Cause Of Hemolysis Research Articles

NonImmune hemolytic anemia secondary to vitamin B12 deficiency-A case report.

This case report emphasizes the crucial need to include vitamin B12 deficiency in the differential diagnosis of hemolytic anemia, despite its rarity as a presentation. The case illustrates that non-immune hemolytic anemia can occur secondary to severe vitamin B12 deficiency, which can be effectively treated with vitamin B12 supplementation. Early recognition and comprehensive evaluation are essential for identifying this uncommon yet significant cause of hemolysis, ensuring prompt and appropriate treatment to improve patient outcomes.

Outcomes of patients with thrombotic thrombocytopenic purpura treated in an intensive care unit.

Background Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder characterized by widespread microthrombosis that can predispose to multiple organ failure. The literature is sparse on the outcomes of critically ill patients with TTP managed in intensive care units (ICUs). We aimed to determine the mortality of ICU patients admitted with TTP and evaluate the predictors of survival. We also compared the incidence of nosocomial infection among those who did or did not receive plasma exchange (PE). Methods We conducted a retrospective study in a tertiary ICU. Two authors screened patients for eligibility from the hospital information system based on peripheral smear reports. Adult critically ill TTP patients managed in ICU were included. Patients with a diagnosis of haemolytic uraemic syndrome, autoimmune causes of haemolysis and pregnancy-related conditions, etc. were excluded. Two authors extracted data from medical charts. No imputation of missing variables was done. Non-parametric statistics were used to report data. Statistical analyses were performed using Stata version 16. Results Of the 535 records that were screened, 33 patients were deemed eligible. Mortality among TTP patients was 14 (42%). The women to men ratio was 7:3. At admission, greater degree of anaemia, thrombocytopenia, and higher lactate dehydrogenase levels were observed in non-survivors compared to survivors (5.4 g/dl [4.8-7.1] v. 7.6 g/dl [6.1-8.9], p=0.05; 17x103 μl v. 21x103 μl, p=0.63; and 2987 (1904-3614) U/L v. 2126 U/L (1941-3319), p=0.71; respectively]. Nineteen (57%) patients had acute kidney injury (AKI), of which 11 survived: 6 recovered completely from renal failure and 5 progressed to end-stage renal disease. Nosocomial infection rates were not different among those receiving and not receiving PE therapy (7 [33%] v. 3 [25%], respectively). Conclusion TTP is more common in women and has a high mortality. Older age, low haemoglobin and higher platelet transfusions are predictors of poor survival. Nosocomial infection rates were similar irrespective of receiving PE therapy.

Collage of cases and brief review of the laboratory diagnosis and molecular testing in autoimmune haemolytic anaemia.

Autoimmune haemolytic anaemia (AIHA) is an acquired heterogenous clinical entity with variable presentations like acute haemolysis or mild, chronic haemolysis compounded with acute exacerbation in winters or fatal uncompensated haemolysis. A step-wise approach to the diagnosis and characterisation of AIHA should be undertaken, firstly the diagnosis of haemolysis followed by the establishment of immune nature with the aid of direct agglutination tests (DAT). Simultaneously the other causes of immune haemolysis need to be excluded too. In light of advancements in diagnostics, a wide array of investigations can be used like absolute reticulocyte count, bone marrow responsiveness index to establish the evidence of haemolysis, sensitive gel technology, enhanced DAT assays, e.g., modified DAT with low ionic strength saline solution (LISS) at 4°C, DAT assays utilizing reagents such as anti-IgA and anti-IgM and DAT by flowcytometry, to detect RBC bound autoantibodies (Abs) and monospecific DAT to establish immune causes of haemolysis and characterisation of the autoantibodies. The compensatory role of bone marrow and synchronous pathologies like clonal lymphoproliferation, dyserythropoiesis, fibrosis are important factors in the evolution of the disease and aid in the customisation of treatment modalities. The laboratory work up should aim to diagnose underlying diseases like chronic lymphoproliferative disorders, autoimmune disorders and infectious diseases. Also, tests like autoimmune lymphoproliferative syndromes (ALPS) screening panel and Next-generation sequencing (NGS) panel for RBC membrane disorders, RBC enzymopathies, and congenital dyserythropoietic aneamia have found their place. It is incumbent upon the clinicians to use the all-available diagnostic modalities for the accurate diagnosis, prognostication and customisation of the therapy.

Hb Mizuho Case Report; Early Genomic Testing Facilitates a Life Changing Diagnosis

Unstable variant hemoglobinopathies are an uncommon cause of hemolysis in the pediatric patient and may cause a delay in diagnosis if there is not a high index of suspicion. Hemoglobin (Hb) Mizuho is a rare unstable hemoglobinopathy caused by a pathogenic variant of the HBB gene with a severe phenotype. Here we report on the first known case of Hb Mizuho in Australia, presenting with features of acute and chronic hemolysis. The morphological features on blood film review, in conjunction with biochemical findings and other clinical features, did not immediately suggest an alternative diagnosis and a Next Generation Sequencing gene analysis approach was taken to investigate genes associated with red blood cell disorders and atypical uremic syndrome. The HBB Mizuho variant was detected and established the diagnosis. This report highlights the challenge of diagnosing Hb Mizuho on conventional testing and the need for early genomic testing to clarify a diagnosis.

Thoracic endovascular aortic repair for hemolysis 17 years after insertion of classical elephant trunk: a case report

BackgroundThe classical elephant trunk (ET) technique is a very useful surgical procedure; however, haemolysis in the aorta associated with ET has been previously reported. It normally occurs within several years after the surgery, and it is a rare case of rapidly progressing haemolysis 10 or more years after aortic arch replacement with ET.Case presentationA 53-year-old man with a history of Stanford type A aortic dissection (DeBakey type Is), who was treated with total arch aortic replacement and aorto-femoral bypass using a prosthetic graft 17 years ago, developed severe progressive haemolytic anaemia. The ET used for the initial surgery was narrowed, and mechanical haemolysis was suspected. We assumed that progressive mechanical haemolysis occurred because of degeneration of the prosthetic graft. Thoracic endovascular aortic repair was performed, and haemolysis and anaemia were mitigated postoperatively.ConclusionsHaemolysis occurred 17 years after the initial surgery with ET. When haemolysis is suspected in a patient with ET, it must be identified as a cause of haemolysis even if 10 years or more have passed since the ET was inserted. To prevent this complication, attention should be paid to an appropriate ET length and diameter to avoid folding of the ET, particularly when the true cavity diameter is small.

A-123 Increased Rate of Recurrent Hemolysis Is Associated With COVID-19 Infection and Propofol Administration in the Intensive Care Unit (ICU)

Abstract Background Hemolysis is a pre-analytic issue that challenges clinical laboratories. When a specimen with significant hemolysis is obtained in our laboratory, tests where the hemolysis threshold is exceeded are not reported and blood is recollected. Unfortunately, in a subset of patients, the recollected specimen is also hemolyzed (termed “recurrent hemolysis”). In these cases, the second recollection utilizes special collection and handling protocols (i.e., hand-carried to lab, plasma collection, etc.) to mitigate preventable causes of hemolysis. Lipemia is also a known contributor to specimen hemolysis. Mayo Clinic Rochester experienced an increased rate of hemolysis as well as recurrent hemolysis during the height of the COVID-19 pandemic (January 2022). The objective of this study was to investigate the increased rate of recurrent hemolysis and compare to a rate prior to the COVID-19 pandemic (January 2019). Methods Retrospective data analysis was performed for January 2019 (negative control) and January 2022. The number of Mayo Clinic Rochester hospital admissions, admitting diagnosis, medications administered, and number of test collections performed were obtained using reporting functionality in our Electronic Health Record (EHR), Epic. The Laboratory Information System, SoftLab, was utilized to obtain recollection rates due to hemolysis. Data were normalized to total patient admissions for the specified period to account for inter-year variability. R/R Studio was utilized to analyze and visualize the data. Pearson’s Chi-squared test for independence was performed on categorical variables (significance defined as P < 0.05). Results A 28% decrease in hospital admissions (n = 3958:Jan-22 vs n = 5517:Jan-19) and a 23% decrease in total specimens collected on hospitalized patients (n = 40 557:Jan-22 vs n = 52 373:Jan-19) was observed between Jan-19 to Jan-22. However, the laboratory observed a 6% increase in hemolyzed specimens (n = 256:Jan-22 vs n = 241:Jan-19). Recurrent hemolysis occurred in 24% (n = 62/256) of Jan-22 and 17% (n = 43/241) of Jan-19 hemolyzed specimens and required special handling protocols. COVID positive patients comprised 48% (n = 30/62) of those requiring special handling protocols in Jan-22. Further examination revealed 34% (n = 21/62) of Jan-22 and 12% (n = 5/43) of Jan-19 cases of recurrent hemolysis were due to lipemia (P < 0.01). Chart review highlighted a potential source of lipemia from the administration of propofol, a lipophilic sedative emulsion. Of those COVID positive cases with recurrent hemolysis due to lipemia (n = 15/30), 93% (n = 14/15) were receiving propofol at the time of collection (P < 0.001). Conclusion We observed increased rates of specimen hemolysis during the COVID-19 pandemic. While most hemolysis resolves upon specimen recollection, patients with recurrently hemolyzed specimens often require special specimen collection and handling protocols to mitigate hemolysis. Approximately half of the recurrent hemolysis cases in Jan 2022 were COVID-19 positive patients. Lipemia-induced hemolysis was also more frequent in this patient population presumably due to the administration of propofol, a lipophilic emulsion sedative commonly used for COVID positive patients requiring sedation and ICU-level care. It is critical to understand the variety of factors which may contribute to recurrent hemolysis, as intervening with specialized collection and handling protocols to reduce observed pre-analytical hemolysis is required to provide accurate and timely laboratory results.

Hemolytic Anemia Occurring 14 Years after Ascending Aortic Replacement for Acute Type A Aortic Dissection Due to Aortic Pseudoaneurysm Derived from Anastomotic Leakage: A Case Report.

A 64-year-old male patient who presented with symptoms indicative of hemolytic anemia was referred to our hospital. After obtaining the patient's history, it was found that hemolysis occurred 14 years after he underwent ascending aortic replacement for acute type A aortic dissection. Enhanced computed tomography revealed an aortic pseudoaneurysm at the proximal anastomosis, which was thought to be the cause of hemolysis. Furthermore, aortic valve regurgitation and dilatation of the sinus of Valsalva were also found on a transthoracic echocardiogram. Therefore, the Bentall procedure was performed. During the surgery, aortic pseudoaneurysm formation and vascular graft stenosis were observed. The postoperative course was uneventful, and hemolysis diminished soon after the surgery.

Patient-specific stent for hemolytic anemia due to a kinked ascending aortic graft.

Hemolytic anemia after thoracic aortic surgery is rare. A 69-year-old female patient, who underwent ascending aorta replacement for acute type A aortic dissection 7 years ago, was transferred from another hospital due to hemolytic anemia necessitating regular blood transfusions. After engaging in interdisciplinary discussions, we ruled out alternative medical diagnoses and treatments, ultimately identifying the kinked aortic graft as the primary cause of hemolysis. Due to the patient's comorbidity, it was deemed high-risk to perform a redo thoracic aorta surgery for the replacement of the kinked aortic graft. Therefore, we decided to insert a patient-specific bare-metal stent into the kinked aortic graft to alleviate the kinking and promote improvement in hemolysis. We decided to perform the stent procedure under local anesthesia. The bare-metal stent was designed to be approximately 15% larger than the size of the pre-existing kinked aortic graft. A stent measuring 32 mm × 80 mm was successfully inserted and ballooned within the patient's ascending aortic graft. As a result, the patient's hemolytic condition showed gradual improvement over time. In this way, we aim to share our experience of inserting a patient-specific bare-metal stent under local anesthesia in patients who exhibit mechanical hemolysis due to a kinked aortic graft, especially when redo surgery is considered high-risk.

Carbon monoxide as a clinical marker of hemolysis.

Carbon monoxide (CO)-based tests have precisely measured hemolysis for over 40 years. End-tidal CO was the primary marker in clinical hematology research, followed by carboxyhemoglobin. Quantification of CO reflects heme oxygenases degrading heme in a 1:1 stoichiometric ratio, making CO a direct marker of hemolysis. CO in alveolar air can be quantified using gas chromatography, whose high resolution allows detecting mild and moderate levels of hemolysis. CO can be elevated in active bleeding, resorbing hematoma, and smoking. Clinical acumen and other markers remain necessary to diagnose the cause of hemolysis. CO-based tests constitute an opportunity for bench-to-bedside technology transfer.

Prevalence of Glucose-6-Phosphate Dehydrogenase Deficiency in Male Newborn Infants and Its Relationship with Neonatal Jaundice in Thailand.

The study aimed to explore the prevalence of glucose-6-phosphate dehydrogenase (G-6-PD) deficiency among male newborn infants in northeastern Thailand and its relationship with neonatal jaundice (NJ). This prospective cohort study included male newborn infants with gestational age (GA) ≥35 weeks born between July 1, 2019, and March 1, 2021. Cord blood was sent for G-6-PD fluorescent spot test (FST) and results were reported as normal, partial, or complete deficiency. Infants with NJ would have blood tested for total serum bilirubin (TSB) level and other possible causes of NJ. Duration of phototherapy, length of hospital stays, and complications were documented. There were 922 male infants included in this study with 854 (93.1%) term and 63 (6.9%) preterm infants. FST showed 132 infants (14.4%) had G-6-PD deficiency. Incidence of NJ was significantly higher among infants with G-6-PD deficiency compared with infants with normal G-6-PD level (47.7 vs. 25.8%; relative risk [RR]: 2.62, 95% confidence interval [CI]: 1.79-3.82; p < 0.001). Regardless of G-6-PD level, preterm infants had significantly higher incidence of NJ than term infants (52.4 vs. 27.3%; RR: 2.93, 95% CI: 1.75-4.92; p < 0.001). Duration of phototherapy was significantly longer in infants with G-6-PD deficiency with NJ but hospital stays were similar. Infants with combined G-6-PD deficiency and other causes of hemolysis did not have higher TSB level than infants with isolated G-6-PD deficiency. Risk factors associated with NJ were G-6-PD deficiency and preterm infants, whereas more advance GA was associated with reduced risk for NJ. G-6-PD deficiency and preterm infants were important risk factors for NJ. Routine G-6-PD screening, close monitoring for signs of NJ in infant with risks, and appropriate parental counseling should be implemented. · G-6-PD deficiency increases risk of neonatal jaundice.. · Preterm infants have higher risk for neonatal jaundice.. · G-6-PD deficiency does not link with severe jaundice..

Frequency of ABO incompatibility as a cause of neonatal jaundice in neonatal unit of Ayub Teaching Hospital, Abbottabad

Background: Neonatal jaundice is a common problem encountered by neonatologists. After prevention of Rh-isoimmunization ABO incompatibility has become the most common cause of hemolysis. For infants delivered of 35 weeks gestation or later ABO incompatibility have been found to be the major risk factors for development of severe hyperbilirubinemia. Objective: To assess the frequency of ABO incompatibility as a cause of neonatal jaundice. Methodology: The current study was Cross-sectional descriptive study carried out at the Neonatal units of Ayub teaching hospital, Abbottabad. Study was carried out over a period of six months from 01-06-2011 to 30-11-2011. The calculated sample size based on using the WHO software was 246 patients. Investigations were done regarding mother and baby’s blood group, serum bilirubin levels (total, direct and indirect). Data was entered into computer using SPSS version 10. Results: A total of 246 neonates were included in this study. Mean age of the neonates was 4.62±4.09. Males were 157 (63.8%) and females were 89 (36.2%).

Depolarized Mitochondrial Membrane Potential and Elevated Calcium in Platelets of Sickle Cell Disease.

Hemolysis, a crucial feature of Sickle cell disease (SCD), is a key player for cellular activation leading to various complications including thrombosis. In response to hemolysis, platelets get activated and release components that are necessary for further platelet activation and aggregation. Thus, it is believed that platelets contribute to the development of thrombotic complications. Platelets in SCD are expected to be affected due to common cause of hemolysis. To measure the surface markers of platelets including P-Selectin, Phosphatidyl Serine and integrin αIIbβ3 in SCD patients and healthy controls in order to understand the status of the platelets in SCD. To measure the surface markers of activated platelets using flow cytometry. Since mitochondria and calcium play an important role in cellular functions, the mitochondrial membrane potential and calcium content of platelets in SCD were also evaluated using flow cytometry. In the present study, we have observed significant increase of calcium level in SCD platelets. Further, the loss of mitochondrial membrane potential in SCD platelets was found to be significantly higher when compared to platelets of healthy controls. Though the surface markers of activated platelets in SCD remain unchanged, increased level of calcium and mitochondrial membrane potential loss suggest that the platelets in SCD are more prone to become activated. In order to understand the status of the platelets in SCD, apart from the surface markers, it is also important to assess the calcium levels and mitochondrial membrane potential of platelets.

Drugs and Conditions That May Mimic Hemolysis.

Visual inspection of posttransfusion plasma for hemolysis is a key laboratory method in the investigation of possible acute hemolytic transfusion reactions (AHTRs). Many substances and physiologic conditions can mimic hemolysis in vitro. Isolated reports describe specific cases of interference, but a comprehensive listing is lacking. Using an illustrative case, we summarize available literature on substances and conditions that may mimic hemolysis in vitro. We further describe other substances and conditions that may discolor plasma but are unlikely to be mistaken for hemolysis on visual inspection. At least 11 substances and conditions have been reported to discolor plasma, in colors ranging from orange to red to brown, including relatively common therapies (eg, eltrombopag, hydroxocobalamin, iron dextran). Other substances are unlikely to be encountered in everyday practice but may mimic hemolysis in particular patient populations. Additional substances may cause plasma discoloration, ranging from blue to green to white, and are associated with a wide variety of therapies and conditions. An awareness of the possible preanalytic confounding factors that may mimic hemolysis can aid in the workup of a suspected AHTR. Review of the medical record, use of ancillary testing, and consideration for nonimmune causes of hemolysis can aid in ruling out AHTR.

Successful treatment of pyruvate kinase deficiency and inflammatory bowel disease comorbid with exocrine pancreatic insufficiency and hypocobalaminaemia in a cat

AbstractA 3‐year‐old Abyssinian cat presented with chronic diarrhoea and weight loss. Blood examination and abdominal ultrasound examination identified severe macrocytic hypochromic anaemia and thickened intestinal walls. Pyruvate kinase deficiency and inflammatory bowel disease (IBD) were diagnosed based on gene analysis and intestinal biopsy findings, respectively. Additional blood evaluation identified exocrine pancreatic insufficiency (EPI) and hypocobalaminaemia. To eliminate the primary cause of haemolysis, we performed splenectomy, which stabilised the progression of anaemia for approximately 37 days. However, anaemia progressed on day 43 after splenectomy; therefore, 1 mg/kg prednisolone (PO q24h) was administered for stabilising erythrocytes and management of IBD. Significant improvements in the severity of anaemia, echinocyte counts and gastrointestinal clinical signs caused by IBD and EPI were observed with gastrointestinal diet, cobalamin and pancreatic enzymes supplementation. Prednisolone dose was then tapered to 0.5 mg/kg PO every 72 hours. There was no relapse of severe anaemia or signs of IBD or EPI.

P1547: ACTIVATE-KIDS: MITAPIVAT IN CHILDREN WITH PYRUVATE KINASE DEFICIENCY WHO ARE NOT REGULARLY TRANSFUSED

Background: Pyruvate kinase (PK) deficiency is a rare, inherited disorder caused by PKLR gene mutations that is associated with chronic hemolytic anemia and lifelong complications. Clinical management includes red blood cell (RBC) transfusions and splenectomy, which only partially improves anemia. No pharmacotherapies are approved for use in children, and therapies targeting the underlying cause of hemolysis are needed. Mitapivat (AG-348), a first-in-class, oral, allosteric activator of the RBC PK enzyme, demonstrated significant and durable improvements in hemoglobin (Hb) and markers of hemolysis, hematopoiesis, and iron metabolism in adults with PK deficiency who were not regularly transfused (ACTIVATE NCT03548220, long-term extension study NCT03853798). Safety and efficacy data support the evaluation of mitapivat in children with PK deficiency who are not regularly transfused. Aims: Report the design of ACTIVATE-Kids (NCT05175105), a phase 3 study, which will evaluate the efficacy and safety of mitapivat in children with PK deficiency who are not regularly transfused. Methods: ACTIVATE-Kids, a global, multicenter, double-blind, placebo-controlled study, will stratify 30 children by age (1 to <6, 6 to <12, and 12 to <18 years); a minimum of 6 patients in each age group will be randomized (2:1) to mitapivat or placebo. The double-blind period comprises an 8-week dose-titration period and a 12-week fixed-dose period. A 5-year open-label period will follow the double-blind period. The study drug will be dosed (1–50 mg twice daily) according to patient age and weight, based on pharmacokinetic modeling data of adults with PK deficiency. Key inclusion criteria: ≥2 PKLR mutant alleles with ≥1 missense mutation, ≤5 RBC transfusions in the past year, Hb concentrations of ≤10 g/dL (12 to <18 years) or ≤9 g/dL (1 to <12 years). Key exclusion criteria: R479H homozygosity, 2 non-missense mutations without a missense mutation in PKLR, or prior stem cell transplantation. The primary endpoint is Hb response, defined as a ≥1.5 g/dL increase in Hb concentration from baseline that is sustained at ≥2 scheduled assessments at Weeks 12, 16, and 20 in the double-blind period. Secondary endpoints will evaluate the effect of mitapivat on Hb, hemolysis, erythropoiesis, iron metabolism and overload, safety, quality of life, and pharmacokinetics. Results: Global site recruitment is in-progress; an update on recruitment and patient enrollment will be provided. Summary/Conclusion: The phase 3 ACTIVATE-Kids study of children with PK deficiency who are not regularly transfused will evaluate treatment with mitapivat, a potential disease-modifying pharmacotherapy.

Metabolic Shades of S-D-Lactoylglutathione.

S-D-lactoylglutathione (SDL) is an intermediate of the glutathione-dependent metabolism of methylglyoxal (MGO) by glyoxalases. MGO is an electrophilic compound that is inevitably produced in conjunction with glucose breakdown and is essentially metabolized via the glyoxalase route. In the last decades, MGO metabolism and its cytotoxic effects have been under active investigation, while almost nothing is known about SDL. This article seeks to fill the gap by presenting an overview of the chemistry, biochemistry, physiological role and clinical importance of SDL. The effects of intracellular SDL are investigated in three main directions: as a substrate for post-translational protein modifications, as a reservoir for mitochondrial reduced glutathione and as an energy currency. In essence, all three approaches point to one direction, namely, a metabolism-related regulatory role, enhancing the cellular defense against insults. It is also suggested that an increased plasma concentration of SDL or its metabolites may possibly serve as marker molecules in hemolytic states, particularly when the cause of hemolysis is a disturbance of the pay-off phase of the glycolytic chain. Finally, SDL could also represent a useful marker in such metabolic disorders as diabetes mellitus or ketotic states, in which its formation is expected to be enhanced. Despite the lack of clear-cut evidence underlying the clinical and experimental findings, the investigation of SDL metabolism is a promising field of research.

Acute Hemolytic Anemia Induced by Levofloxacin in A Woman With G6PD Deficiency: A Case Report

Introduction: Glucose 6-Phosphate Dehydrogenase deficiency (G6PD) is an X-linked recessive disorder recognized as the most prevalent enzyme deficiency around the world. G6PD deficiency has a high prevalence in Iran, especially in the northern regions. As we know, hemolysis in G6PD patients was not reported with levofloxacin previously. Case Report: In this report, we introduce a 54-year-old G6PD deficient woman who experienced the symptoms of hemolytic anemia following completion of treatment with levofloxacin. Result: After ruling out other causes of hemolysis, by using the Naranjo scale, levofloxacin was considered as a possible cause of hemolysis. Conclusion: Though the hemolytic anemia induced by levofloxacin is extremely rare in G6PD deficient patients, drug-induced hemolytic anemia should be considered as one of the differential diagnoses. It would be appropriate to use an alternative antibiotic instead of levofloxacin in a G6PD deficient patient.

Predictors of Mortality and Outcomes of Liver Transplant in Spur Cell Hemolytic Anemia

BACKGROUND :Spur cell hemolytic anemia (SCHA) is a rare, acquired, non-immune hemolytic anemia of decompensated cirrhosis resulting from abnormal lipid composition of the red cell membrane. Treatment is limited to red cell transfusion. Data describing prognostic impact, outcomes of liver transplant, and clinical hematologic characteristics of SCHA are absent or limited; published data on SCHA are limited to single-patient case reports and small single-center case series. The independent prognostic impact of SCHA on patients with cirrhosis remains unclear, and it is not known if hemolytic anemia severity has a significant impact on survival of these patients. As a result, SCHA is not formally considered during liver transplant evaluation, and patients do not receive MELD exception points for this diagnosis.METHODS :We performed a multicenter, 24-year observational cohort study of patients with SCHA, retrospectively analyzing hepatic and hematologic parameters, independent predictors of mortality, and long-term outcomes of liver transplant. Strict diagnostic criteria for SCHA were applied, requiring all of the following: (1) decompensated cirrhosis; (2) anemia; (3) objective laboratory evidence of hemolysis; (4) no alternate contributing cause of hemolysis or acanthocytosis; and (5) chart documentation of a confirmed diagnosis of SCHA incorporating peripheral blood film examination. The primary outcome was mortality at 3 months after date of SCHA diagnosis. The impact of hemolytic parameters on 3-month mortality was evaluated utilizing multivariable logistic models. Observed mortality vs. expected mortality (per MELD-Na score and Child-Turcotte-Pugh class) was compared using standardized mortality ratios. Given the limited survival of this population, red cell transfusion dependence was defined as 4 or more units of red cells transfused during the 60-day peri-diagnostic period.RESULTS :Patients: 69 patients with SCHA were included (FIGURE 1). The median (interquartile range) age was 53 (42-59) years; 46.4% were female, and 11 (15.9%) received liver transplant. Alcohol contributed to the etiology of cirrhosis in 53 patients (76.8%). The median (IQR) survival from SCHA diagnosis of patients not receiving liver transplant was 58 (23-113) days. 39 patients (56.5%) were red cell transfusion-dependent. Hematologic parameters are described in TABLE 1.Outcomes of Liver Transplant: All 11 patients undergoing transplant had rapid and complete resolution of SCHA, with an improvement in median hematocrit from 22.1% to 34.6% post-transplant (P=0.001) (TABLE 2) and excellent post-transplant outcomes, with 9 patients still alive after 6.1 years median follow-up.Independent Predictors of Mortality in SCHA: In multivariable logistic models adjusting for age, sex, etiology of cirrhosis, active/recent variceal bleeding, and Child-Turcotte-Pugh score, transfusion dependence had an OR for 90-day mortality of 9.14 (95% CI, 2.46-34.00) and reduced pre-transfusion hematocrit had an OR of 4.73 (95% CI, 1.42-15.82) per 6% decrease; increased red cell transfusion requirement, reduced hemoglobin, increased lactate dehydrogenase, and increased indirect bilirubin were also independently predictive of higher 90-day mortality (FIGURE 2).Performance of MELD-Na and Child-Turcotte-Pugh Scores in Estimating 90-Day Mortality: MELD-Na and Child-Turcotte-Pugh scores consistently significantly underestimated 90-day mortality, with standardized mortality ratios (SMRs) >1 across all scores/classes [MELD-Na 20-29, SMR 2.42 (1.18-4.44); Child-Turcotte-Pugh class B, SMR 4.46 (1.64-9.90)], FIGURE 3.CONCLUSIONS :In this largest study of SCHA to date, SCHA was associated with substantial excess mortality than was predicted by MELD-Na or Child-Turcotte-Pugh scores. Several clinical and laboratory parameters of hemolytic anemia severity, including transfusion burden, hemoglobin, and markers of hemolysis were each independent predictors of 90-day mortality in SCHA. Despite its unique morbidity as a complication of decompensated cirrhosis, outcomes of liver transplant were excellent in all 11 patients undergoing this intervention, with total resolution of SCHA and no evidence of recurrence. These findings should promote greater awareness of SCHA as a clinical entity and broader consideration of MELD exception points for afflicted patients when transplant-eligible. [Display omitted] DisclosuresAl-Samkari: Novartis: Consultancy; Amgen: Research Funding; Argenx: Consultancy; Rigel: Consultancy; Dova/Sobi: Consultancy, Research Funding; Agios: Consultancy, Research Funding; Moderna: Consultancy.

Hemolysis following a mitral valve prosthesis surgery: etiology, diagnosis and treatment

Domas Grigoravičius1 1 Vilnius University, Faculty of Medicine, Vilnius, Lithuania Abstract Introduction: anemia caused by heart valve prostheses is not a common but significant type of hemolytic anemia, despite the lack of information on this complication in the literature. There is no clear pathogenesis of the etiology of this condition. The primary mechanism of hemolytic anemia after valve replacement surgery is regurgitation due to valve leakage. Aim: this work aims to review the literature on the etiology, diagnosis, and treatment of valve-induced hemolytic anemia. Literature search strategy: the literature search was conducted using the PubMed and Google Scholar databases. The following keywords and their combinations were used: “hemolysis,” “valve replacement,” “treatment,” “diagnosis,” “surgery”. Conclusions: the diagnosis of hemolytic anemia caused by valve prostheses is problematic because it can be an early and late postoperative complication, and the condition must be differentiated from other possible causes of hemolysis even after the detection of hemolytic markers. Medication is sufficient to treat mild hemolytic anemia caused by valve prostheses, but surgical treatment and replacement of a defective valve are the most common. Keywords: valve replacement; hemolytic anemia; postoperative complication.

Factors affecting the severity of RH Incompatibility newborn

Rh incompatibility is a not common pediatric problem, that cause morbidity and mortality in children, and it is an important cause of hemolysis, anemia and jaundice in newborn. To study Rhesus hemolytic disease, its severity, its complications; to evaluate if ABO incompatibility is protective or not, so to find out the efficacy of anti-D globulin ; to evaluate the efficacy of phototherapy and exchange transfusion as a treatment.&#x0D; This study was done on neonates with jaundice, seventy five patients (1-10 days old) who had Rh incompatibility were studied during period from the first of January 2008 to the 30th of June 2008.&#x0D; History was taken about age, gender and gestational age of the patients, determination of gestational age and hepatosplenomegally as a cause of extramedullary hematopoiesis. Investigations done for patients were hemoglobin, total serum bilirubin, reticulocyte count, blood group and Rh, and direct coombs test. From 75 patients studied, 55 patients (73%) required treatment for jaundice;25 of them (46%) required only phototherapy due to mild degree of hemolysis, and 30 of them (54%) required exchange transfusion with phototherapy due to severe degree of hemolytic. The remaining 20 patients (27%) required observation alone.&#x0D; Family history of previous hemolysis was positive in 44 patients and it was a risk factor for having hemolysis in present pregnancy. Early evaluation of patients for jaundice was useful in early recovery. ABO incompatibility in association with Rh incompatibility was not necessarily protective against hemolysis. The administration of anti-D globulin to the mother within first 72 hours after delivery was protective against sensitization.&#x0D; Early and proper management of of Rh incompatibility may reduce need for exchange transfusion. ABO incompatibility was not necessarily protective against hemolysis. Anti-D globulin administered to mothers within 3 days after delivery was protective against sensitization, History of hemolytic in previous siblings is considered as a risk factor for present hemolytic in neonates with Rh incompatibility.&#x0D;