Metformin-Induced Hemolysis in a Glucose-6-Phosphate Dehydrogenase-Deficient Patient: A Case Report.

Metformin is a first-line medication used in the treatment of type 2 diabetes mellitus along with other conditions such as insulin resistance and polycystic ovarian syndrome. Overall, metformin appears to be well tolerated with a low incidence of side effects; however, in certain high-risk populations, it can trigger a hemolytic crisis. This case report describes a middle-aged man who was initiated on metformin for new-onset diabetes, following which he had an acute hemoglobin drop and was diagnosed to be having a hemolytic crisis requiring hospitalization. He was diagnosed with a deficiency of the enzyme glucose-6-phosphate dehydrogenase (G6PD) on admission. Extensive workup was done to rule out other causes of hemolysis, all of which came back to be negative. The offending agent was stopped and the patient received supportive care after which he improved. This case highlights a rare, yet important, side effect of metformin that needs to be observed in certain individuals, especially patients with G6PD deficiency. Routine testing of high-risk populations known to be G6PD deficient should be considered before initiating metformin.

The Public Health Risks of ß-Hemolytic Bacillus pumilus Bacteria Resistant to Gastrointestinal Conditions from Medicinal Plant.

In numerous countries, the utilization of plants for both nutritional and therapeutic purposes is a common practice. However, the inadvertent use of these plants can pose risks due to their active molecules or microbiota. The traditional use of Herniaria glabra L. (H. glabra) plant in treating various diseases is well-known; however, its application in yogurt production raises concerns. In this study, Bacillus pumilus isolated from H. glabra was identified through 16s rRNA sequencing and MALDI-TOF MS (Matriks Assisted Lazer Desorption Ionization Time of Flight Massspectrometry). The bacterium's resistance under simulated gastrointestinal tract (GIT) conditions was assessed, followed by investigations into its aggregation ability, antibiotic resistance, hemolytic activity, and antagonistic potential through in vitro tests. The study revealed that B. pumilus exhibited 100% resistance to GIT conditions. Notably, the bacterium demonstrated strong autoaggregation (34.48%) and coaggregation abilities (49.82% for Escherichia coli, 49.13% for Listeria monocytogenes), signifying a potent aggregative potential. Sensitivity to most tested antibiotics was observed, while no antagonistic activity against tested bacteria was evident. Furthermore, the bacterium exhibited ß-hemolytic activity, indicative of potential virulence. The findings suggest that this resistant yet virulent bacterium, with its hemolytic activity, could disrupt the GIT balance, posing serious health risks. The study underscores the need for caution and awareness regarding the potential dangers posed by bacteria in plant microbiota in herbal therapies.

Thrombotic Microangiopathy in Pregnancy: Current Understanding and Management Strategies.

Thrombotic microangiopathy (TMA) represents a heterogeneous group of disorders characterized by microvascular thrombosis and end-organ damage. Pregnancy-associated thrombotic microangiopathy (p-TMA) has emerged as a distinct clinical entity with unique diagnostic challenges. Identifying the specific form of p-TMA is critical for appropriate and timely management. This review offers a comprehensive overview of the various forms of thrombotic microangiopathies associated with pregnancy, highlighting our current understanding of their pathophysiology and the evolving landscape of diagnosis and treatment for each.

Diagnostic and Therapeutic Strategies in Evans Syndrome: A Case Report and Literature Review.

Evans syndrome (ES) is characterized by a combination of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). Immune dysregulation, which results in the development of antibodies against blood cells, is its defining feature. ES being a diagnosis of exclusion requires a thorough workup to rule out other probable illnesses like lymphoproliferative diseases and systemic lupus erythematosus (SLE). We present the case of a 38-year-old male who experienced shortness of breath, chest discomfort, and generalized weakness. His medical history included recurrent anemia, thrombocytopenia, and pulmonary tuberculosis in remission. Hemolysis, thrombocytopenia, and a large pericardial effusion were discovered during the physical examination and investigations. An initial treatment strategy that included pericardiocentesis was performed. In combination with AIHA and ITP, the clinical and laboratory findings strongly suggested ES, which improved with prednisolone therapy. First-line treatments consist of corticosteroids and intravenous immunoglobulin; refractory cases may also require rituximab, thrombopoietin receptor antagonists, and sirolimus. Achieving remission and lowering relapse rates need careful patient monitoring and customized treatment programs.

Risk of low levels of blood group antibodies mediating hemolysis in ABO-incompatible neonates with negative three hemolysis tests.

Objective: To explore the risk of low-level blood group antibody-mediated hemolysis in ABO-incompatible newborns with negative three hemolysis tests, aiming to assist in the identification and management of neonatal jaundice. Methods: A retrospective case-control study was performed in 892 children with jaundice. The patients were divided into three groups: group I, ABO compatible, negative three hemolysis tests; group II, ABO incompatible, negative three hemolysis tests; and group III, ABO incompatible, positive three hemolysis tests. We analyzed the differences in clinical data, blood routine and biochemical laboratory results. Results: (1) Patients in group II had higher levels of mean corpuscular volume (MCV), standard deviation of red blood cell volume distribution width (RDW-SD), alanine aminotransferase (ALT), lactate dehydrogenase (LDH), alkaline phosphatase (ALP), and bile acid (BA) than those in group I (P < 0.05). However, there were no statistically significant differences in the MCV, ALT, ALP and BA levels between groups II and III (P > 0.05). (2) Mean corpuscular hemoglobin concentration (MCHC) >359.5 g/L, cell volume distribution width (RDW-CV) >15.95%, and reticulocyte count (RET) >4.235% were identified as independent predictors of positive hemolysis test results (P < 0.001). The combination of MCHC, RDW-CV, and RET% yielded an AUC of 0.841. Conclusion: Low-level blood group antibody-mediated hemolysis may occur in ABO-incompatible neonates even when three hemolysis tests are negative. Changes in liver function parameters must be monitored. The combination of MCHC, RDW-CV, and RET% can be used to improve the detection rate of HDN.

Microangiopathic Hemolytic Anemia as a Paraneoplastic Syndrome in a Patient with Metastatic Gastric Cancer.

Cancer-associated microangiopathic hemolytic anemia (CA-MAHA) is a rare paraneoplastic syndrome. The most effective approach to treating CA-MAHA is to address the underlying malignancy. Documented cases of CA-MAHA are limited to fewer than 50 patients in the literature. Herein, we present a 51-year-old female patient who developed CA-MAHA as a complication of gastric adenocarcinoma. Despite receiving neoadjuvant and adjuvant chemotherapy for gastric cancer, the patient experienced disease progression with metastatic lesions in the liver, pancreas, and other sites. This report highlights the challenges in diagnosing and distinguishing CA-MAHA from other similar conditions such as disseminated intravascular coagulation (DIC), hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), and rheumatological paraneoplastic syndromes. Additionally, it concludes that CA-MAHA is associated with a poor prognosis and limited clinical benefit from treatment, emphasizing the need for early diagnosis and effective management strategies.

Autoimmune hepatitis presenting as severe anemia.

Autoimmune hepatitis (AIH) is relatively rare in children. Herein, our case demonstrates a unique presentation of AIH in a previously healthy 18-year-old female presenting with a mild cough, fatigue, and severe anemia (hemoglobin 2.9 g/dL). Initial evaluation revealed jaundice and scleral icterus, prompting transfer of care and further testing, which demonstrated severe microcytic anemia, pancytopenia, elevated liver enzymes, direct hyperbilirubinemia, and marked splenomegaly. Concern for autoimmune hemolytic anemia resulted in a delayed diagnosis. The combination of triple antibody positivity (anti-nuclear antibodies, anti-actin, and anti-liver-kidney microsomal-1) and liver histology findings confirmed the diagnosis of AIH. Intravenous methylprednisolone was initiated to induce remission. Due to pancytopenia and persistently elevated international normalized ratio, tacrolimus was chosen as the maintenance immunosuppression instead of azathioprine. This case highlights several significant considerations for clinicians, including the importance of a timely clinicopathologic diagnosis, the severe anemia presentation secondary to hypersplenism, and the rare finding of triple autoantibody-positive AIH.

The efficacy and safety of eculizumab in patients and the role of C5 polymorphisms.

Eculizumab is an orphan drug with indications for extremely rare autoimmune disorders. It is primarily prescribed for use in patients with paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome; but is also highly effective in the treatment of myasthenia gravis, among others. By binding to the C5 protein in the complement system, eculizumab effectively inhibits cellular hemolysis and autoimmune reactions. Despite this effective treatment, some patients reported no improvement in symptoms. Genetic sequencing revealed three distinct C5 mutations in the non-responders and these polymorphisms appeared to be most prevalent among Japanese, Korean and African populations. Here, we present an overview of the current and potential future applications of eculizumab, as well as the disadvantages of eculizumab treatment in patients with C5 polymorphisms.

An expert discussion on the atypical hemolytic uremic syndrome nomenclature-identifying a road map to precision: a report of a National Kidney Foundation Working Group.

The term atypical hemolytic uremic syndrome has been in use since the mid-1970s. It was initially used to describe the familial or sporadic form of hemolytic uremic syndrome as opposed to the epidemic, typical form of the disease. Over time, the atypical hemolytic uremic syndrome term has evolved into being used to refer to anything that is not Shiga toxin-associated hemolytic uremic syndrome. The term describes a heterogeneous group of diseases of disparate causes, a circumstance that makes defining disease-specific natural history and/or targeted treatment approaches challenging. A working group of specialty-specific experts in the thrombotic microangiopathies was convened to review the validity of this broad term in an era of swiftly advancing science and targeted therapeutics. A Delphi approach was used to define and interrogate some of the key issues related to the atypical hemolytic uremic syndrome nomenclature.

Exploring the antimicrobial and antioxidant properties of Lentzea flaviverrucosa strain E25-2 isolated from Moroccan forest soil.

The alarming rise in antimicrobial resistance (AMR) has created a significant public health challenge, necessitating the discovery of new therapeutic agents to combat infectious diseases and oxidative stress-related disorders. The Lentzea flaviverrucosa strain E25-2, isolated from Moroccan forest soil, represents a potential avenue for such research. This study aimed to identify the isolate E25-2, obtained from soil in a cold Moroccan ecosystem, and further investigate its antimicrobial and antioxidant activities. Phylogenetic analysis based on 16S rRNA gene sequences revealed the strain's classification within the Lentzea genus, with a sequence closely resembling that of Lentzea flaviverrucosa AS4.0578 (96.10% similarity). Antimicrobial activity in solid media showed moderate to strong activity against Staphylococcus aureus ATCC 25923, Bacillus cereus strain ATCC 14579, Escherichia coli strain ATCC 25922, Candida albicans strain ATCC 60193 and 4 phytopathogenic fungi. In addition, ethyl acetate extract of this isolate demonstrated potent antimicrobial activity against 7 clinically multi-drug resistant bacteria. Furthermore, it demonstrated antioxidant activity against 2,2-diphenyl-1-picrylhydrazyl (DPPH) and 2,2'-azino-bis (3-ethylbenzothiazoline-6-sulfonic acid) (ABTS) free radicals, as well as a significant increase in ferric reducing antioxidant power. A significant positive correlation was observed between antioxidant activities and total content of phenolic compounds (p < 0.0001), along with flavonoids (p < 0.0001). Furthermore, gas chromatography-mass spectrometry (GC-MS) analysis revealed the presence of amines, hydroxyl groups, pyridopyrazinone rings, esters and pyrrolopyrazines. The Lentzea genus could offer promising prospects in the fight against antibiotic resistance and in the prevention against oxidative stress related diseases.

Ravulizumab in Atypical Hemolytic Uremic Syndrome: An Analysis of 2-Year Efficacy and Safety Outcomes in 2 Phase 3 Trials.

Rationale & Objective: Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) caused by complement dysregulation. Ravulizumab is a C5i approved for the treatment of aHUS. This analysis assessed long-term outcomes of ravulizumab in adults and pediatric patients with aHUS. Study Design: This analysis reports 2-year data from 2 phase 3, single-arm studies. Setting & Participants: One study included C5i-naïve adults (NCT02949128), and the other included 2 cohorts of pediatric patients (C5i-naïve and those who switched to ravulizumab from eculizumab [pediatric switch patients]; NCT03131219). Exposure: Patients received intravenous ravulizumab every 4-8 weeks, with the dose depending on body weight. Outcomes: The primary endpoint in the studies of C5i-naïve patients was complete TMA response, which consisted of platelet count normalization, lactate dehydrogenase normalization, and ≥25% improvement in serum creatinine concentrations from baseline, at 2 consecutive assessments ≥4 weeks apart. Analytical Approach: All analyses used descriptive statistics. No formal statistical comparisons were performed. Results: In total, 86 and 92 patients were included in efficacy and safety analyses, respectively. Complete TMA response rates over 2 years were 61% and 90% in C5i-naïve adults and pediatric patients, respectively. The median increase in estimated glomerular filtration rate from baseline was maintained over 2 years in C5i-naïve adults (35 mL/min/1.73 m2) and pediatric patients (82.5 mL/min/1.73 m2). Most adverse events and serious adverse events occurred during the first 26 weeks. No meningococcal infections were reported. Improvement in the Functional Assessment of Chronic Illness Therapy - Fatigue score achieved by 26 weeks was maintained over 2 years. Limitations: Limitations were the small sample of pediatric switch patients and limited availability of genetic data. Conclusions: Long-term treatment with ravulizumab is well tolerated and associated with improved hematologic and renal parameters and quality of life in adults and pediatric patients with aHUS.

This research tested a drug called ravulizumab for the treatment of atypical hemolytic uremic syndrome (aHUS). aHUS is a rare disease that causes clots in tiny blood vessels. This can damage the kidneys and other organs. We analyzed data from 2 clinical trials in which children and adults with aHUS received ravulizumab through a tube placed in a vein (intravenous line). They received ravulizumab every 4-8 weeks depending on their weight. We found that treating patients for 2 years with ravulizumab was associated with improved blood health, kidney function, and quality of life and was well tolerated. These results support ravulizumab as a long-term treatment for people with aHUS.

[Clinical characteristics and genetic profile of complement system in renal thrombotic microangiopathy in patients with severe forms of arterial hypertension].

BACKGROUND: The spectrum of diseases characterized by the development of renal thrombotic microangiopathy (TMA) encompasses the malignant hypertension (MHT). TMA in MHT has conventionally been regarded as a variation of secondary TMA, the treatment of which is restricted to the stabilization of blood pressure levels, a measure that frequently fails to prevent the rapid progression to end-stage renal disease in patients. Nevertheless, there exists a rationale to suggest that, in certain instances, endothelial damage in MHT might be rooted in the dysregulation of the complement system (CS), thereby presenting potential opportunities for the implementation of complement-blocking therapy. AIM: To study clinical manifestations and genetic profile of CS in patients with morphologically confirmed renal TMA combined with severe AH. MATERIALS AND METHODS: 28 patients with morphologically verified renal TMA and severe AH were enrolled to the study. Patients with signs of microangiopathic hemolysis and thrombocytopenia were not included in the study due to possible compliance with the criteria for atypical hemolytic uremic syndrome (aHUS). The prevalence of rare genetic defects (GD) of the CS was assessed by molecular genetic analysis (search for mutations in the clinically significant part of the human genome - exome) by next-generation sequencing technology (NGS). RESULTS: GD of CS were detected in a quarter of patients. Rare genetic variants classified as "likely pathogenic" including defects in CFI, C3, CD46, CFHR4, CFHR5 genes were detected in five cases. Two patients were found to have chromosomal deletions containing CFH-related proteins genes (CFHR1, CFHR3). CONCLUSION: Rare variants of CS genes linked to aHUS were found in 25% of patients with renal TMA, the genesis of which was originally thought to be secondary and attributed to MHT, with partial or complete absence of hematological manifestations of microangiopathic pathology. The key to confirming TMA associated with MHT, particularly in the absence of microangiopathic hemolysis and thrombocytopenia, elucidating its nature, and potentially effective complement-blocking therapy in patients with GD of CS, appears to be a genetic study of CS combined with a morphological study of a renal biopsy.

Identification of mutations resulting in de-repression of the intermedilysin gene by sequential mutagenesis of its promoter region in Streptococcus intermedius.

Streptococcus intermedius secretes the human-specific cytolysin intermedilysin (ILY), a crucial factor in the pathogenicity of this bacterium. Previously, we reported that a lactose phosphotransferase repressor (LacR) represses ily expression and that its mutation increases ILY production. Interestingly, UNS40, a strain isolated from a liver abscess, produces high levels of ILY despite the absence of mutations in the lacR promoter and coding regions. Our results showed that a G > A mutation at the - 90th position from the transcription start point in the UNS40 ily promoter region increased hemolytic activity and decreased the binding ability to LacR. To elucidate the regions involved in the repression of ily expression, we generated mutant strains, in which point or deletion mutations were introduced into the ily promoter region, and then compared their hemolytic activity. Among the point mutations, -120 C > A and -90 G > A and their flanking mutations increased hemolytic activity. These results indicated that these mutations may increase the virulence of S. intermedius.

Microbiological Characteristics of Clinically Isolated Staphylococcus aureus with Different Hemolytic Phenotypes in China.

Purpose: This study aimed to investigate the microbiological characteristics of clinically isolated Staphylococcus aureus with different hemolytic phenotypes in China. Materials and Methods: Using the three-point inoculation method, the hemolytic phenotypes of 1295 clinically isolated S. aureus strains were detected and categorized. Antimicrobial susceptibility testing of all strains was performed using a VITEK 2 Compact System. After sample size matching, plasma coagulase activity, catalase activity, mRNA expression of hemolysin genes (hla, hlb, hlc, and hld), biofilm formation, growth kinetics, inflammatory response of macrophages and cytotoxicity of S. aureus with different hemolytic phenotypes using the rabbit plasma kit, the catalase test on slides, qRT-PCR, crystal violet staining, the microcultivation assay, the ELISA kits, and the CCK-8 assay, respectively. Results: Seven categories of hemolytic phenotypes were identified. Accordingly, strains were categorized into seven different groups, including S. aureus with complete hemolytic phenotype (SCHP), S. aureus with weak hemolytic phenotype (SWHP), S. aureus with incomplete hemolytic phenotype 1 (SIHP-1), SIHP-2, SIHP-3, SIHP-4 and SIHP-5, the last three of which were reported for the first time. Except for the hemolytic phenotype, all seven groups differed in clinical isolation rates, antibiotic resistance profile, plasma coagulase activity, mRNA expression of hemolysin genes, biofilm formation, growth kinetics, inflammatory response of macrophages, and cytotoxicity. Conclusion: S. aureus with different hemolytic phenotypes have distinctive microbiological characteristics. Clinical microbiologists need to be vigilant about the hemolytic phenotypes when culturing S. aureus strains, and actively enhance communication with clinicians to optimize the treatment of infection.

Loxosceles reclusa Envenomation Causing Acute Hemolytic Anemia: A Case Report on Loxoscelism.

This case report has the main objective of providing education surrounding the presentation, evaluation, diagnosis, and treatment of Loxosceles reclusa envenomation and presenting a case of loxoscelism that occurred in an adult that subsequently presented to the emergency department. A secondary objective of this case report is to add to the literature of images bite wound images associated with loxoscelism that resulted in inpatient admission and treatment for acute hemolytic anemia.

Secondary Evans Syndrome Presenting With Lupus Anticoagulant.

Evans syndrome (ES), characterized by autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP), often poses diagnostic challenges due to its varied etiology and clinical presentation. We present a case of secondary ES in a 41-year-old male with a history of AIHA and ITP, who presented with lower extremity erythema, warmth, and sensation of chest pressure. Initial laboratory investigations revealed thrombocytopenia, mild anemia, and a prolonged activated partial thromboplastin time (aPTT), prompting further evaluation. Subsequent testing revealed positive lupus anticoagulant (LA), anti-cardiolipin antibodies, and anti-beta-2-glycoprotein 1 antibodies, along with lower extremity deep vein thrombosis (DVT) and bilateral pulmonary embolism (PE). Treatment with therapeutic anticoagulation led to clinical improvement, highlighting the importance of recognizing hypercoagulable states in ES patients. This case underscores the significance of comprehensive differential diagnosis and timely intervention in optimizing outcomes for patients with ES.

[Cold agglutinin syndrome associated with infectious mononucleosis: A case report]. / Síndrome por aglutininas frías secundario a mononucleosis infecciosa: reporte de un caso.

Background: Cold agglutinin syndrome (CAS) is a hemolytic anemia mediated by antibodies, mainly IgM, whose maximum activity occurs at 4 °C. It happens secondary to infectious, autoimmune or neoplastic diseases, due to the formation of antibodies that cross-react against erythrocyte antigens, particularly of the I system. Here, we describe a case of CAS associated to Epstein-Barr virus (EBV) reactivation in a patient with primary human immunodeficiency virus (HIV) infection. Clinical case: 22-year old man with no medical history, hospitalized due to mononucleosis and anemic syndrome. Hemoglobin of 3.7 g/dL and elevation of lactate dehydrogenase were documented. In the peripheral blood smear it was observed spherocytosis, polychromasia and nucleated erythrocytes. EBV infection was confirmed with serology and viral load, as well as seronegative HIV infection with positive viral load. The C3d monospecific direct antiglobulin test was positive and an irregular antibody screening revealed the presence of an anti-I antibody. The patient received transfusion support and conservative treatment, with remission of the symptoms 2 weeks after admission. Conclusions: Cold agglutinin syndrome is a rare, potentially fatal complication of infectious mononucleosis, which should be considered in the face of findings suggestive of hemolysis in order to initiate support measures in a timely manner.

Introducción: el síndrome por aglutininas frías (SAF) es una anemia hemolítica mediada por anticuerpos principalmente de tipo IgM, cuya máxima actividad se da a 4 °C. Se presenta en el contexto de enfermedades infecciosas, autoinmunes o neoplásicas por la formación de anticuerpos que tienen reacción cruzada contra antígenos eritrocitarios, particularmente del sistema I. En este trabajo presentamos un caso de SAF asociado a reactivación del virus de Epstein-Barr (VEB) en un paciente con primoinfección por el virus de la inmunodeficiencia humana (VIH). Caso clínico: hombre de 22 años, sin antecedentes patológicos, hospitalizado por síndrome mononucleósico y anémico. Presentó hemoglobina de 3.7 g/dL y elevación de lactato deshidrogenasa. En el frotis de sangre periférica se observó esferocitosis, policromasia y eritrocitos nucleados. Se confirmó infección por VEB con serología y carga viral, así como infección por VIH seronegativa, con carga viral positiva. La prueba de antiglobulina directa monoespecífica a C3d fue positiva y el rastreo de anticuerpos irregulares demostró un anticuerpo anti-I. El paciente recibió soporte transfusional y tratamiento conservador, con remisión del cuadro a las 2 semanas de su ingreso. Conclusiones: el SAF es una complicación poco frecuente de la mononucleosis infecciosa, potencialmente mortal, la cual debe ser considerada ante hallazgos sugestivos de hemólisis con la finalidad de iniciar medidas de soporte de forma oportuna.

Recent data on atypical hemolytic uremic syndrome associated with pregnancy, kidney transplantation or hypertensive emergency / Syndrome hémolytique et urémique atypique dans sa forme associée à la grossesse, la transplantation rénale ou l'urgence hypertensive : données récentes.

Les microangiopathies thrombotiques (MAT) sont un groupe hétérogène de pathologies caractérisées à différents degrés par une anémie hémolytique mécanique, une thrombopénie et une occlusion microvasculaire histologique. Les MAT peuvent se compliquer de multiples atteintes d'organe, principalement neurologiques et néphrologiques (définissant alors le syndrome hémolytique et urémique). La classification fondée sur la physiopathologie de la maladie a mis en évidence l'importance de la dérégulation de la voie alterne du complément dans les SHU atypiques (SHUa) (non-liés aux infections). Cependant, la physiopathologie et le traitement de nombreuses formes de SHUa dans des contextes spécifiques (tels que la grossesse, la transplantation rénale ou l'urgence hypertensive) restent mal caractérisés. Dans cet article, nous reprenons les avancées diagnostiques et thérapeutiques récentes dans ces trois formes de SHUa aux contextes spécifiques. Nous montrons les spécificités de ces formes et décrivons les enjeux actuels et futurs de leur prise en charge.

Thrombotic microangiopathies (TMA) are a heterogeneous group of disorders characterized to varying degrees by mechanical hemolytic anemia, thrombocytopenia and histological microvascular occlusion. TMA can be complicated by multiple organ disorders, mainly neurological and nephrological (defining the hemolytic uremic syndrome, HUS). Classification based on disease pathophysiology has highlighted the importance of dysregulation of the alternative complement pathway in atypical (non-infection-related) HUS. However, the pathophysiology and treatment of many forms of aHUS in specific contexts (such as pregnancy, renal transplantation or hypertensive emergencies) remain poorly characterized. In this article, we review recent diagnostic and therapeutic advances in these three forms of aHUS in specific contexts. We show the specificities of these forms and describe the current and future challenges of their management.

A Rare Presentation of Glucose-6-Phosphate Dehydrogenase Deficiency.

Approximately 400 million individuals globally experience glucose-6-phosphate dehydrogenase (G6PD) insufficiency, an enzymatic condition that may be hazardous. Because of mutations in the G6PD gene, which result in functional variants alongside a variety of biochemical and clinical symptoms, this condition is an X-linked hereditary genetic disorder. Our case is that of a 12-year-old male child who presented with acute liver failure and later on, exhibited signs of hemolysis as well. We had to rule out the possibilities of acetaminophen toxicity and hepatitis A before reaching the conclusion that an underlying G6PD deficiency was being exacerbated by viral infection and simultaneous ingestion of non-steroidal anti-inflammatory drugs (NSAIDs).

Clinical and laboratory profile of patients with positive direct antiglobulin test attended at a university hospital in Goias, Brazil.

OBJECTIVE: To clinically and laboratory characterize patients with a positive direct antiglobulin test (DAT) treated at the Hospital das Clínicas of the Federal University of Goiás (HC-UFG). METHODS: A retrospective, descriptive, cross-sectional study was carried out collecting data from medical records of patients with a positive DAT who were treated at HC-UFG between August 2021 and August 2022. RESULTS: Eighty-four patients with positive polyspecific DAT results were screened in the clinical laboratory. Fifty-four patients had a laboratory profile compatible with autoimmune hemolytic anemia (AIHA), however, among these, 16 patients already had a diagnosis of AIHA in their medical records. The most common symptoms present among AIHA patients were pallor, asthenia, fatigue and dyspnea. For the remaining patients, the most common symptoms were severe thrombocytopenia, anemia, renal dysfunction, fever, myalgia, headache, thrombosis, asthenia, hematuria and joint pain. Only one patient had primary AIHA, that is, he had no evident underlying disease. The majority of AIHA patients (75 %) underwent corticosteroid therapy with 60 % having a positive response. For patients without AIHA, prednisone was the most frequently prescribed medication in 17 (25 %) patients, followed by hydroxychloroquine (14 patients - 20.1 %). CONCLUSION: It is essential to evaluate patients with positive DAT in detail in order to understand the real clinical case. The DAT serological result alone does not arrive at a conclusive diagnosis of AIHA, and so it must be evaluated in conjunction with both clinical data and other laboratory tests, such as hemoglobin concentration and hemolysis tests (reticulocytes, lactate dehydrogenase and/or haptoglobin).