal cytogenetics seen, think myelodysplastic syndrome (MDS) What is “severe” aplastic anemia? • Marrow cellularity ≤25% AND • Two of the following peripheral blood features: – Absolute neutrophil count (ANC) < 0.5 x 109/L – Platelet count <20 x 109/L – Absolute reticulocyte count < 40 x 109/L • “Very” severe aplastic anemia – Same as above except ANC <0.2 x 109/L NORMAL BttBottom Li ne: Severely Reduced HtitiHematopoietic Stem Cell Precursors SEVERE APLASTIC ANEMIA IBIn Bone M arrow Epidemiology • Half of cases seen in first 3 decades of life • Incidence: – 2 cases/m illion in WtWestern countitries – 2‐3 fold higher in Asia • Ethnic predisposition: – Asian • Genetics vs different environmental exposures? • Sex predisposition: M:F is 1:1 Pathoppyhysiology : 3 Main Mechanisms Proposed 1. Immune‐mediated – HthiHypothesis: RdRevved‐up T cells dtdestroy stem cells – Observation: Immunosuppression improves blood counts 2. Stem‐cell “depletion” or “defect” – Hypothesis: Drugs or viruses directly destroy stem cells -
Eosinophilic Gastroenteritis Presenting with Severe Anemia and Near Syncope
J Am Board Fam Med: first published as 10.3122/jabfm.2012.06.110269 on 7 November 2012. Downloaded from BRIEF REPORT Eosinophilic Gastroenteritis Presenting with Severe Anemia and Near Syncope Nneka Ekunno, DO, MPH, Kirk Munsayac, DO, Allen Pelletier, MD, and Thad Wilkins, MD Eosinophilic gastrointestinal disorders or eosinophilic digestive disorders encompass a spectrum of rare gastrointestinal disorders that includes eosinophilic esophagitis, eosinophilic gastroenteritis, and eosinophilic colitis. Eosinophilic gastroenteritis is a rare inflammatory disease characterized by eosino- philic infiltration of the gastrointestinal tract. The clinical manifestations include anemia, dyspepsia, and diarrhea. Endoscopy with biopsy showing histologic evidence of eosinophilic infiltration is consid- ered definitive for diagnosis. Corticosteroid therapy, food allergen testing, elimination diets, and ele- mental diets are considered effective treatments for eosinophilic gastroenteritis. The treatment and prognosis of eosinophilic gastroenteritis is determined by the severity of the clinical manifestations. We describe a 24-year-old woman with eosinophilic gastroenteritis presenting as epigastric pain with a history of severe iron deficiency anemia, asthma, eczema, and allergic rhinitis, and we review the litera- ture regarding presentation, diagnostic testing, pathophysiology, predisposing factors, and treatment recommendations. (J Am Board Fam Med 2012;25:913–918.) Keywords: Case Reports, Eosinophilic Gastroenteritis, Gastrointestinal Disorders copyright. A 24-year-old nulliparous African-American woman During examination, her height was 62 inches, was admitted after an episode of near syncope asso- weight 117 lb, and body mass index 21.44 kg/m2. Her ciated with 2 days of fatigue and dizziness. She re- heart rate was 111 beats per minute, blood pressure ported gradual onset of dyspepsia over 2 to 3 121/57 mm Hg, respiratory rate 20 breaths per minute, months. -
The Hematological Complications of Alcoholism
The Hematological Complications of Alcoholism HAROLD S. BALLARD, M.D. Alcohol has numerous adverse effects on the various types of blood cells and their functions. For example, heavy alcohol consumption can cause generalized suppression of blood cell production and the production of structurally abnormal blood cell precursors that cannot mature into functional cells. Alcoholics frequently have defective red blood cells that are destroyed prematurely, possibly resulting in anemia. Alcohol also interferes with the production and function of white blood cells, especially those that defend the body against invading bacteria. Consequently, alcoholics frequently suffer from bacterial infections. Finally, alcohol adversely affects the platelets and other components of the blood-clotting system. Heavy alcohol consumption thus may increase the drinker’s risk of suffering a stroke. KEY WORDS: adverse drug effect; AODE (alcohol and other drug effects); blood function; cell growth and differentiation; erythrocytes; leukocytes; platelets; plasma proteins; bone marrow; anemia; blood coagulation; thrombocytopenia; fibrinolysis; macrophage; monocyte; stroke; bacterial disease; literature review eople who abuse alcohol1 are at both direct and indirect. The direct in the number and function of WBC’s risk for numerous alcohol-related consequences of excessive alcohol increases the drinker’s risk of serious Pmedical complications, includ- consumption include toxic effects on infection, and impaired platelet produc- ing those affecting the blood (i.e., the the bone marrow; the blood cell pre- tion and function interfere with blood cursors; and the mature red blood blood cells as well as proteins present clotting, leading to symptoms ranging in the blood plasma) and the bone cells (RBC’s), white blood cells from a simple nosebleed to bleeding in marrow, where the blood cells are (WBC’s), and platelets. -
December Is National Aplastic Anemia Awareness Month
December Is National Aplastic Anemia Awareness Month What Is Aplastic Anemia? Aplastic anemia is a non-cancerous disease that occurs when the bone marrow stops making enough blood cells. The body makes three types of blood cells: red blood cells, which contain hemoglobin and deliver oxygen to all parts of the body white blood cells, which help fight infection platelets, which help blood clot when you bleed These blood cells are made in the bone marrow, which is the soft, sponge-like material found inside bones. The bone marrow contains immature cells called stem cells that produce blood cells. Stem cells grow into red cells, white cells, and platelets or they can make more stem cells. In patients who have aplastic anemia, there are not enough stem cells in the bone marrow to make enough blood cells. Picture of blood cells maturing from stem cells. Experts believe that aplastic anemia is an autoimmune disorder. This means that the patient’s immune system (which helps fight infection) reacts against the bone marrow and the bone marrow is not able to make blood cells. Stem cells are no longer being replaced and the left over stem cells are not working well. Therefore, the amount of red cells, white cells, and platelets begin to drop. If blood levels drop too low, a person can feel very tired (from low red cells), have bleeding or bruising (from low platelets), and/or have many or severe infections (from low white cells). What Are the Key Statistics About Aplastic Anemia? Aplastic anemia can occur in anyone of any age, race, or gender. -
Concurrent Sickle Cell Anemia and Alpha-Thalassemia. Effect on Pathological Properties of Sickle Erythrocytes
Concurrent sickle cell anemia and alpha-thalassemia. Effect on pathological properties of sickle erythrocytes. S H Embury, … , G Monroy, N Mohandas J Clin Invest. 1984;73(1):116-123. https://doi.org/10.1172/JCI111181. Research Article The concurrence of sickle cell anemia and alpha-thalassemia results in less severe hemolytic anemia apparently as a result of reduced intraerythrocytic concentration of hemoglobin S and its retarded polymerization. We have evaluated the effect of alpha-globin gene number on several interrelated properties of sickle erythrocytes (RBC) that are expected to correlate with the hemolytic and rheologic consequences of sickle cell disease. The irreversibly sickled cell number, proportion of very dense sickle RBC, and diminished deformability of sickle RBC each varied directly with alpha-globin gene number. Sickle RBC density was a direct function of the mean corpuscular hemoglobin concentration (MCHC). Even in nonsickle RBC, alpha-globin gene number varied directly with RBC density. Despite differences in alpha-globin gene number, sickle RBC of the same density had the same degree of deformability and dehydration. These data indicate that the fundamental effect of alpha-thalassemia is to inhibit the generation of sickle RBC having high density and MCHC, and that the other beneficial effects of sickle RBC are secondary to this process. The less consistent effect on overall clinical severity reported for subjects with this concurrence may reflect an undefined detrimental effect of alpha-thalassemia, possibly on the whole blood viscosity or on sickle RBC membrane-mediated adherence phenomena. Find the latest version: https://jci.me/111181/pdf Concurrent Sickle Cell Anemia and a-Thalassemia Effect on Pathological Properties of Sickle Erythrocytes Stephen H. -
Aplastic Anemia Pre-HSCT Data (Form 2028)
Instructions for Aplastic Anemia Pre-HSCT Data (Form 2028) This section of the CIBMTR Forms Instruction Manual is intended to be a resource for completing the Aplastic Anemia Pre-HSCT Data Form. E-mail comments regarding the content of the CIBMTR Forms Instruction Manual to: [email protected]. Comments will be considered for future manual updates and revisions. For questions that require an immediate response, please contact your transplant center’s CIBMTR liaison. TABLE OF CONTENTS Key Fields ............................................................................................................. 2 Disease Assessment at Diagnosis ........................................................................ 2 Laboratory Studies at Diagnosis ........................................................................... 5 Transfusion Status from Diagnosis to the Start of the Preparative Regimen ........ 7 Laboratory Findings Prior to the Start of the Preparative Regimen ....................... 8 Aplastic Anemia Pre-HSCT Data Aplastic Anemia is a disease in which the bone marrow does not produce enough red blood cells, white blood cells, or platelets for the body. The disease can be idiopathic, or can be caused by environmental exposure, pharmaceutical or drug exposure, or exposure to viral hepatitis. Symptoms of aplastic anemia include, but are not limited to pallor, weakness, frequent infection, and/or easy bruising. The Aplastic Anemia Pre-HSCT Data Form is one of the Comprehensive Report Forms. This form captures aplastic -
Increased Red Cell Distribution Width in Fanconi Anemia: a Novel Marker Of
Sousa et al. Orphanet Journal of Rare Diseases (2016) 11:102 DOI 10.1186/s13023-016-0485-0 RESEARCH Open Access Increased red cell distribution width in Fanconi anemia: a novel marker of stress erythropoiesis Rosa Sousa1, Cristina Gonçalves2, Isabel Couto Guerra3, Emília Costa3, Ana Fernandes4, Maria do Bom Sucesso4, Joana Azevedo5, Alfredo Rodriguez6, Rocio Rius6, Carlos Seabra7, Fátima Ferreira8, Letícia Ribeiro5, Anabela Ferrão9, Sérgio Castedo10, Esmeralda Cleto3, Jorge Coutinho2, Félix Carvalho11, José Barbot3 and Beatriz Porto1* Abstract Background: Red cell distribution width (RDW), a classical parameter used in the differential diagnosis of anemia, has recently been recognized as a marker of chronic inflammation and high levels of oxidative stress (OS). Fanconi anemia (FA) is a genetic disorder associated to redox imbalance and dysfunctional response to OS. Clinically, it is characterized by progressive bone marrow failure, which remains the primary cause of morbidity and mortality. Macrocytosis and increased fetal hemoglobin, two indicators of bone marrow stress erythropoiesis, are generally the first hematological manifestations to appear in FA. However, the significance of RDW and its possible relation to stress erythropoiesis have never been explored in FA. In the present study we analyzed routine complete blood counts from 34 FA patients and evaluated RDW, correlating with the hematological parameters most consistently associated with the FA phenotype. Results: We showed, for the first time, that RDW is significantly increased in FA. We also showed that increased RDW is correlated with thrombocytopenia, neutropenia and, most importantly, highly correlated with anemia. Analyzing sequential hemograms from 3 FA patients with different clinical outcomes, during 10 years follow-up, we confirmed a consistent association between increased RDW and decreased hemoglobin, which supports the postulated importance of RDW in the evaluation of hematological disease progression.