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Red cell membrane Na+ transport systems in hereditary spherocytosis: relevance to understanding the increased Na+ permeability.

Red blood cells (RBCs) in hereditary spherocytosis (HS) show high sodium (Na+) and potassium (K+) movement across the membrane, resulting in dehydration. In general, these abnormal cation fluxes have been interpreted as "increased leaks" due to passive or electrodiffusional permeability of the RBC membrane. A study to elucidate the contribution of concomitant ouabain-resistant pathways (Na-K-2Cl cotransport and Na-Li countertransport) to abnormal Na+ permeability present in RBCs of subjects with HS has been undertaken. Accordingly, erythrocyte Na+ and K+ content and transmembrane cation moveme

Acute Hemolytic Anemia and Immune Thrombocytopenia: A Rare Case of Parvovirus B19-Induced Evans Syndrome.

Evans syndrome is a rare condition that can be seen among patients with pre-existing rheumatological disorders such as lupus, rheumatoid art … emolysis. The peripheral smear showed normocytic anemia with severe thrombocytopenia with scattered spherocytes. Flow cytometry showed no evidence of lymphoma. Extensive work-ups for ADAMTS13, HIV, and hepatitis were negative. The IgM titer of parvovirus B19 was significantly elevated. The patient also had positive lupus anticoagulant and positive antibodies …

Rare Loxoscelism-Associated IgG Coombs-Positive Hemolytic Anemia Treated Successfully With Systemic Corticosteroids.

Loxoscelism-associated hemolytic anemia is a rare but critical complication of brown recluse spider bites. It may lead to various systemic manifestations, including jaundice, dark urine, and anemia-related symptoms, in addition to general loxoscelism symptoms such as skin lesions, fever, myalgia, nausea, and vomiting. Prompt diagnosis is crucial and requires recognizing typical laboratory findings such as low hemoglobin, elevated lactate dehydrogenase, reduced haptoglobin levels, and possibly a positive direct antiglobulin test. There is no definitive guideline for the treatment of loxoscelism

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… Figure 2 Peripheral blood smear obtained from the patient showing spherocytes (red arrows) A polyspecific direct antiglobulin test (DAT) was negative than DAT using monospecific IgG and complement was performed, and the IgG was weakly positive. … In our case, the combination of low hemoglobin, low haptoglobin, elevated LDH, spherocytes on peripheral blood smear, and IgG-positive Coombs test, all lead to the presumption that RBCs destruction was induced by warm autoimmune hemolytic anemia. …

[Arginase activity in plasma and erythrocytes in children with hematologic diseases].

INTRODUCTION: Arginase (EC 3.5.3.1) is one of the essential enzymes in the terminal stages of the urea cycle in the liver which participates in the elimination of ammonia from the human body [1, 7]. Except in liver tissue arginase is also present in many human tissues and in the circulating blood cells, especially in erythrocytes and leukocytes. Arginase splits arginase to urea and ornithine that serve for biosynthesis of amino acid proline, glutamic acid and biosynthesis of polyamines-spermine, spermidine and putrescine. Arginase activity is high during the mitotic cycle, with the function in

Hemolytic anemias due to erythrocyte enzyme deficiencies.

Red blood cells can only fulfil their functions over the normal period of approximately 120 days with 1.7 x 10(5) circulatory cycles efficiently if they withstand external and internal loads. This requires ATP and redox equivalents, which have to be permanently regenerated by the energy and redox metabolism. These pathways are necessary to maintain the biconcave shape of the cells, their specific intracellular cation concentrations, the reduced state of hemoglobin with a divalent iron and the sulfhydryl groups of enzymes, glutathione and membrane components. If an enzyme deficiency of one of t

Необычный случай тромбоцитопенсии и ренального провала, возрождающего злодеяние - ассоциированная тромботическая микроангиопатия.

Тромботическая микроангиопатия (TMA) — процесс, характеризующийся тромбоцитопенией, микроангиопатичной гемолитической анемией и конечной травмой. ТМА, связанная с злоумышленностью, встречается редко и может быть похожа на первичные синдромы ТМА, особенно в тех случаях, когда преобладает почечная дисфункция, а периферийные мазки ограничены. Мы представляем случай биопсии, подтвержденной почечной ТМА, при установлении недавно диагностированной метастатической скучной клеточной карциномы (SCC) после значительной диагностической неопределенности. 59-летний мужчина с сахарным диабетом, зависящим от

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… markers Hemoglobin (13.5–18.0 g/dL) 10.5 8.5, nadir 8.6 Platelets (130–400 × 10³/μL) 57–66 57, nadir 118 LDH (84–246 U/L) 571 571, peak 334 Haptoglobin (34–200 mg/dL) <20 <20, nadir <20 Reticulocyte count (0.5–2.0%) 8.7 8.9, peak 2.9 Peripheral smear Rare spherocytes … MRI, magnetic resonance imaging Although the peripheral blood smear showed rare spherocytes and few schistocytes, the remaining hematologic studies raised concern for a microangiopathic process. …

Klinefelter's syndrome presenting with leg ulcers.

A 54-year-old man of Persian origin presented to our department with a 1-year history of ulcers on the right leg that had been unresponsive … mean corpuscular hemoglobin volume, and red blood cell distribution width (16%). Blood smear showed spherocytes, slight hypochromia, anisocytosis, macrocytosis, and microcytosis. Blood chemistry values were taken for iron (4 micro g/dL [normal range 40-150 micro g/dL]), transferrin (193 mg/dL [normal range 220-400 mg/dL]), ferritin (1128 ng/mL [normal range 1 …

Hematology and Immune-Related Disorders

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… Iron deficiency may also cause increased fragmentation. 18 Spherocytes are smaller cells that are the product of immune-mediated removal of antibody-coated parts of the erythrocyte membrane, after which the cell is reconfigured into a sphere. … Spherocytes are less able to deform, making passage through the spleen more difficult. …

Haematological Diseases in the Tropics

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The choice of new treatments in autoimmune hemolytic anemia: how to pick from the basket?

Autoimmune hemolytic anemia (AIHA) is defined by increased erythrocyte turnover mediated by autoimmune mechanisms. While corticosteroids remain first-line therapy in most cases of warm-antibody AIHA, cold agglutinin disease is treated by targeting the underlying clonal B-cell proliferation or the classical complement activation pathway. Several new established or investigational drugs and treatment regimens have appeared during the last 1-2 decades, resulting in an improvement of therapy options but also raising challenges on how to select the best treatment in individual patients. In severe w

Understanding Rare Anemias: Emerging Frontiers for Diagnosis and Treatment.

Background-This review provides a comprehensive overview of rare anemias, emphasizing their hereditary and acquired causes, diagnostic advancements, and evolving treatment strategies. It outlines the significance of rare anemias within public health, historical challenges in recognition and treatment, and the role of European initiatives like ENERCA and EuroBloodNet in advancing care. Content-This document discusses diagnostic technologies like next-generation sequencing and the impact of artificial intelligence, alongside the promising avenues of gene therapy, targeted drug treatments, and st

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… HS is easily diagnosed with a simple morphology examination on a stained blood smear when circulating spherocytes are evident ( Figure 7 ). Genetic study has identified the most frequent mutations in HS to be SPTB (41%), followed by ANK1 (32%), SLC4A1 (18%), SPTA1 (4.5%), and EPB42 (4.5%). …

Evans syndrome: clinical perspectives, biological insights and treatment modalities.

Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura with a positive direct anti-human globulin test. It is classified as primary and secondary, with the frequency in patients with autoimmune hemolytic anemia being 37%-73%. It predominates in children, mainly due to primary immunodeficiencies or autoimmune lymphoproliferative syndrome. ES during pregnancy is associated with high fetal morbidity, including severe hemolysis and intracranial bleeding with neurological sequelae and death. The clinical presenta

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… Characterization of laboratory features requires a complete blood count and direct examination of peripheral blood; anemia, thrombocytopenia, reticulocytosis, poikilocytosis, mainly due to the presence of spherocytes, 5 , 9 increased indirect bilirubin and lactate dehydrogenase and a positive direct …

Hematology

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… Disappear after splenectomy BURR CELLS (echinocytes)—uremia, artifact SPUR CELLS (acanthocytes)—chronic liver disease, abetalipoproteinemia, malabsorption, anorexia nervosa SPHEROCYTES —due to loss of membrane surface area. …

Hematology

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… Disappear after splenectomy BURR CELLS (echinocytes)—uremia, artifact SPUR CELLS (acanthocytes)—chronic liver disease, abetalipoproteinemia, malabsorption, anorexia nervosa SPHEROCYTES —due to loss of membrane surface area. …

Резюме МКАРЕ 2023 77th Национальный конгресс &quot; СИААРТИ &quot;

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2020 ACVIM Forum On Demand Research Abstract Program.

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Р

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Резюме книги для 27th Конгресс Европейской ассоциации гематологии

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Abstracts from the 37th Annual Meeting of the Society of General Internal Medicine, 2014, San Diego, CA, USA.

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ISEV2021 Abstract Book

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