Hemolytic Anemia Research Articles

Feto-maternal outcome of Rh-Negative pregnancy presenting in a tertiary healthcare centre

Background: The term "Rhesus incompatibility" describes the mismatched Rh types of the mother and the fetus. It is linked to the onset of hemolytic disease of the newborn (HDN) ranging from haemolytic anemia to hydrops fetalis and also related to maternal Rh sensitization. The disease incidence is currently declining globally, having dropped from 1.3%–1.7% in the 1980s to 0.17% in the 1990s1. Erythroblastosis foetalis is one of the most terrible consequences of an ABO incompatible or Rh incompatible pregnancy. About 10% of all Rh-negative pregnancies result in Rh incompatibility in the Rh-negative mother carrying a Rh-positive fetus. Methods: This observational study was carried out among 60 Rh negative pregnant women attending our antenatal clinic and delivered in our institution from October 2022 to March 2024.The Rh negative women were followed up with a series of investigations such as Indirect Coombs Test (ICT), MCA-PSV and with regular antenatal care (ANC). After birth neonates were followed up with blood grouping and Rh typing, direct coombs test (DCT), duration of phototherapy, duration of NICU stay , need for immunoglobulin and exchange transfusion were recorded to evaluate the maternal and neonatal outcomes.The data was collected and tabulated in Microsoft excel sheet and the percentages were calculated. Results: This study included a total of 60 Rh negative mothers ,where 48 (80%) of them were aged less than 30 years and 12 of them (20%) were more than 30 years of age. Antenatally, 51 (85%) women were ICT negative and received RAADP, 9(15%) women were ICT positive and anti D titres were less than 1:16 followed up with MCA-PSV which was normal/less than 1.5MoM. The total preterm births were 6(10%).The total admissions to NICU were 12(20%) in our study. All the 9 babies born to ICT positive mothers turned out to be Direct Coombs test (DCT) positive and were managed with double surface phototherapy. For 2(3.3%) babies, human immunoglobulin was given. Only 1(1.6%) baby required exchange transfusion. Conclusion: We conclude that, severe hyperbilirubinemia and hydrops foetalis, which were observed previously are drastically reduced with recent advances and the use of Routine antenatal Anti D prophylaxis (RAADP). Neonatal morbidity and mortality reduced drastically with newer advances like immunoglobulin reducing the need for exchange transfusion and better NICU care. ICT positivity of 15% despite of postnatal immunisation suggest that there is chances of silent fetomaternal haemorrhage during antenatal period, health care professionals should be more knowledgeble with prenatal screening, the value of blood grouping and Rh typing, anti-D immunisation following sensitising events like MTP, abortion, ectopic pregnancy, ECV and regular implementation of RAADP in clinical practice.

Post-COVID-19 complement-mediated TMA: A case report.

Systemic COVID-19 disease is associated with a variety of organ involvement in infected patients. A rarely reported complication is the induction of complement-mediated thrombotic microangiopathy (TMA). TMA is an extremely rare pathological condition that results in thrombosis in capillaries and small arterioles, due to an endothelial injury. It is often combined with thrombocytopenia, Coombs-negative hemolytic anemia, and end-organ damage. This case involves a patient who was admitted to our hospital for the purpose of diagnosis and treatment of acute kidney injury (AKIN 3) with severe proteinuria after a preceding SARS-CoV-2 infection. A 77-year-old male patient had COVID-19 pneumonia in January 2021 with the need of high-flow oxygen therapy in the intensive care unit. In March 2021, he was hospitalized again due to elevated serum creatinine levels and proteinuria. The patient exhibited normal vital parameters. A renal biopsy showed severe TMA. A diagnosis of COVID-19-associated TMA was made, and treatment with high-dose glucocorticoid therapy and plasma exchange was initiated. Additionally, therapy with eculizumab was established. Unfortunately, the kidney failure was initially progressive, so that hemodialysis (HD) was temporarily necessary. In May 2021, kidney function recovered to an estimated glomerular filtration rate of ~ 30 mL/min/1.73m2 corresponding to chronic kidney disease stage 3bA3 - 4A3. COVID-19-associated TMA is an extremely rare disease. TMA may be a possible long-term complication with the risk of end-stage renal disease if not properly diagnosed and treated.

Flawed Value Assessment of Mitapivat to Manage Hemolytic Anemia in Adults With Pyruvate Kinase Deficiency.

Flawed Value Assessment of Mitapivat to Manage Hemolytic Anemia in Adults With Pyruvate Kinase Deficiency.

A review of attitudes to urgent RhD-positive transfusions in female patients and the risk for hemolytic disease of the fetus and newborn.

A review of attitudes to urgent RhD-positive transfusions in female patients and the risk for hemolytic disease of the fetus and newborn.

Microangiopathic thrombocytopenia caused by vitamin B12 deficiency responding to plasma exchange.

A young adult African American female presented with normocytic microangiopathic haemolytic anaemia, elevated lactate dehydrogenase and thrombocytopenia. The patient responded to therapeutic plasma exchanges (TPE) for presumed thrombotic microangiopathy caused by thrombotic thrombocytopenic purpura (TTP). After relapsing, the patient was found to have pancytopenia, megaloblastic bone marrow and low vitamin B12 consistent with pernicious anaemia, which improved with intramuscular B12 and discontinuation of TPE. B12-deficient macrocytosis was not seen at presentation due to concomitant alpha-thalassaemia. Initial clinical/laboratory improvement is attributed to B12 present in TPE plasma. B12 deficiency can mimic TTP. Vigilance is needed regarding atypical presentations of pernicious anaemia.

Immune-related hemolytic anemia of cats - a modern approach to therapy

Immune-related hemolytic anemia of cats - a modern approach to therapy

Efficacy of sutimlimab for cold agglutinin disease in a patient on chronic hemodialysis.

Reports of cold agglutinin disease (CAD), an autoimmune hemolytic anemia, in dialysis patients are limited. Recently, sutimlimab for CAD was covered by insurance. Herein, we report a case in which sutimlimab was effective in the treatment of CAD in a patient undergoing hemodialysis (HD). The patient was a 73year-old Japanese man with an 11year history of HD for diabetic nephropathy. He was admitted to our hospital for examination and treatment of erythropoiesis-stimulating agent (ESA)-induced hyporesponsive anemia and fatigue, which was present in the previous year October to March when temperatures were cooler. The patient was diagnosed with hemolytic anemia based on decreased hemoglobin levels, elevated reticulocyte count, elevated lactate dehydrogenase levels, and decreased haptoglobin levels. Furthermore, he was diagnosed with CAD based on a positive direct antiglobulin test for C3 and cold agglutinin tests. The patient did not respond well to an elevated dialysate temperature or rituximab therapy. After initiating sutimlimab treatment, an increase in the hemoglobin level was observed despite a decrease in temperature, and his fatigue disappeared. Anemia in hemodialysis patients is generally renal; however, some ESA resistance exists, which may be due to hemolytic anemia. In this case, the use of sutimlimab was effective in controlling hemolytic anemia due to CAD.

Alectinib-induced Non-immune Hemolytic Anemia due to Erythrocyte Membrane Alterations: A Retrospective Evaluation

Alectinib-induced Non-immune Hemolytic Anemia due to Erythrocyte Membrane Alterations: A Retrospective Evaluation

Anti-C5 monoclonal antibody treatment showing pathological resolution of complement-mediated atypical hemolytic uremic syndrome: a case report

BackgroundNo reports have shown histological changes before and after anti-C5 monoclonal antibody treatment in patients with atypical hemolytic uremic syndrome (aHUS). Here, we report a rare case of complement-mediated aHUS with a complement factor H (CFH) mutation and anti-CFH antibodies who underwent multiple kidney biopsies.Case presentationA 53-year-old woman developed aHUS with CFH gene mutation [c.3572C > T (p. Ser1191 Leu)] and anti-CFH antibodies. Her father had succumbed to acute kidney injury (AKI) in his 30 s. She exhibited AKI, thrombocytopenia, and hemolytic anemia with schistocytes. After improving the platelet count with one session of plasma exchange, a kidney biopsy was performed one month after the onset of symptoms. Blood vessel thrombosis, obvious endothelial swelling, endocapillary hypercellularity, and subendothelial exudative lesions in the glomeruli and arterioles were detected. Anti-C5 monoclonal antibody treatment with eculizumab immediately improved disease activity. A second biopsy 3 months later revealed marked improvement of endothelial injuries with residual membrane double contours and exudative lesions. A third biopsy at 17 months after gradual improvement of kidney function showed a further decrease of double contours along with alterations of the exudative lesions to fibrous intimal thickening.ConclusionsThis is the first report showing the pathophysiology of aHUS in the kidneys and the efficacy of anti-C5 monoclonal antibody treatment by presenting serial kidney pathological features before and after anti-C5 monoclonal antibody treatment. Since her CFH mutation was considered the most important pathological condition, treatment centered on eculizumab was administered, resulting in a good long-term prognosis. In addition, kidney pathological resolution in aHUS occurred over 1 year after anti-C5 monoclonal antibody treatment.

PREVALENCE OF KELL BLOOD GROUP SYSTEM IN BLOOD DONORS ATTENDING A TERTIARY CARE CENTRE IN NORTHWESTERN INDIA

Objective: The Kell blood group system is amongst one of the clinically significant blood group system in blood transfusion, consisting of different types of antigens with high immunogenicity which can be a potential cause of serious transfusion reactions and hemolytic disease of fetus and newborn. Knowledge of the antigenic frequency is crucial to assess the risk of alloimmunisation and to guide the probability of finding antigen-negative donor blood, which can be useful for a patient with corresponding or multiple red cell alloantibodies. Methods: This is a retrospective study which was done at Department of Immunohematology and Transfusion medicine, SMS Medical College, Jaipur over a period of one year from 1st Jan to 31st Dec 2020. During the study period, blood both voluntary donors as well as replacement donors were typed for Kell antigens by automated red cell antigen typing by capture-R technology in neo immucor. Results: A Total of 9677(Nine Thousand Six Hundred Seventy Seven) Blood donors were typed for Kell Antigens. Out of these 9677 samples, 288 were Positive for Kell positive (K+) resulting in overall frequency of Kell (K) Antigen as 2.9%. Conclusion: This is the first study that set out to determine the prevalence of Kell antigens among Blood Donors in Northwestern India. These results appear to be useful in providing better care for patients by implementing tests that should become a routine in blood banks. The Kell system is very important in Transfusion medicine practice.

RBCs regulate platelet function and hemostasis under shear conditions through biophysical and biochemical means

AbstractRed blood cells (RBCs) have been hypothesized to support hemostasis by facilitating platelet margination and releasing platelet-activating factors such as adenosine 5′-diphosphate (ADP). Significant knowledge gaps remain regarding how RBCs influence platelet function, especially in (patho)physiologically relevant hemodynamic conditions. Here, we present results showing how RBCs affect platelet function and hemostasis in conditions of anemia, thrombocytopenia, and pancytopenia and how the biochemical and biophysical properties of RBCs regulate platelet function at the blood vessel wall interface and in the fluid phase under flow conditions. We found that RBCs promoted platelet deposition to collagen under flow conditions in moderate (50 × 103/μL) but not severe (10 × 103/μL) thrombocytopenia in vitro. Reduction in hematocrit by 45% led to increased bleeding in mice with hemolytic anemia. In contrast, bleeding diathesis was observed in mice with a 90% but not with a 60% reduction in platelet counts. RBC transfusion improved hemostasis by enhancing fibrin clot formation at the site of vascular injury in mice with severe pancytopenia induced by total body irradiation. Altering membrane deformability changed the ability of RBCs to promote platelet aggregation. RBC-derived ADP contributed to platelet activation and aggregation in vitro under pathologically high shear stresses, as observed in patients supported by left ventricular assist devices. These findings demonstrate that RBCs support platelet function and hemostasis through multiple mechanisms, both at the blood vessel wall interface and in the fluidic phase of circulation.

Autologous haematopoiesis stem cell transplantation (AHSCT) for treatment-refractory autoimmune diseases in children

ObjectivesTo evaluate the long-term effectiveness and safety of autologous haematopoiesis stem cell transplantation (AHSCT) for severe, refractory autoimmune diseases in paediatric patients.MethodsA single-centre study of consecutive children and adolescents with...

Multiple lung abscesses and cold agglutinin syndrome following coronavirus disease 2019: a case report

BackgroundCold agglutination syndrome is a subtype of autoimmune hemolytic anemia. The condition is referred to as “cold” because the antibodies become active and induce hemolysis at cold temperatures, typically 3–4 °C, which is not always the case in other kinds of autoimmune hemolytic anemia. Whereas primary cold agglutination syndrome may occur in the absence of underlying conditions, secondary cold agglutination syndrome is associated with the presence of underlying infections, including coronavirus disease 2019.Case presentationWe report the case of a 69-year-old Japanese woman with periodontitis who was referred to our hospital with complaints of brown-colored urine and chest pain. Her hemoglobin level was 6.1 g/dL. Computed tomography revealed multiple lung abscesses. Her direct antibody test results were positive (2+) for anti-complement direct antiglobulin and negative for immunoglobulin G, and her cold agglutinin titer was elevated at 1:4096. Workup for anemia revealed a positive result for cold agglutination syndrome. The patient had received the fourth dose of coronavirus disease 2019 vaccination. Nasopharyngeal swab test for detecting severe acute respiratory syndrome coronavirus 2 using a real-time reverse-transcription polymerase chain reaction gave a cycle threshold value of 42.3, and the level of virus-specific immunoglobulin G was elevated at 7.71 S/C (normal range −1.4 S/C).ConclusionA decrease in hemoglobin in patients with coronavirus disease 2019 may be associated with secondary cold agglutination syndrome. The patient was hypothesized to have developed multiple lung abscesses with secondary cold agglutination syndrome following coronavirus disease 2019. Thus, following coronavirus disease 2019, patients can develop secondary cold agglutination syndrome, which could worsen owing to associated bloodstream bacterial infections.

Serum microRNAs as new biomarkers for detecting subclinical hemolysis in the nonacute phase of G6PD deficiency

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is one of the most common enzymopathies worldwide. Patients with G6PD deficiency are usually asymptomatic throughout their life but can develop acute hemolysis after exposure to free radicals or certain medications. Several studies have shown that serum miRNAs can be used as prognostic biomarkers in various types of hemolytic anemias. However, the impact of G6PD deficiency on circulating miRNA profiles is largely unknown. The present study aimed to assess the use of serum miRNAs as biomarkers for detecting hemolysis in the nonacute phase of G6PD deficiency. Patients with severe or moderate G6PD Viangchan (871G > A) deficiency and normal G6PD patients were enrolled in the present study. The biochemical hemolysis indices were normal in the three groups, while the levels of serum miR-451a, miR-16, and miR-155 were significantly increased in patients with severe G6PD deficiency. In addition, 3D analysis of a set of three miRNAs (miR-451a, miR-16, and miR-155) was able to differentiate G6PD-deficient individuals from healthy individuals, suggesting that these three miRNAs may serve as potential biomarkers for patients in the nonhemolytic phase of G6PD deficiency. In conclusion, miRNAs can be utilized as additional biomarkers to detect hemolysis in the nonacute phase of G6PD deficiency.

RBCs Regulate Platelet Function and Hemostasis under Shear Conditions through Biophysical and Biochemical Means.

Red blood cells (RBCs) have been hypothesized to support hemostasis by facilitating platelet margination and releasing platelet-activating factors such as adenosine diphosphate (ADP). Significant knowledge gaps remain regarding how RBCs influence platelet function, especially in (patho)physiologically relevant hemodynamic conditions. Here we present results showing how RBCs affect platelet function and hemostasis in conditions of anemia, thrombocytopenia, and pancytopenia, and how the biochemical and biophysical properties of RBCs regulate platelet function at the blood-vessel wall interface and in the fluid phase under flow conditions. We found that RBCs promoted platelet deposition to collagen under flow conditions in moderate (50  103/L) but not severe (10  103/L) thrombocytopenia in vitro. Reduction in hematocrit by 45% led to increased bleeding in mice with hemolytic anemia. In contrast, bleeding diathesis was observed in mice with a 90% but not with a 60% reduction in platelet counts. RBC transfusion improved hemostasis by enhancing fibrin clot formation at the site of vascular injury in mice with severe pancytopenia induced by total body irradiation. Altering membrane deformability changed the ability of RBCs to promote platelet aggregation. RBC-derived ADP contributed to platelet activation and aggregation in vitro under pathologically high shear stresses, as observed in patients supported by left ventricular assist devices. These findings demonstrate that RBCs support platelet function and hemostasis through multiple mechanisms, both at the blood-vessel wall interface and in the fluidic phase of circulation.

Screening and Identification of Erythrocyte Antibodies: A Narrative Literature Review

Red blood cell (RBC) alloimmunization, the development of antibodies against foreign red blood cell antigens, is a critical concern in transfusion medicine. Alloantibodies can lead to adverse transfusion reactions, including hemolytic disease of the fetus and newborn (HDFN) and delayed hemolytic transfusion reactions (DHTR). This comprehensive literature review explores the intricacies of RBC alloimmunization, focusing on the screening and identification of erythrocyte antibodies. We delve into the prevalence and clinical significance of various alloantibodies, the underlying immunological mechanisms, and the evolution of laboratory techniques for their detection. Additionally, we discuss the challenges and future directions in managing alloimmunization, emphasizing the importance of personalized medicine and innovative approaches to ensure safe and effective blood transfusions.

Hemolytic versus malproductive anemia in large granular lymphocytic leukemia.

Hemolytic versus malproductive anemia in large granular lymphocytic leukemia.

Mixed Autoimmune Hemolytic Anemia - A Rare Case Report

Abstract Mixed autoimmune hemolytic anemia (MAIHA) is a rare clinical condition where both warm and cold antibodies cause red cell destruction, leading to anemia. MAIHA can present as a blood group discrepancy or crossmatch incompatibility, which can be very challenging for transfusion medicine services to diagnose and issuing of best-matched blood. Therefore, a comprehensive immunohematology workup, including a monospecific direct antiglobulin test, and indirect antiglobulin test is performed to determine the thermal amplitude and titer of autoantibodies. It is important to be aware of this phenomenon because, like warm AIHA and MAIHA should be treated immediately, with steroids. We hereby report a case of a 14-year-old girl presenting with a history of generalized weakness and dizziness; preliminary investigations revealed anemia, increased reticulocyte count, and antinuclear antibodies positive. Hence, further diagnosis of MAIHA associated with systemic lupus erythematosus was made. A high-dose steroid therapy was given to the patient, and significant improvement was seen clinically.

Cluster analysis of clinical manifestations assigns systemic lupus erythematosus-phenotype subgroups: A multicentre study on 440 patients

ObjectiveSystemic lupus erythematous (SLE) is a heterogenous disease characterised by a large panel of autoantibodies and a wide spectrum of clinical signs and symptoms that engender different outcomes. We aimed to identify distinct, homogeneous SLE patients’ phenotypes. MethodsThis retrospective study enrolled SLE patients meeting the Systemic Lupus International Collaborating Clinics (SLICC) classification criteria, enrolled in the French multicentre “APS (antiphospholipid syndrome) and SLE” Registry. Based on 29 variables selected to cover a broad range of clinical and laboratory (excluding autoantibodies) SLE manifestations, unsupervised multiple correspondence analysis followed by hierarchical ascendent-clustering analysis assigned different phenotypes. ResultsWe included 440 patients, mostly women (94.3%). Median age at SLE diagnosis was 24 (IQR 19–32) years. Cluster analysis yielded three distinct subgroups based on cumulative clinical manifestations, not autoantibody pattern. Cluster 1 (n=91) comprised mostly Caucasian patients, with APS-associated clinical and biological manifestations, e.g., livedo, seizure, thrombocytopaenia and haemolytic anaemia. Cluster 2 (n=221), the largest, included patients with mild clinical manifestations, mainly articular, more frequently associated with Sjögren's syndrome and with less frequent autoantibody-positivity. Cluster 3 (n=128) consisted of patients with the largest panel of SLE-specific clinical manifestations (cutaneous, articular, proliferative nephritis, pleural, cardiac and haematological), the most frequent autoantibody-positivity, low complement levels, and more often of Asian and sub-Saharan African origin. ConclusionThis unsupervised clustering method distinguished three distinct SLE patient subgroups, highlighting SLE heterogeneity.

Method for quantifying free hemoglobin, distinct from the hemoglobin-haptoglobin complex, in human serum

The measurement of free hemoglobin (free Hb) in blood is crucial for assessing the risk of organ damage in patients with hemolytic diseases. However, the colorimetric method, commonly used in clinical practice, does not distinguish between free Hb and the hemoglobin-haptoglobin complex (Hb-Hp) in the blood, instead reflecting the total Hb level. Although size-exclusion high-performance liquid chromatography (SEC-HPLC) can specifically measure free Hb, its clinical use is limited by long assay times. Here, we developed a novel assay method for the rapid quantification of free Hb in serum, distinguishing it from Hb-Hp, using a latex agglutination immunoturbidimetric assay (LATIA). This method could be used to measure free Hb in sera in the range of 1–100 μg/mL in approximately 15 min using an automatic biochemistry analyzer. Using Hb-spiked serum samples from healthy adults, there was a high correlation with Hb levels determined using the newly developed method and SEC-HPLC, indicating a high specificity for free Hb. This novel assay can be used to monitor levels of free Hb in patients with various hemolytic diseases and to design therapeutic strategies based on measured values. However, further studies are required to assess its clinical performance.