Relapsed Multiple Myeloma with Anaplastic Transformation
(Source: Indian Journal of Hematology and Blood Transfusion)
Source: Indian Journal of Hematology and Blood Transfusion - June 3, 2023 Category: Hematology Source Type: research

Combining Low-Dose Rituximab and Bortezomib as Immunosuppressive Therapy for Acquired Hemophilia A: 6 Case Series
AbstractAcquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against factor VIII. Persistently positive inhibitor titers increase the risk of bleeding. Upfront combined regimen with multiple mechanisms has the potential to shorten remission time to lower bleeding risk and reduce immunosuppressor exposure time. We administered lowered dose of rituximab and bortezomib in combination with corticosteroids and cyclophosphamide (rituximab: 100  mg weekly × 4 or 500 mg once in week 1, bortezomib: 0.65 mg/m2 once in week 1, prednisone: 1 mg/kg daily, cyclophosphamide: 11.5–2 mg/kg daily). We...
Source: Indian Journal of Hematology and Blood Transfusion - May 26, 2023 Category: Hematology Source Type: research

A Pedigree Investigation of H-antigen Deletion Caused by Mutation of 658 C to T in the FUT1 Gene
AbstractH-antigen deletion is often caused by FUT1 gene mutation, which is a very rare blood group. In this case, the H-antigen phenotype, FUT1, FUT2 sequences, and family genetic investigation of a 26-year-old patient (proband) and her three family members were studied. The results showed that the proband and little her brother were H-deficient phenotype, their ABO genotype of both was A/O1, her father was A/B, and her mother was O1/O1. The proband and her little brother ’s FUT1 phenotype were both h3|h3, with a homozygous mutation 658C >  T in their FUT1 gene, and the FUT1 phenotype of their parents’ were H|h3...
Source: Indian Journal of Hematology and Blood Transfusion - May 24, 2023 Category: Hematology Source Type: research

Comparison Pharmacokinetic Dosing Tools in Hemophilia A Children
AbstractProphylaxis is the gold standard for the management of hemophilia A patients. It has been shown that prophylaxis regulated with pharmacokinetic (PK) data reduces frequency of bleeding and cost of treatment. To determine the best prophylaxis regimen, PK dosing tools using the Bayesian method have been developed. We aimed to compare two PK dosing tools. Blood samples were drawn before, 4, 24, and 48  h after FVIII infusions from patients with severe hemophilia A and inhibitor negative. FVIII levels were measured by PTT-based one-stage assay method. PK parameters obtained using WAPPS and myPKFiT, which are web-access...
Source: Indian Journal of Hematology and Blood Transfusion - May 22, 2023 Category: Hematology Source Type: research

Monocyte Distribution Width (MDW) in Patients with COVID-19: An Indicator of Disease Severity
AbstractIdentifying patients with Coronavirus disease-2019 (COVID-19) who may have a severe illness is essential for timely intervention and decreasing the fatality rate. In the present study, we evaluated the performance of Monocyte Distribution Width (MDW) as a prognostic marker for identifying disease severity in COVID-19 patients. We included 145 patients with PCR-confirmed COVID-19 infection in the study. The performance of MDW was evaluated by calculating the area under the receiver operating characteristic curve (AUC), specificity, sensitivity, negative predictive value, and positive predictive value. Further analys...
Source: Indian Journal of Hematology and Blood Transfusion - May 20, 2023 Category: Hematology Source Type: research

A Screening Approach for Inherited Erythrocytosis due to the VHL:c.598C   & gt;  T Mutation (Chuvash Polycythemia)
AbstractGenetic work-up of unexplained erythrocytosis that is suspected to be inherited in nature currently requires either laborious exon-by-exon gene panel testing by Sanger sequencing or expensive next-generation sequencing. A high prevalence of Chuvash polycythemia (61%) has been previously reported among north Indian erythrocytosis patients. We assessed PCR-RFLP forVHL c.598C  >  T mutation as a first-line test in 99 persons withJAK2 V617F-negative, unexplained erythrocytosis.  We enrolled two groups: Group A (n = 38) had erythrocytosis patients (n = 33) or their first-degree relatives (n = 5), and...
Source: Indian Journal of Hematology and Blood Transfusion - May 14, 2023 Category: Hematology Source Type: research

The Prognostic Impact of Tumor Microenvironment and Checkpoint Blockade-Associated Molecules (PD-1, PD-L1, CD163 and CD14) in Nodal Diffuse Large B-cell Lymphoma, NOS
AbstractIt is aimed to determine expression of programmed cell death-1 (PD-1), programmed cell death ligand-1 (PD-L1), CD163 and CD14 in diffuse large B-cell lymphomas (DLBCL), and whether these markers may predict prognosis in DLBCL cases. A total of 52 nodal DLBCL, NOS cases with no known extranodal involvement at the time of diagnosis were evaluated. PD-1, PD-L1, CD163, and CD14 were studied by immunohistochemistry. The relationships between the results and clinical and laboratory prognostic markers were investigated. It was observed that patients with PD-1 expression  <  5 positive cells/HPF had worse overall su...
Source: Indian Journal of Hematology and Blood Transfusion - May 11, 2023 Category: Hematology Source Type: research

Hemoglobin Shimonoseki HBA1:c.164A   & gt;  G Illustrating the Continuing Utility of the Sickling Test and the Challenges in Antenatal Genetic Counselling
(Source: Indian Journal of Hematology and Blood Transfusion)
Source: Indian Journal of Hematology and Blood Transfusion - May 11, 2023 Category: Hematology Source Type: research

Analysis of an Inherited FXII Deficiency Pedigree Associated with Double Heterozygous Mutations in the F12 Gene
This article is intended to identify the potential mutations of the FXII gene (F12) in an inherited FXII deficiency pedigree and illuminate the pathogenesis of the disease. The coagulation FXII activity (FXII:C) and FXII antigen (FXII:Ag) were inspected by one-stage clotting assay and enzyme-linked immunosorbent assay(ELISA), respectively. Polymerase chain reaction amplification (PCR) was performed and theF12 gene was sequenced directly. A molecular model of FXIIprotein was established for further analysis. ClustalX-2.1-win and online bioinformatic software were used to estimate the conservatism and possible impact of the ...
Source: Indian Journal of Hematology and Blood Transfusion - May 10, 2023 Category: Hematology Source Type: research

Plasminogen Activator Inhibitor-1 4G/5G Promoter Polymorphism in Indian Patients with Deep Vein Thrombosis
AbstractA single guanosine deletion/insertion (4G/5G) polymorphism in the promoter region of plasminogen activator inhibitor-1 (PAI-1) gene encoding PAI-1 protein has been investigated in deep vein thrombosis (DVT) patients. The association betweenPAI-1 4G/5G polymorphism and increased risk of DVT has been reported in some studies, while others have reported a lack of association. The present study aimed to investigate if thePAI-1 4G/5G polymorphism is associated with an increased risk of DVT in the Indian population and to assess its association with thrombophilic risk factors.  Fifty-two adult patients with a history of...
Source: Indian Journal of Hematology and Blood Transfusion - May 8, 2023 Category: Hematology Source Type: research

Metformin as a Fetal Hemoglobin Inducer in Non-transfusion Dependent Thalassemia Patients
Conclusion: Metformin intake, in addition to hydroxyurea, did not yield any extra benefit among patients with NTDT. (Source: Indian Journal of Hematology and Blood Transfusion)
Source: Indian Journal of Hematology and Blood Transfusion - May 5, 2023 Category: Hematology Source Type: research

A Potential Novel Entity for Polycythemia Vera: JAK2 Exon 24 Mutation
(Source: Indian Journal of Hematology and Blood Transfusion)
Source: Indian Journal of Hematology and Blood Transfusion - May 3, 2023 Category: Hematology Source Type: research

CG02N Analyzer Accurately Measures Fibrinogen Level in Whole Blood in the Presence of Low- or High-Dose Heparin
In this study, we aimed to investigate the accuracy of Fbg level measured using CG02N, with whole blood contained in lithium-heparinized syringes with two different concentrations of heparin. Blood samples were collected in lithium-heparinized tubes, namely PREZA-PAK ®II (low-dose heparin group [LG], 7 IU/mL) and Pro-Vent® Plus (high-dose heparin group [HG], 70.5 IU/mL). The Fbg levels in LG and HG were compared with those of citrated plasma Fbg (standard-Fbg). Strong correlations with respect to the Fbg level were observed between standard-Fbg and LG or HG (r = 0.968, p <  0.0001; r = 0.970, p < â€...
Source: Indian Journal of Hematology and Blood Transfusion - May 2, 2023 Category: Hematology Source Type: research

A Retrospective Cohort Study to Evaluate the Outcomes of HIV-Associated High-Grade B-Cell Non-Hodgkin Lymphoma (NHL) Treated with Dose Adjusted EPOCH (+/ −R) Regimen
We report our analysis on our cohort of patients who were treated with DA-EPOCH(+/−R). We retrospectively analyzed treatment-naïve HIV-associated High-grade B-cell NHL patients (ag ed ≥ 18) treated with DA-EPOCH(+/−R) regimen from 2011 to 2015. Descriptive statistics were summarized with median and range; survival outcomes were analyzed with Kaplan–Meier method. The cohort comprised of 40 patients [DLBCL(19), Burkitt’s Lymphoma(16), High-grade B-Cell Lymphoma-Uncla ssifiable(09), and Plasmablastic Lymphoma(01)] and the median CD4 + T cell count was 202/mm3. CNS prophylaxis was administered with intrathec...
Source: Indian Journal of Hematology and Blood Transfusion - April 21, 2023 Category: Hematology Source Type: research

Potential Clinical Role of LncRNA miR503HG in Multiple Myeloma and its Effect on the Proliferation and Adhesion of Myeloma Cells
This study mainly explored the role of lncRNA miR503HG in multiple myeloma and the potential downstream regulatory mechanism affecting disease. Real-time quantitative polymerase chain reaction was used to measure the expression levels of miR503HG and miR-103. A cell counting kit-8 assay was performed to detect cell viability. The concentrations of adhesion-related factors (MUC-1, VCAM-1, ICAM-1) were determined using enzyme-linked immunosorbent assay. The targeting relationship between miR503HG and miR-103 was detected by dual-luciferase reporter assay. The miR503HG expression in peripheral blood of multiple myeloma patien...
Source: Indian Journal of Hematology and Blood Transfusion - April 21, 2023 Category: Hematology Source Type: research