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Objective Hemoglobin Constant Spring (HbCS) is often missed by routine hemoglobin analysis. The aim of this research was to study HbCS stability as identified by capillary electrophoresis (CE) to determine the specimen storage time limit. Methods The EDTA blood of 29 HbCS samples were kept at 4°C and analyzed every workday until CE could not detect HbCS or until 7 weeks after blood collection. The genotypes were confirmed by multiplex polymerase chain reaction. Results The median subject age was 27 years and 10 subjects were male. The HbCS levels were stable during the first 7 days but became undetectable in 5 cases (17.2%) after 1 week. All of them were heterozygous HbCS. Longer detection times were correlated with the higher baseline HbCS levels, with a correlation coefficient of 0.582 (P  ≤ 0.001) Conclusion Routine hemoglobin typing and quantitation should be performed within 1 week after blood collection to detect low HbCS levels, especially in heterozygous HbCS. [ABSTRACT FROM AUTHOR]

Background and Objectives: Due to rebalanced haemostasis in cirrhosis, viscoelastometric testing (VET) is more accurate than standard coagulation tests (SCTs) in preprocedural haemostatic evaluation, resulting in decreased unnecessary transfusion. We aimed to determine the impact of VET‐guided strategy on postprocedural bleeding, periprocedural transfusion rates and quantities, transfusion‐related adverse events (TRAEs), lengths of stay (LOS) and mortality from randomized controlled trials (RCTs) of cirrhotic patients. Methods: PubMed and EMBASE were searched for RCTs comparing VET‐guided with SCT‐guided transfusion in cirrhotic adults undergoing esophagogastroduodenoscopy, liver transplantation or other invasive interventions. Using random‐effects models, the pooled risk ratios (RRs) and/or mean differences (MDs) of postprocedural bleeding‐free events and the other outcomes were estimated alongside 95% confidence intervals (CIs). Results: Of seven included RCTs (n = 421; 72.2% men; mean age 49.1 years), VET‐guided transfusion did not change postprocedural bleeding‐free statuses (RR 1.05; 95% CI 0.94–1.17). However, VET‐based algorithms decreased the rates of fresh frozen plasma (FFP; RR 0.52; 95% CI 0.35–0.77) and platelet transfusions (RR 0.34; 95% CI 0.16–0.73), the quantities of transfused FFP (MD −1.39 units; 95% CI −2.18 to −0.60), platelets (MD −1.06 units; 95% CI −2.01 to −0.12) and cryoprecipitate (MD −7.13 units; 95% CI −14.20 to −0.07) and the risk of TRAEs (RR 0.42; 95% CI 0.27–0.65). The overall mortality rates and LOS were not significantly different between two groups. Conclusion: Compared with conventional SCT‐guided, VET‐guided strategy decreases periprocedural plasma and platelet transfusions and TRAEs, without increasing haemorrhagic complications, LOS or mortality in cirrhosis. [ABSTRACT FROM AUTHOR]

Daratumumab rapidly reduces high-titre factor VIII inhibitors in haemophilia A patients during life-threatening haemorrhages Alloantibodies against factor VIII (FVIII), arising in 20%-35% of severe haemophilia A patients, significantly increase both haemophilia-related mortality and the financial burden due to the high cost of bypassing agents (BAs) and immune tolerance induction (ITI). Despite detectable low-level inhibitors, the patient achieved a partial response with 75.3% recovery of FVIII activity (FVIII:C) and an infused FVIII half-life (T/2) of 9.4 hours. Although we could not evaluate plasma cells in the bone marrow and FVIII-specific IgG in the plasma, peripheral CD19 SP + sp B cells were depleted in all patients (Figure S1), and class-switched memory B-cell counts were persistently low despite B-cell reconstitution14 postdaratumumab (Figure S2). [Extracted from the article]

Objectives: A sensitive screening for the coexistence of α0-thalassemia and the hemoglobin E (Hb E) trait is important to identify at-risk couples for hydrops fetalis. However, previous cutoff values have shown a positive predictive value (PPV) of only 50% or less. This study aimed to define more specific indicators to reduce the need for DNA tests. Methods: Patients with Hb E trait, as diagnosed by high performance liquid chromatography (HPLC) and/or isoelectric focusing (IEF) techniques, were tested for α0-thalassemia and α+-thalassemia deletions using multiplex gap polymerase chain reaction. Iron deficiency anemia (IDA) were excluded using a red cell distribution width (RDW) of more than 14.5%. Results: From 390 specimens, suitable cutoff values showing a 100% sensitivity for detection of heterozygous α0-thalassemia were an Hb E level of less than 22% by HPLC, a mean corpuscular volume (MCV) of less than 72 fL, and a mean corpuscular hemoglobin (MCH) level of less than 22.5 pg. Comparable results were obtained in the validation cohort (N = 179). Using a combination of Hb E with either MCV or MCH cutoff points gave a PPV of 76.2% and 77.4%, respectively. Discussion: IDA was reported to interfere with Hb E level. In this study, we excluded IDA using RDW of more than 14.5% to enhance the test specificity. Conclusion: Lower cutoff screening values can be used to exclude α0-thalassemia in the Hb E trait yielding a higher specificity in a normal RDW condition. This can save the cost and labor of DNA testing. [ABSTRACT FROM AUTHOR]