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Secondary antibody deficiencies (SADs) are characterized by impaired humoral immunity, which can cause recurrent and severe infections. Several factors may contribute to SAD development, making it difficult to establish a clear etiological classification. This heterogeneity also leads to clinical variability, further complicating patient management and treatment strategies. Various diagnostic and therapeutic algorithms are often adapted from those used in primary antibody deficiencies, potentially resulting in under- or over-treatment. Key points include the decision to initiate Immunoglobulin Replacement Therapy (IgRT) and the duration of the treatment. Given the increasing prevalence of SADs and the limited availability of immunoglobulin products, it is important to clarify when IgRT should be started. In this review, we summarize and update the different etiologies of SADs and propose a diagnostic algorithm applicable regardless of the underlying cause. We also examine the possible treatment options and diagnostic tools that can assist in making the correct therapeutic choice.

Deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive disease caused by ADA2 gene mutation that is characterized by three phenotype domains: vasculopathy and inflammation, hematological abnormality, and immunodeficiency. Most patients are pediatric patients; adult-onset patients are only occasionally reported. To describe a Chinese case of adult-onset DADA2 in a Chinese patient and explore the genotype and phenotype characteristics of adult-onset DADA2. We examined the clinical, serological, and genetic features of a Chinese adult-onset DADA2 patient. English literature on DADA2 was reviewed. The clinical and genetic characteristics of different age and mutation subgroups were compared. A Chinese Han male presented with recurrent fever, rash, immunodeficiency, and significant vascular events since the age of 25 years. Serum ADA2 activity was diminished, and genotyping revealed a unique compound heterozygous mutation of exon2-10del/exon7del in the ADA2 gene leading to complete exon 7 deletion. Treatment with a TNFα inhibitor achieved disease control. A total of 269 cases carrying 102 mutations were analyzed through a literature review. Adult-onset patients had few symptoms in all three clinical domains; vasculopathy and inflammation were the major symptoms. Patients with null mutations had early disease onset and more frequent hematological abnormalities and immunodeficiency. Patients in all subgroups responded well to TNFα inhibitors. We reported the first Chinese adult-onset DADA2 patient, with a unique mutation. Screening for and differentiation of DADA2 are recommended for patients of all ages, as they might become symptomatic later in life and treatment strategies differ from those of traditional vasculitis.Key Points• We report a novel compound heterozygous deletion mutations of exons 2–10 and exon 7, leading to complete loss of exon 7 in the ADA2 gene.• Adult-onset DADA2 patients had high similarity to systemic vasculitis.• Null mutations contribute to earlier disease onset and more aggressive disease.• We suggest screening for DADA2 in patients with significant central vasculitis, hematological abnormality and immunodeficiency.

Abstract The enduring SARS-CoV-2 pandemic necessitates robust tools for severity assessment. This study, conducted at Islamabad Diagnostic Center across Pakistan from January 2021 to August 2022, aimed to investigate hematological abnormalities among suspected SARS-CoV-2 subjects. Initial enrollment included 130,347 cases, with 53,078 confirmed positive and 77,269 negative. An additional 11,786 samples expanded the dataset to 142,133. The Omicron and Centaurus variants, in confirmed positive patients, exhibited a slightly higher frequency of hematological abnormalities (30.42%) than negative participants (27.01%). Notably, lymphocyte count reduction (40.95%) suggested its potential as an alternative diagnostic parameter for clinical COVID-19. Decreased levels of NA (37.99%), HGB (26.17%), MCV (20.60%), PLT (6.15%), and ALB (2.28%) were observed. Abnormally elevated NEU, CR, MONO, RBCs, WBC, and EOS levels affected 26.00%, 24.28%, 30.79%, 22.02%, 6.28%, and 5.53% of subjects, respectively. Comparatively, positive patients exhibited higher abnormal blood parameters—LYMP count (57.40%), NEU count (46.08%), EOS count (62.48%), MONO count (31.61%), RBC count (30.32%), ALC count (43.60%), CR count (30.91%), NA count (40.53%), CRP count (68.46%), and DD (63.08%) than negative counterparts. The study underscores lymphocytopenia's potential as a cost-effective, early diagnostic biomarker for clinical COVID-19, preceding real-time PCR diagnosis. This supports its consideration in resource-limited settings for strategic screening and policy-making in the ongoing SARS-CoV-2 battle. Resumo A pandemia duradoura do SARS-CoV-2 exige ferramentas robustas para avaliação da gravidade clínica. Este estudo, realizado no Islamabad Diagnostic Center em todo o Paquistão, de janeiro de 2021 a agosto de 2022, teve como objetivo investigar anormalidades hematológicas em indivíduos suspeitos de infecção por SARS-CoV-2. A triagem inicial incluiu 130.347 casos, com 53.078 positivos confirmados e 77.269 negativos. Um acréscimo de 11.786 amostras expandiu o conjunto de dados para 142.133. As variantes Ômicron e Centaurus, em pacientes positivos confirmados, exibiram uma frequência ligeiramente maior de anormalidades hematológicas (30,42%) em comparação com os participantes negativos (27,01%). Notavelmente, a redução da contagem de linfócitos (40,95%) sugeriu seu potencial como parâmetro diagnóstico alternativo para COVID-19 clínica. Foram observados níveis reduzidos de sódio (NA – 37,99%), hemoglobina (HGB – 26,17%), volume corpuscular médio (MCV – 20,60%), plaquetas (PLT – 6,15%) e albumina (ALB – 2,28%). Níveis anormalmente elevados de neutrófilos (NEU), creatinina (CR), Monócitos (MONO), hemácias (RBCs), leucócitos totais (WBC) e eosinófilos (EOS) afetaram 26,00%, 24,28%, 30,79%, 22,02%, 6,28% e 5,53% dos indivíduos, respectivamente. Comparativamente, os pacientes positivos exibiram parâmetros sanguíneos anormais mais elevados – contagem de linfócitos (LYMP – 57,40%), contagem de neutrófilos (NEU – 46,08%), contagem de eosinófilos (EOS – 62,48%), contagem de monócitos (MONO – 31,61%), contagem de hemácias (RBC – 30,32%), contagem de absoluta de linfócitos (ALC – 43,60%), contagem de creatinina (CR – 30,91%), contagem de sódio (NA – 40,53%), contagem de proteína C-reativa (CRP – 68,46%) e dímero (DD – 63,08%), em comparação aos pacientes negativos. O estudo ressalta o potencial da linfocitopenia como biomarcador de diagnóstico precoce e econômico para a COVID-19 clínica, precedendo o diagnóstico por PCR em tempo real. Essas descobertas reforçam sua aplicabilidade em contextos com recursos limitados, contribuindo para o rastreamento estratégico e formulação de políticas de saúde no enfrentamento contínuo do SARS-CoV-2.

Background Thrombocytopenia is a common haematological abnormality in malaria patients that is associated with an increased risk of mortality. Given the endemic nature of malaria in Ethiopia, it is crucial to comprehend the prevalence of thrombocytopenia in this setting to enhance clinical care. Therefore, this study aimed to systematically review and synthesize the available evidence on the prevalence of thrombocytopenia among malaria patients in Ethiopia. Methods This systematic review and meta-analysis reviewed studies on thrombocytopenia prevalence in malaria patients, using databases including PubMed, Google Scholar, EMBASE, African Journals online database, and Hinary. STATA version 17 software was used for statistical analysis. A random-effects model was used to estimate pooled effect sizes. Heterogeneity among the included studies was assessed using Galbraith, Cochran’s Q test, and I 2 statistics. Subgroup analysis, sensitivity analysis, and meta-regression were conducted to identify the source of heterogeneity. Publication bias was evaluated using a funnel plot and Egger's test. Results Of the 154 studies identified, 31 that fulfilled the eligibility criteria were included in the meta-analysis consisting of 1173 study participants and 823 thrombocytopenic cases. The pooled prevalence of thrombocytopenia was 70% (95% CI: 63, 77) with significant heterogeneity. Subgroup analysis showed the highest pooled prevalence of thrombocytopenia in the Southern Nations Nationalities and Peoples’ region (78.34%) followed by the Amhara region (69.7%), whereas the lowest prevalence was observed in the Gambella Region (63.4%). The sample size was responsible for the observed heterogeneity among the studies, as indicated by the statistically significant result in the meta-regression analysis (p = 0.001). Conclusion Thrombocytopenia is a frequent abnormality finding among malaria patients in Ethiopia, affecting a substantial percentage of individuals. The high frequency found in this research emphasizes the significance of regular platelet monitoring in the treatment of malaria patients. Further studies are needed to investigate the clinical implications of thrombocytopenia in malaria patients.

Background: Hematological abnormalities are a common complication of malaria infection. However, there is a paucity of evidence regarding it among malaria-infected adult patients in association with the ABO blood group in Ethiopia, particularly in the Harari Region. Therefore, this study aimed to assess the hematological abnormalities among malaria-infected adult patients in association with ABO blood groups at Jinella Health Center, Harar, Eastern Ethiopia. Methods: An institutional-based cross-sectional study was conducted from July 10, 2022, to January 10, 2023. Four milliliters of venous blood were collected from each study participant. Drops of blood were used for blood film preparation. ABO blood group was determined by agglutination test using monoclonal anti-sera (Agape Diagnostics Ltd., India). A complete blood count was done using the D * H 800 (Beckman Coulter, Inc, Miami, FL) hematology analyzer. The data were analyzed using SPSS version 26. Bivariable and multivariable logistic regression models were fitted. The level of significance was declared at a p-value of <0.05. Results: The study revealed that 47.2% (95% CI: 41.0 53.6) of the participants were anemic. Being female (AOR = 3.18, 95% CI = 1.67, 6.04), having the A blood group (AOR = 2.75, CI = 1.20, 6.31), and being infected with P. falciparum (AOR = 2.64, CI = 1.26, 5.53) were all significantly associated with malaria anemia. The overall prevalence of thrombocytopenia was also 67.7% (95% CI: 61.7-73.4%). It was significantly associated with P. falciparum infection (AOR = 8.03, CI = 3.53, 18.25) and high parasitemia levels (AOR = 4.40, CI = 1.57, 12.32). Conclusion: Patients with malaria who belonged to the \"A\" blood group in the study area had anemia as a serious health problem. Hence, frequently checking for anemia in patients with malaria who have blood group \"A\" can help with early detection and better management of anemia. Keywords: malaria, hematological abnormalities, blood groups, Harar, Ethiopia

Background Thyroid hormones are essential for metabolic control and red blood cell production. Imbalances in these hormones can cause anemia by disrupting erythropoiesis. Low thyroid hormone levels in hypothyroidism impair red blood cell production by inhibiting erythroid progenitor activity and erythropoietin (EPO) synthesis. Hyperthyroidism, on the other hand, can cause oxidative stress and accelerate red blood cell turnover, resulting in increased red cell distribution width (RDW), a measure of size variability. This research emphasizes the complex relationship between thyroid dysfunction and anemia, emphasizing the need for a better understanding of these mechanisms in order to improve anemia diagnosis and management in patients with thyroid disease. Methods A cross-sectional study was conducted at the University Gondar Comprehensive Specialized Hospital in Northwest Ethiopia from March 12, 2022, to May 26, 2022. The study involved 308 participants selected consecutively. Socio-demographic and clinical data were collected using a structured questionnaire and data extraction sheet. Venous blood samples were analyzed for complete blood cell counts using the Beckman-Coulter DxH-800 hematology analyzer. Data entry was performed with EpiData version 3.1, and statistical analysis was conducted using Stata version 14. Binary and multivariable logistic regression analyses were employed to identify factors associated with hematological abnormalities. Statistical significance was determined by a P-value of less than 0.05. Results The overall magnitude of anemia, was 26.3%. Hypothyroidism (AOR = 2, 95% CI:1.0- 3.6), alcohol consumption (AOR = 4, 95% CI: 1.7–9.2), not consume meat (AOR = 4, 95% CI: 1.6–10.4), vegetable consumption (AOR = 2.5, 95% CI:1.1–5.5) and febrile illness (AOR = 2.6, 95% CI:1.3–5.4) were found to be associated with anemia. Conclusion Anemia was a moderate public health problem among thyroid dysfunction patients, mainly normocytic normochromic anemia was the most common type of anemia, leukopenia was second major hematological abnormality. Hypothyroidism, alcohol consumption, meat consumption, vegetable consumption, and febrile illness were associated with anemia. Thus, all patients with thyroid dysfunction should have regular anemia screenings, particularly those with important risk factors. This could aid in the early identification and efficient treatment of anemia, improving the patients’ quality of life.

Purpose This study aimed to identify functional brain connectivity patterns associated with cognitive performance in Beta‐thalassemia major (β‐TM) children and to determine whether hematological factors influence cognition indirectly through alterations in connectivity. Method We recruited 25 children with β‐TM and 35 age‐matched healthy controls. Cognitive performance was assessed using the Wechsler Intelligence Scale (WIS). Resting‐state functional MRI data were processed to construct whole‐brain functional connectivity matrices. We applied network‐based statistics (NBS) to compare connectivity differences between groups and connectome‐based predictive modeling (CPM) with cross‐validation to predict cognitive scores. Mediation analyses were further conducted to test whether hematological metrics (hemoglobin level, red blood cell distribution width) impacted cognition through functional connectivity. Finding Compared to controls, β‐TM children showed significantly reduced WIS scores and widespread disruptions in functional connectivity, particularly in cerebellar, motor, and temporal networks. The CPM approach identified a predictive network that largely overlapped with the NBS‐derived network and robustly predicted WIS scores. Mediation analysis revealed that hemoglobin and red blood cell distribution width influenced cognitive scores indirectly through altered connectivity, indicating a full mediation effect. Conclusion This study provides evidence that hematological abnormalities in β‐TM children impair cognitive performance via their impact on functional brain networks. Functional connectivity signatures derived from CPM may serve as early neuromarkers of cognitive vulnerability and could inform future monitoring and intervention strategies in this population. Connectome predictive modeling (CPM) revealed reduced functional connectivity linked to cognitive underperformance in children with β‐thalassemia major (β‐TM), which appeared to be modulated by multiple hematological factors.

In neonatal sepsis, anemia, leukocytosis, thrombocytopenia, and a shortened coagulation time are the most common hematologic abnormalities. However, there is inadequate information regarding the hematological abnormalities in neonatal sepsis. Thus, we aimed to determine the magnitude of hematological abnormalities in neonatal sepsis. This is a cross-sectional study that included 143 neonates with culture proven sepsis aged 1-28 days from September 2020 to November 2021 at the University of Gondar Specialized Referral Hospital. The sociodemographic data was collected using a pre-tested structured questionnaire, and the clinical and laboratory data was collected using a data collection sheet. A total of 2 mL of venous blood was taken using a vacutainer collection device for the complete blood count (CBC) and blood culture analysis. A univariate and multivariate logistic regression model was used to investigate factors associated with hematological abnormalities in neonatal sepsis. Statistical significance was declared when a p-value was less than 0.05. The prevalence of anemia, thrombocytopenia, and leucopenia in neonatal sepsis was 49% (95% CI: 40.89-57.06), 44.7% (95% CI: 36.8-52.9), and 26.6% (95% CI: 22.01-29.40), respectively. On the other hand, leukocytosis and thrombocytosis were found in 7.7% (95% CI: 4.35-13.25) and 11.9% (95% CI: 7.56-18.21), respectively. Being female (AOR: 3.3; 95% CI: 1.20-3.82) and being aged less than 7 days (AOR: 2.44; 95% CI: 1.6-6.9) were found to be significant predictors of anemia. The magnitude of anemia, leucopenia, and thrombocytopenia is high in neonatal sepsis. Furthermore, being female and being younger than 7 days were risk factors for anemia. Thus, the diagnosis and treatment of anemia, leucopenia, and thrombocytopenia prevents further complications in neonatal sepsis.

This is the first reported case of Fabry disease (FD) coexisting with myelodysplastic syndrome (MDS). While the coexistence of FD and MDS may be incidental, the case underscores the importance of considering FD in patients with unexplained systemic and hematological abnormalities, particularly those with a family history.

Background Asthma is a chronic inflammatory disease that affects the lungs. Variation in whole blood cell lines is caused by the progression and severity of asthma. Common hematological abnormalities encountered during asthma include eosinophilia, neutrophilia, leukocytosis, and increased erythrocyte sedimentation rate. The main aim of this study was to assess the selected hematological abnormalities and their associated factors among asthmatic patients in Northwest Ethiopia from March to May 2021. Methodology A hospital-based cross-sectional study was conducted on a total of 320 asthmatic patients in Northwest Ethiopia. A simple random sampling technique was employed to select study participants. A pre-tested structured questionnaire and a checklist were used to collect data. Blood samples were collected from asthmatic patients for complete blood count and erythrocyte sedimentation rate determination. Hematological profiles were analyzed by Unicel DxH 800 (Beckman Coulter, Ireland). The erythrocyte sedimentation rate was determined by using the Westergren method. The data were entered into EpiData version 3.0.4 and analyzed with a statistical package for social science version 20 software . The bi-variable and multi-variable binary logistic regression models were used to assess the factors associated with hematological abnormalities. A p value of less than 0.05 in the multivariable logistic regression analysis was considered statistically significant. Results The overall prevalence of neutrophilia, eosinophilia, thrombocytopenia, leukocytosis, and basophilia was 35.3%, 20%, 11.9%, 10.3%, and 4.1%, respectively. Neutrophilia was associated with a lack of physical activity (AOR = 3.25; 95% CI 1.43–7.37) and a history of taking non-asthmatic drugs within the previous three months (AOR = 2.63; 95% CI 1.22–5.65). Being admitted to the emergency department (AOR = 0.27; 95% CI 0.11–5.67) was found to be associated with eosinophilia. In addition, being admitted to the emergency department (AOR = 5.44; 95%CI: 2.6–11.3) was associated with thrombocytopenia. Conclusion The current study demonstrated the predominant prevalence of neutrophilia, followed by eosinophilia, among asthma patients. Therefore, hematological abnormalities should be taken into account for proper monitoring and management of asthmatic patients.