Hematological Features Research Articles

Think VEXAS: a case report of Vexas syndrome

VEXAS syndrome is a rare auto-inflammatory disorder characterized by heterogeneous inflammatory and hematologic features. First identified in 2020, it predominantly affects men over the age of 50. Clinical manifestations commonly include recurrent fever, weight loss, skin lesions, and diverse inflammatory presentations across multiple organ systems. Diagnosing VEXAS syndrome can be challenging due to the overlap of symptoms with other autoimmune and inflammatory conditions, often requiring extensive medical evaluation before a definitive diagnosis is made. This case report highlights these diagnostic challenges and underscores the importance of early recognition to improve clinical management and outcomes.

Development and Validation of Machine Learning Models for Predicting Tumor Progression in OSCC.

Development of a prediction model using machine learning (ML) method for tumor progression in oral squamous cell carcinoma (OSCC) patients would provide risk estimation for individual patient outcomes. This predictive modeling study was conducted of 1163 patients with OSCC from Hospital of Stomatology, SYSU and SYSU Cancer Center from March 2009 to October 2021. Clinical, pathological, and hematological features of the patients were collected. Six ML algorithms were explored, and model performance was assessed by accuracy, sensitivity, specificity, f1 score, and AUC. SHAP values were used to identify the variables with the greatest contribution to the model. Among the 1163 patients (mean [SD] age, 55.36 [12.91] years), 563 are from development cohort and 600 are from validation cohort. The Logistic Regression algorithm outperformed all other models, with a sensitivity of 94.7% (68.2%), a specificity of 55.3% (63.7%), and the AUC of 0.76 ± 0.09 (0.723) in the development (validation) cohort. The most predictive feature was neutrophil count. This study demonstrated ML models can improve clinical prediction of oral squamous cell carcinoma progression through basic information of patients. These tools could be used to provide individual risk estimation and may help direct intervention.

Thrombocytopenia and Other Hematological Features in COVID-19 Patients Referred to Shahid Mostafa Khomeini Hospital

Thrombocytopenia and Other Hematological Features in COVID-19 Patients Referred to Shahid Mostafa Khomeini Hospital

Myeloid neoplasm with concurrent BCR::ABL1 translocation, and JAK2 and SF3B1 co-mutations with severe fibrosis: a case report

Abstract Introduction/Objective Myeloproliferative neoplasms (MPNs) arise from hematopoietic stem cells with somatically altered tyrosine kinase signaling. Classification of MPNs is based on hematologic, histopathologic and molecular characteristics including the presence of BCR::ABL1 and JAK2 V617F. Methods/Case Report We present a 72-year-old male with a myeloid neoplasm and severe fibrosis, with concurrent BCR::ABL1 translocation, and co-mutations in JAK2 and SF3B1. Initially, the patient was diagnosed with anemia, thrombocytosis and folic acid deficiency and was prescribed supplemental folic acid. At follow up, normocytic anemia and thrombocytosis were seen. Bone marrow biopsy showed overtly fibrotic marrow with maturing trilineage hematopoiesis, markedly increased megakaryopoiesis and ring sideroblasts. Chromosome analysis and sequential GTG-fluorescence in situ hybridization (FISH) of metaphases using the dual-fusion three color probe BCR/ABL1- ASS1 revealed abnormal results. A t(9;22)(q34;q11.2) was detected along with an atypical FISH signal pattern with a single BCR::ABL1 fusion on the derivative chromosome 22 instead of the typical two fusions, resulting in loss of one BCR::ABL1 fusion signal including loss of the ASS1 on the derivative chromosome 9. Next-generation sequencing (NGS) analysis detected double driver mutations, JAK2 p. Val617Phe and SF3B1 p. Lys700Glu in the patient. JAK2 V617F mutation was further confirmed by real-time quantitative PCR (qPCR) with variant allele frequencies of 39.7%. Additionally, the patient was positive for BCR::ABL1 p190 fusion transcript (10%). The presence of JAK2 and SF3B1 co-mutations along with ring sideroblasts and the patient’s clinical presentation suggests the diagnosis of myelodysplastic/myeloproliferative neoplasm with thrombocytosis and SF3B1 mutation. Results (if a Case Study enter NA) NA Conclusion Although, the detection of t(9;22) by chromosome analysis is a diagnostic feature of chronic myeloid leukemia (CML), our case adds to the limited information on co-existence of BCR::ABL1 and JAK2 V617F with SF3B1 mutations in severe fibrosis. The frequency of co-occurrence, the temporal order of acquisition and clonal relationship of these alterations is poorly understood, and it is also unclear for this case since there is no prior bone marrow biopsy or diagnostic work up. Literature search of these rare cases show that some patients with similar findings progress to myelofibrosis as in this patient. It is possible that the presence of two mutated tyrosine kinase genes accelerates disease progression.

A study on subcutaneous infection model of colibacillosis in broilers: Selected immunological and hematological aspects

Colibacillosis is still a major challenge to poultry industry. This study examined selected immune and hematological features of broilers experimentally infected with E. coli via subcutaneous (sc) route. Fifty-one 5-week-old chickens were randomly assigned to three groups: two controls (NC and SCC) and one experimental (SC) group. The SC group was inoculated by O2 E. coli suspension via sc route in the inguinal region. Three days after inoculation, blood sampling was performed. Bacterial count of liver was determined. Histopathological samples of liver, lung, spleen, thymus, and bursa were also collected. Serum total protein and globulin significantly increased in birds of SC group as compared to control birds (P < 0.05). Birds in SC group also showed decreased albumin/globulin ratio. Serum levels of IL-6, IL-10, and IFN-γ remained statistically the same among groups. Lymphocytosis, monocytosis, thrombocytosis and heterophilia were present in SC group. Heterophils/lymphocytes ratio increased in SC birds. Left shift was observed in SC group with cells showing vacuolated cytoplasm that contained toxic granules. Histologically, lung involvement was less severe than liver. Lymphoid depletion was present in all examined lymphoid organs. E. coli growth was observed in liver samples from SC group but not in control birds. In conclusion, using sc route for induction of colibacillosis was associated with a systemic inflammatory response in broilers and histopathological lesions of thymus, bursa, and spleen three days post-infection. It seems that detection of changes in serum cytokine levels (IL-6, IL-10, and IFN-γ) should be performed in earlier hours of the disease in this model.

Distinct clinical profiles and patient outcomes in aCML and CNL.

The classification of atypical chronic myeloid leukemia (aCML) and chronic neutrophilic leukemia (CNL) as a single disease entity remains a topic of debate. To elucidate the characteristics of both entities, this retrospective cohort study was conducted, encompassing 36 cases of aCML and 18 cases of CNL. We discovered that aCML and CNL presented distinct blood counts, genetics, molecular profiles and outcomes. Specifically, hemoglobin levels (P < 0.001) and platelet counts (P < 0.001) were significantly lower in aCML cases than in CNL cases, with no significant difference in mean white blood cells (P = 0.637). The proportion of abnormal karyotypes was higher in aCML cases compared with CNL cases (P = 0.010). Notably, we found that aCML and CNL showed distinct gene expression profiles by transcriptome sequencing technology. The median follow-up duration for the entire cohort was 8months (rang 0.4 to 36.6months), and the median overall survival (OS) was significantly shorter in aCML cases (7.3months, 95%CI 5.4 to 20.5months) than in CNL cases (median OS not reached). The one-year OS rate for aCML patients was 31.0% (9/29), compared to 92.9% (13/14) for CNL patients. In conclusion, our study supports the notion that aCML and CNL are indeed distinct disease entities characterized by unique hematological features and clinical outcomes.

Clinico-haematological and Serum Biochemical Changes Associated with Foot and Mouth Disease in some Breeds of Cattle in Oyo State, Nigeria

Foot and mouth disease is considered one of the major hindrances to the growth of animal husbandry in Nigeria, with a projected annual loss of about 7 billion naira. We sought to establish the hematological and biochemical features in Nigerian indigenous cattle naturally infected with FMDV by comparing the hematological and biochemical features with breed, sex, and age. Forty cattle showing clinical manifestations and confirmed positive for FMD with the FMD rapid diagnostic kit were sampled for this study. Standard hematological and serum biochemical techniques were adopted for the analysis. Demography findings revealed more clinical FMD cases in the White Fulani breed (50%) compared to the least in Red Bororo (10%), females (60%) compared to males (40%), and adults (60%) compared to young (40%) cattle, respectively. There were generalized neutrophilia, lymphopenia, hypoproteinemia, increased ALT, and decreased AST enzymes when compared with reference ranges. All parameters analyzed regarding sex revealed no significance in their mean ± standard deviation, except neutrophils and absolute neutrophils, with p-values of 0.022 and 0.003, respectively. The results of all parameters regarding ages showed no statistical significance when comparisons of their mean ± SD were analyzed. We therefore conclude that there were more incidences of FMD in white Fulani, female, and adult cattle compared to other breeds, male and young animals, respectively, in Nigeria. The hematological and biochemical features appeared better in the Red Bororo breed, female and adult FMD-infected cattle compared to other breeds, male and young FMD-infected cattle respectively.

Assessment of Hematological Parameters in Mycobacterium Tuberculosis-Infected Patients at Hayatabad Medical Complex, Peshawar, Pakistan

Background: Mycobacterium tuberculosis is a major causative agent of pulmonary tuberculosis, significantly contributing to morbidity and mortality worldwide. In South Asia, including Pakistan, TB remains a primary cause of prolonged hospital stays and economic burden. It primarily affects the lungs and can alter various hematological parameters.Objective: To assess the prevalence and characterize the hematological features of pulmonary tuberculosis patients at Hayat Abad Medical Complex, Peshawar, Pakistan.Methods: A descriptive cross-sectional study was conducted from April to August 2024. A total of 220 sputum samples were collected from male and female patients of all ages with suspected TB. TB was confirmed in 31 patients using TB PCR. Hematological parameters, including WBC, RBC, Hb, HCT, MCV, MCH, and platelets, were analyzed using a Sysmex hematology analyzer. Data were analyzed using SPSS version 25.Results: The prevalence of TB was 14% (31/220). All confirmed TB patients had elevated WBC and neutrophil counts (100%), decreased Hb and HCT (100%), and reduced MCV (96%). Lymphopenia was present in 90% of the cases.Conclusion: Hematological deviations can be cost-effective markers for early TB diagnosis in resource-limited settings.

Construction of machine learning diagnostic models for cardiovascular pan-disease based on blood routine and biochemical detection data

BackgroundCardiovascular disease, also known as circulation system disease, remains the leading cause of morbidity and mortality worldwide. Traditional methods for diagnosing cardiovascular disease are often expensive and time-consuming. So the purpose of this study is to construct machine learning models for the diagnosis of cardiovascular diseases using easily accessible blood routine and biochemical detection data and explore the unique hematologic features of cardiovascular diseases, including some metabolic indicators.MethodsAfter the data preprocessing, 25,794 healthy people and 32,822 circulation system disease patients with the blood routine and biochemical detection data were utilized for our study. We selected logistic regression, random forest, support vector machine, eXtreme Gradient Boosting (XGBoost), and deep neural network to construct models. Finally, the SHAP algorithm was used to interpret models.ResultsThe circulation system disease prediction model constructed by XGBoost possessed the best performance (AUC: 0.9921 (0.9911–0.9930); Acc: 0.9618 (0.9588–0.9645); Sn: 0.9690 (0.9655–0.9723); Sp: 0.9526 (0.9477–0.9572); PPV: 0.9631 (0.9592–0.9668); NPV: 0.9600 (0.9556–0.9644); MCC: 0.9224 (0.9165–0.9279); F1 score: 0.9661 (0.9634–0.9686)). Most models of distinguishing various circulation system diseases also had good performance, the model performance of distinguishing dilated cardiomyopathy from other circulation system diseases was the best (AUC: 0.9267 (0.8663–0.9752)). The model interpretation by the SHAP algorithm indicated features from biochemical detection made major contributions to predicting circulation system disease, such as potassium (K), total protein (TP), albumin (ALB), and indirect bilirubin (NBIL). But for models of distinguishing various circulation system diseases, we found that red blood cell count (RBC), K, direct bilirubin (DBIL), and glucose (GLU) were the top 4 features subdividing various circulation system diseases.ConclusionsThe present study constructed multiple models using 50 features from the blood routine and biochemical detection data for the diagnosis of various circulation system diseases. At the same time, the unique hematologic features of various circulation system diseases, including some metabolic-related indicators, were also explored. This cost-effective work will benefit more people and help diagnose and prevent circulation system diseases.

A Study of the Clinical Profile of Iron Deficiency Anemia in Children With Sickle Cell Disease in a Tertiary Care Center.

Background Sickle cell disease (SCD) and iron deficiency anemia (IDA) are significant global health concerns, particularly in pediatric populations. This study investigates the prevalence, clinical impact, and management challenges of IDA in children with SCD. Methods A prospective observational study was conducted at the Department of Pediatrics, Jawaharlal Nehru Medical College(JNMC) and Acharya Vinoba Bhave Rural Hospital (AVBRH), Sawangi, from June 2022 to May 2024. The study included 60 children diagnosed with SCD. Comprehensive assessments were performed, including medical histories, physical examinations, and hematological investigations. Diagnosis of IDA was based on hemoglobin levels, mean corpuscular volume (MCV), red cell distribution width (RDW), peripheral blood smears, serum iron levels,and serum ferritin concentrations. Results Of the 60 participants, 15% exhibited iron deficiency. No significant gender differences were found in iron deficiency status. A significant association was observed between SCD type and the presence of pallor (p = 0.025) and sickle cell crises (p = 0.023). The study also found no significant association between SCD type and the presence of organomegaly (p = 0.079) or iron deficiency status (p = 0.675). The mean hemoglobin levels varied across SCD types, with sickle cell anemia patients showing lower levels than those with sickle cell trait or disease. Conclusion Diagnosing and managing IDA in children with SCD is complex due to overlapping hematological features and the risk of iron overload from frequent transfusions. Tailored diagnostic and therapeutic approaches are essential to improving hemoglobin levels, reducing complications, and enhancing the quality of life for affected children. This study provides valuable insights for refining clinical practices and emphasizes the need for a multidisciplinary approach to managing these intertwined conditions.

Interactions of electrophoretically silent hemoglobin Hekinan II [HBA1:c.84G>T] with various forms of α-thalassemias and other hemoglobinopathies: novel insights into the molecular and hematological characteristics and genetic origins

ABSTRACT To determine the molecular basis, genotype – phenotype relationship, and genetic origin of Hemoglobin (Hb) Hekinan associated with several forms of α-thalassemia and other hemoglobinopathies for a better understanding of its diverse clinical phenotypes. Seventeen participants with suspected abnormal Hb were studied. Hb analysis was performed using high-performance liquid chromatography (HPLC) and capillary electrophoresis (CE). Mutational and α-haplotypic and structural analyses were conducted, and the effects of mutations on globin-chain stability were determined. All participants harbored Hb Hekinan II (HBA1:c.84 G>T) co-inherited with another α-globin gene anomaly. Three novel genotypes, (ααHekinan/αCSα), (ααHekinan/αCSα,βA/βE), and (ααHekinan/αCSα,βE/βE), were characterized. Despite being co-inherited with both α- and β-Hb variants Hb Hekinan II led to minimal changes in erythrocyte parameters, suggesting a non-pathological nature. HPLC but not CE revealed a distinct small shoulder-like Hb pattern. Thai Hb Hekinan II was strongly associated with haplotype [+ - S + - - -] and the possibility of four different haplotypes, while two Burmese Hb Hekinan II were associated with haplotypes [± - S + - + -] and [± - S + - - -]. The novel genotypes identified provide a fresh perspective on Hb Hekinan II diversity. HPLC has superior identification capabilities for samples of Hb Hekinan II co-inherited with α-thalassemia. Thai and Burmese Hb Hekinan II have diverse origins.

The effects of time and anticoagulant type on hematologic values in cockatiels (Nymphicus hollandicus)

BackgroundAvian hematologic characteristics complicate evaluation; therefore, complete blood cell counts require manual analysis for best accuracy. Furthermore, the quality of samples may be affected by prolonged shipment. MethodsBlood was collected from the right jugular vein of twenty-four, 7-year-old (14 male, 10 female), wild-type cockatiels (Nymphicus hollandicus). Smears were prepared using fresh, untreated blood. The remainder was divided into dipotassium ethylenediaminetetraacetic acid (EDTA) and lithium heparin microtainers. A hematocrit tube and blood smear were prepared at baseline, 24, 48, and 72 hours after venipuncture. Packed cell volume (PCV), total solids (TS), estimated white blood cell (WBC) count, WBC differential (heterophils, lymphocytes, monocytes, eosinophils, basophils), hemolysis index (HI), and preparation quality were assessed and compared to baseline. After log transformation, the effects of anticoagulants across time points were compared. ResultsHeparinized samples had significant differences in WBC, heterophil, and lymphocyte counts at 48 hours, and PCV and TS at 72 hours compared to baseline. There was a positive proportional bias in PCV and TS from EDTA anticoagulant samples at 24, 48, and 72 hours, and from heparinized samples at 48 and 72 hours compared to baseline. The EDTA samples had increased PCV and TS compared to heparin at all time points, and higher WBC and heterophil counts at 48 hours compared to baseline. A type II methodological error cannot be excluded. ConclusionThese results suggest that the anticoagulant used and time spent in anticoagulant alters hematologic values in cockatiels, which may have significant clinical implications. The importance of sample processing within 24 hours and anticoagulant-specific reference intervals for hematologic variables should be considered.

Haematological features of telomere biology disorders diagnosed in adulthood: A French nationwide study of 127 patients.

Data on haematological features of telomere biology disorders (TBD) remain scarce. We describe haematological, extra-haematological characteristics and prognosis of 127 genetically confirmed TBD patients diagnosed after the age of 15. Ninety-three index cases and 34 affected relatives were included. At diagnosis of TBD, 76.3% of index cases had haematological features, half pulmonary features and a third liver features. At diagnosis, bone marrow failure (BMF) was present in 59 (46.5%), myelodysplastic syndrome (MDS) in 22 (17.3%) and acute myeloid leukaemia (AML) in 2 (1.6%) while 13 (10.2%) developed or worsened bone marrow involvement during follow-up. At diagnosis, compared to MDS/AML patients, BMF patients were younger (median 23.1 years vs. 43.8, p= 0.007), and had a better outcome (4-year overall survival 76.3% vs. 31.8%, p < 0.001). While frequencies and burden of cytogenetical and somatic mutations increased significantly in myeloid malignancies, some abnormalities were also observed in patients with normal blood counts and BMF, notably somatic spliceosome variants. Solid cancers developed in 8.7% patients, mainly human papillomavirus-related cancers and hepatocellular carcinomas. TBD is a multiorgan progressive disease. While BMF is the main haematological disorder, high-risk myeloid malignancies are common, and are, together with age, the only factors associated with a worse outcome.

Deep Learning-Based Blood Abnormalities Detection as a Tool for VEXAS Syndrome Screening.

VEXAS is a syndrome described in 2020, caused by mutations of the UBA1 gene, and displaying a large pleomorphic array of clinical and hematological features. Nevertheless, these criteria lack significance to discriminate VEXAS from other inflammatory conditions at the screening step. This work hence first focused on singling out dysplastic features indicative of the syndrome among peripheral blood (PB) polymorphonuclears (PMN). A deep learning algorithm is then proposed for automatic detection of these features. A multicentric dataset, comprising 9514 annotated PMN images was gathered, including UBA1 mutated VEXAS (n = 25), UBA1 wildtype myelodysplastic (n = 14), and UBA1 wildtype cytopenic patients (n = 25). Statistical analysis on a subset of patients was performed to screen for significant abnormalities. Detection of these features on PB was then automated with a convolutional neural network (CNN) for multilabel classification. Significant differences were observed in the proportions of PMNs with pseudo-Pelger, nuclear spikes, vacuoles, and hypogranularity between patients with VEXAS and both cytopenic and myelodysplastic controls. Automatic detection of these abnormalities yielded AUCs in the range [0.85-0.97] and a F1-score of 0.70 on the test set. A VEXAS screening score was proposed, leveraging the model outputs and predicting the UBA1 mutational status with 0.82 sensitivity and 0.71 specificity on the test patients. This study suggests that computer-assisted analysis of PB smears, focusing on suspected VEXAS cases, can provide valuable insights for determining which patients should undergo molecular testing. The presented deep learning approach can help hematologists direct their suspicions before initiating further analyses.

PSLBII-25 Impact of strain and sex on hematological and serum biochemical characteristics of broiler chickens in Ikorodu, Lagos, Nigeria

Abstract This experiment was conducted to evaluate the influence of strain and sex on the hematology and serum biochemistry of broiler chicken reared in Ikorodu area of Lagos, Nigeria. A total of 153 unsexed broiler birds containing 51 of Marshall, Arbor Acre and Cobb strains each were used for the experiment. The birds were reared under the same condition for 8 wk. Blood samples, collected from the jugular veins, were used to determine hematological parameters including Hemoglobin (Hb), packed cell volume (PCV), red blood cell (RBC), white blood cell (WBC), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), mean corpuscular hemoglobin concentration (MCHC), neutrophils (NEUT), lymphocyte (LYMP) monocytes (MON) and eosinophils (EOS). However, the serum biochemical indices include the total protein (PRO), serum glucose (GLU), serum albumin (ALB), uric acid (URIC), alkaline phosphatase (ALP) and Serum Cholesterol (CHOL) and creatinine. Statistical analysis revealed that Hb, WBC, EOS, MCHC and Serum cholesterol were significantly influenced by strains and sex while PCV, RBC, NEUT, LYMP, PRO, GLU, ALP and uric were significantly affected by sex of broiler chicken (Table 1-4).

The application of lung immune prognostic index in predicting the prognosis of 302 STS patients.

Soft tissue sarcoma (STS) are heterogeneous and rare tumors, and few studies have explored predicting the prognosis of patients with STS. The Lung Immune Prognostic Index (LIPI), calculated based on baseline serum lactate dehydrogenase (LDH) and the derived neutrophils/(leukocytes minus neutrophils) ratio (dNLR), was considered effective in predicting the prognosis of patients with pulmonary cancer and other malignancies. However, the efficacy of the LIPI in predicting the prognosis of patients with STS remains unclear. This study retrospectively reviewed patients with STS admitted to our center from January 2016 to January 2021. Their hematological and clinical characteristics were collected and analyzed to construct the LIPI specific to STS. The correlations between various predictive factors and overall survival (OS) were examined using Kaplan-Meier and Cox regression analyses. Independent risk factors for OS were identified using univariate and multivariate analyses. Finally, a LIPI nomogram model for STS was established. This study enrolled 302 patients with STS, of which 87 (28.9%), 162 (53.6%), and 53 (17.5%) were classified into three LIPI-based categories: good, moderate, and poor, respectively (P < 0.0001). The time-dependent operator curve showed that the LIPI had better prognostic predictive ability than other hematological and clinical characteristics. Univariate and multivariate analyses identified the Fédération Nationale des Centres de Lutte Contre le Cancer grade (FNCLCC/G), tumor size, and LIPI as independent risk factors. Finally, a nomogram was constructed by integrating the significant prognostic factors. Its C-index was 0.72, and the calibration curve indicated that it could accurately predict the three- and five-year OS of patients with STS. The decision and clinical impact curves also indicated that implementing this LIPI-nomogram could significantly benefit patients with STS. This study explored the efficacy of the LIPI in predicting the prognosis of 302 patients with STS, classifying them into three categories to evaluate the prognosis. It also reconstructed a LIPI-based nomogram to assist clinicians in predicting the three- and five-year OS of patients with STS, potentially enabling timely intervention and customized management.

Hematological features and alternate diagnoses in critically ill thrombotic antiphospholipid syndrome patients.

Severe thrombotic antiphospholipid syndrome (APS) frequently affects the kidney, heart, and central nervous system. The precise frequency, clinical picture, differential diagnoses, and outcome of APS-related hematological involvement are lacking, especially in patients requiring ICU admission. This study aimed to describe the hematological manifestations associated with critically ill thrombotic APS patients and catastrophic antiphospholipid syndrome. This French, national, multicenter, retrospective study, conducted, from January 2000 to September 2018, included all APS patients admitted to 24 participating centers' ICUs with any new thrombotic manifestation. The prevalence of hematological manifestations and their associated outcomes were studied. One hundred and thirty-four patients, female 72%, median [IQR] age 45 [34-56] years, with 152 episodes were included. Anemia was present in 95% of episodes and thrombocytopenia in 93%. The lowest values for hemoglobin and platelets were 7.1 [6.3-8.8] g/dL and 38 [21-60] g/L, respectively. The lowest platelet count below 20g/L was significantly associated with a higher in-ICU mortality rate (50%, p < 0.0001). A thrombotic microangiopathy syndrome (TMA) syndrome was seen in 16 patients (12%) and was associated with higher in-hospital mortality (p = 0.05). Median ADAMTS-13 levels were 44% [27-74]. Anti-ADAMTS13 antibodies were tested in 11 patients and found negative in all. A suspicion of heparin-induced thrombocytopenia (HIT) was raised in 66 patients but only four patients were classified as definite HIT. Disseminated intravascular coagulation (DIC) was seen in 51% of patients. Thrombocytopenia is very frequent in severe APS patients and may be related to TMA, HIT, or DIC. Deciphering the mechanisms of thrombocytopenia is decisive in CAPS patients. Key Points • Thrombocytopenia is the hallmark laboratory finding in CAPS. • A complete thrombotic microangiopathy pattern is infrequent in CAPS patients. • Alternate diagnoses of CAPS, especially heparin-induced thrombocytopenia, need to be adequately investigated.

Macular neurodegenerative and vascular changes on OCTA in sickle cell disease are not related to its ocular and systemic complications.

To evaluate macular abnormalities in sickle cell disease (SCD) with OCT-Angiography (OCTA) and to determine associations with sickle cell retinopathy (SCR) and clinical and laboratory characteristics. Complete ophthalmic examination was performed in consecutive SCD patients (HbSS, HbSC, HbSβ0 or HbSβ+ genotype), including fundoscopy and macular SD-OCT/OCTA scans. SCR stage was based on fundoscopic examination (without fluorescein angiography) instead of the Goldberg classification, since fluorescein angiography was only used in case of tentative diagnosis. Medical/ophthalmological history and hematologic characteristics were retrieved from medical records. 249 eyes of 137 patients were analyzed. The mean age was 33.3 ± 12.4 years (range 15-70). Non-proliferative SCR was present in 57 eyes (22.9%) and proliferative SCR in 36 eyes (14.5%). Macular thinning was present in 100 eyes (40.2%) and was associated with lower foveal vessel density (VD) of the superficial capillary plexus (SCP) and deep capillary plexus (DCP) and with enlargement of the foveal avascular zone (FAZ) area, perimeter and acircularity index (AI). Age and female sex were associated with lower (para)foveal VD in the SCP and DCP. No associations were found between SCR presence/severity and macular thinning or VD. Macular abnormalities were common, but did not result in visual impairment. No relation with SCR presence/severity was found. While OCTA-imaging is suitable for detecting maculopathy, it appears to have no diagnostic value in identifying patients at risk for SCR.

Exploring the Relationship between CD66c Expression and Clinico-Haematological Parameters in Pediatric B-Cell Acute Lymphoblastic Leukemia (ALL)

Background: Pediatric B-cell Acute Lymphoblastic Leukemia presents with diverse clinical and hematological manifestations. Understanding these characteristics is crucial for effective management and prognostication.  Methods: This study conducted as prospective observational study at Lady Hardinge Medical College and Kalawati Saran Children Hospital, New Delhi, from November 2017 to March 2019. This study enrolled 30 pediatric patients with B-cell ALL who underwent comprehensive evaluations including routine and specialized hematological tests, bone marrow aspiration (BMA), cytochemistry, and immunophenotyping. Clinical features, hematological parameters, and CD66c expression on lymphoblasts were assessed.  Results: Immunophenotyping revealed that 28 cases (93.3%) were CALLA positive (CD10 positive), while 2 cases (6.7%) were CALLA negative (CD10 negative). The age ranged from 1.5 to 12 years, with a 2:1 male to female ratio. The mean age was 4.8 years.. Common presenting complaints included fever (93.3%) and pallor (60.0%). Hepato-splenomegaly (60.0%) and lymphadenopathy (60.0%) were the most frequent clinical findings. Hematological findings showed moderate to severe anemia (mean hemoglobin 7.19 g/dL), with 56.7% of patients having hemoglobin levels between 4.0-7.9 g/dL. Leukocytosis (&gt;10,000/cumm) was observed in 53.3% of cases, with 20% having total leukocyte counts &gt;50,000/cumm. Thrombocytopenia (platelet count ≤ 25,000/cumm) was noted in 56.7% of patients. Immunophenotyping revealed CD66c expression &gt;20% gated in 46.7% of cases, correlating with more severe clinical features and poorer hematological parameters.  Conclusion: Pediatric B-cell ALL exhibits a spectrum of clinical and hematological features. Elevated CD66c expression correlates with aggressive disease presentation and worse clinical outcomes, suggesting its potential utility as a prognostic marker. Early identification of these parameters can aid in risk stratification and tailored therapeutic approaches.

Quality and stability studies of red blood cell concentrates from umbilical cord blood compared to their adult counterparts.

Prematurity is a significant health issue due to its incidence and associated complications. Anemia is common in extremely preterm infants (EPI) and often requires transfusions. Red blood cells (RBC) from adult blood (AB) donors have been linked to oxygen-related complications in EPI, leading to the exploration of cord blood (CB) as an alternative source. However, standardization of CB-RBC manufacturing and comparison with AB-RBC characteristics are necessary before clinical studies can be conducted. This study investigated the quality and characteristics of leukoreduced, gamma-irradiated CB-RBC obtained using a commercial closed system from CB donations not meeting hematopoietic transplantation criteria. CB-RBC units were compared with AB-RBC units, both stored in saline-adenine-glucose-mannitol (SAGM). Various parameters, including hematological and biochemical characteristics, pH, 2,3-DPG levels, blood gases and potential toxicants, were evaluated during storage. CB-RBC units had acceptable initial quality parameters and a hematocrit (55±2%) comparable to AB-RBC. The main finding during storage was a faster rise in hemolysis compared to AB-RBC. Potassium (K+) significantly increased during storage in both sources. As expected, glucose levels decreased, and conversely, lactate levels increased, indicating similar patterns of anaerobic glycolysis during storage. pH decreased, affecting the oxygen dissociation curve due to reduced 2,3-DPG levels. After irradiation at 14 days of storage, CB-RBC were less stable as hemolysis and K+ significantly increased compared to AB-RBC at 24 hours. Phthalate concentrations, indicative of plasticizers, increased during storage, but significantly less in CB compared to AB-RBC. Most metals measured were within acceptable ranges. The quality of CB-RBC during storage is primarily influenced by levels of hemolysis and extracellular K+ content. Based on the analyzed parameters, we suggest that the expiration date for CB-RBC stored with SAGM should be set at 14 days, with transfusion occurring within <24 hours after irradiation.