International Society on Thrombosis and Haemostasis Clinical Practice Guideline for Treatment of Congenital Haemophilia-A Critical Appraisal.

Introduction: Evidence-based clinical practice guidelines drive optimal patient care and facilitate access to high-quality treatment. Creating guidelines for rare diseases such as haemophilia, where evidence does not often come from randomized controlled trials but from non-randomized and well-designed observational studies and real-world data, is challenging. The methodology used for assessing available evidence should consider this critical fact.

Infrared thermography: An investigative tool for detection of subclinical bleed in hemophilia on prophylaxis.

Background & objectives Subclinical joint bleed is a matter of concern in individuals with hemophilia on prophylaxis. Ultrasonography (USG) is mostly used for the screening of subclinical bleeds in such individuals but it has its own limitations. Thus a more comprehensive technology is required for the detection of the same.

Thalidomide and Hydroxyurea in Transfusion-Dependent Thalassemia: Efficacy, Safety Profile and Impact on Quality of Life.

Transfusion-dependent thalassemia (TDT) is a major public health concern in India, requiring regular transfusions for survival. There is also significant morbidity caused by iron overload and transfusion related infections. Novel therapies targeting fetal hemoglobin induction are the need of the hour in resource-poor institutions for patients where transplant is not feasible for various reasons.

Rationale for the Potential Use of Recombinant Activated Factor VII in Severe Post-Partum Hemorrhage.

Severe post-partum hemorrhage (PPH) is a major cause of maternal mortality worldwide. Recombinant activated factor VII (rFVIIa) has recently been approved by the European Medicines Agency for the treatment of severe PPH if uterotonics fail to achieve hemostasis. Although large randomized controlled trials are lacking, accumulated evidence from smaller studies and international registries supports the efficacy of rFVIIa alongside extended standard treatment to control severe PPH.

Heterogeneity in precision oncology.

Precision oncology is a rapidly evolving concept that holds great promise in cancer treatment. However, a cancer complexity attributed to genomic and acquired tumour heterogeneity limits treatment effectiveness and increases toxicity. These limitations refer to both systemic therapies and radiotherapy, which are two mainstays of non-invasive cancer treatment.

Bendamustine-Rituximab in Young CLL: Relevance in the Era of Small Molecule Inhibitors.

Purpose: Chemo-immunotherapies like Fludarabine-Cyclophosphamide-Rituximab (FCR) are used for treatment of chronic lymphocytic leukemia (CLL) in young and fit patients while Bendamustine-Rituximab (BR) is used in older patients. In a resource constrained setting, managing toxicities of FCR chemotherapy is challenging and this study explores the use of upfront BR treatment in young CLL patients (age < 65).

Methods: Data of 61 CLL patients treated with the BR regimen between 2016 and 2020 was analysed.

Bleeding per rectum during induction chemotherapy: Looking beyond the leukaemia - 2 case reports and review of literature.

Synchronous malignancies involving acute leukemia and a solid organ are rare. Bleeding per rectum is a common manifestation of acute leukemia during induction chemotherapy and might mask the presence of synchronous colorectal adenocarcinoma (CRC). Here we present two rare cases of acute leukemia with synchronous CRC.

Hepatitis A virus (HAV) RNA detection in Polish blood donors and likely transmissions through blood components during the 2017-2019 epidemic.

Background: In Poland, hepatitis A virus (HAV) RNA screening was performed in plasma for fractionation usually immediately before shipment.

Objective: Our goal was to study epidemiology, rate of transfusion transmitted HAV during epidemic (2017-2019), and viral characteristics of infected plasma donors.

Study Design And Methods: HAV RNA was tested in 1,866,590 donations from 1,210,423 donors using RT-PCR in mini pools of 96 (MP96) or TMA in MP16.

Derivative 6 as an additional chromosomal abnormality along with t(15;17): A case report.

Acute promyelocytic leukemia (APL) is a type of acute myeloid leukemia (AML) characterized by the presence of t(15;17)(q22;q21) translocation leading to fusion between PML and RARa gene. Treatment combining all-trans retinoic acid (ATRA) and arsenic trioxide (ATO) has dramatically improved the prognosis of APL. We report a rare finding of primary clone of t(15;17) followed by a sequential clonal evolution of additional derivative chromosome 6 formation by a two hit mechanism.

Thromboelastometry as an Ancillary Tool for Evaluation of Coagulation Status after rFVIIa Therapy in a Pregnant Woman with Severe Hypoproconvertinemia-A Case Series and Review of the Literature.

Factor VII (FVII) deficiency is a rare hemorrhagic diathesis. In females, heavy menstrual and postpartum bleeding can appear as a consequence of its deficiency. Supplementation of the recombinant FVIIa is widely accepted.

Assessing quality of blood components derived from whole blood treated with riboflavin and ultraviolet light and separated with a fully automated device.

Background: Combining pathogen reduction and automated separation of whole blood (WB), together with the use of improved additive solutions, may increase reproducibility and extend shelf-life of blood components.

Materials And Methods: Forty WB units were collected from volunteer donors and randomised 1:1 into two groups: 1) pathogen reduction with riboflavin and ultraviolet light (PRT); or 2) no treatment (Control). After two hours (h) at room temperature, all units underwent fully automated separation into red blood cell concentrate (RBCC), plasma and leukopack components.

Prenatal Screening and Diagnosis of ß-Thalassemia in India: Is ARMS-PCR Enough?

Accurate and timely prenatal diagnosis of thalassemia is cornerstone to the success of thalassemia control; currently parents are screened for ß-thalassemia mutations by ARMS-PCR and subsequently chorionic villus sampling is done. We did an audit to ascertain whether the present design is adequate and determined the role of sequencing for pre-natal diagnosis of beta-thalassemia. This was a retrospective analysis of prenatal testing data collected over 10 years, (2010-2019).

Transmitted HIV drug resistance and subtype patterns among blood donors in Poland.

Surveillance on the HIV molecular variability, risk of drug resistance transmission and evolution of novel viral variants among blood donors remains an understudied aspect of hemovigilance. This nationwide study analyses patterns of HIV diversity and transmitted resistance mutations. Study included 185 samples from the first time and repeat blood donors with HIV infection identified by molecular assay.

Innate Immune Invisible Ultrasmall Gold Nanoparticles-Framework for Synthesis and Evaluation.

Nanomedicine is seen as a potential central player in the delivery of personalized medicine. Biocompatibility issues of nanoparticles have largely been resolved over the past decade. Despite their tremendous progress, less than 1% of applied nanosystems can hit their intended target location, such as a solid tumor, and this remains an obstacle to their full ability and potential with a high translational value.

A Pilot Study on Probing of Imatinib Induced Platelet Dysfunction in Patients with Chronic Myeloid Leukemia-Chronic Phase and Absence of Associated Bleeding Manifestation: Trying to Solve an Enigma.

Imatinib, the first Tyrosine Kinase Inhibitor (TKI) used for the treatment of chronic myeloid leukaemia (CML) has revolutionized the management by inhibiting BCR-ABL tyrosine kinase. According to earlier reports there are concerns regarding the adverse effect of imatinib on haemostasis by causing platelet dysfunction. Here we studied platelet function using platelet aggregometry, in 19 CML chronic phase (CML-CP) patients on imatinib therapy, in complete haematologic response (CHR).

Indian Society of Hematology and Blood Transfusion (ISHBT) Consensus Document on Hematological Practice During COVID-19 Pandemic.

The SARS-CoV-2 (COVID-19) pandemic is a worldwide public health emergency with widespread impact on health care delivery. Unforeseen challenges have been noted during administration of usual haematology care in these unusual COVID-19 times. Medical services have been overstretched and frontline health workers have borne the brunt of COVID-19 pandemic.

Analysis of blood stream infections, antibiograms and clinical outcomes in haematological patients with febrile neutropenia: data from a tertiary care haematology institute in India.

Timely administration of appropriate empirical antibiotics in febrile neutropenia is crucial for favourable patient outcomes. There are guidelines in place recommending such antibiotics. However, regional variations and local epidemiological data must be evaluated to tailor the antibiotics for best possible and rational use.

Safety, Immunogenicity, and Hemostatic Efficacy of Nonacog Gamma in Patients With Severe or Moderately Severe Hemophilia B: A Continuation Study.

This phase 3, prospective, open-label, multicenter, continuation study (NCT01286779) investigated the use of a recombinant factor IX (FIX), nonacog gamma (BAX 326, RIXUBIS) in patients with severe or moderately severe hemophilia B. The study population included 85 patients transitioning from a phase 1/3 pivotal study (NCT01174446), a pediatric study (NCT01488994), and 30 newly recruited patients, naïve to nonacog gamma. Patients received nonacog gamma as prophylaxis treatment (standard, modified or PK-tailored) or on-demand, as determined by the investigator.

Key Determinants of Phenotypic Heterogeneity of Hb E/β Thalassemia: A Comparative Study from Eastern India.

HbE Beta thalassemia is phenotypically very diverse disease. We aim to study role of various genetic factors in determining severity of this disease. 243 diagnosed cases of HbE Beta thalassemia were included in this study.

Risk factors of permanent hypoparathyroidism after total thyroidectomy and central neck dissection for papillary thyroid cancer: a prospective study.

Introduction: Inadvertent removal of, or damage to the parathyroid glands in the course of operations on the anterior neck compartment are responsible for over 80% of cases of chronic hypoparathyroidism (HypoPT). This study searched for factors related to the development of permanent HypoPT after total thyroidectomy and central neck lymphadenectomy in patients with thyroid carcinoma.

Material And Methods: In total, 89 of 103 screened patients met the study's criteria and were put under prospective one-year observation.

Chronic dietary supplementation with kynurenic acid, a neuroactive metabolite of tryptophan, decreased body weight without negative influence on densitometry and mandibular bone biomechanical endurance in young rats.

Kynurenic acid (KYNA) is a neuroactive metabolite of tryptophan. KYNA naturally occurs in breast milk and its content increases with lactation, indicating the role of neonatal nutrition in general growth with long-term health effects. KYNA is also an antagonist of ionotropic glutamate receptors expressed in bone cells.

Seronegative hepatitis C virus infection in Polish blood donors-Virological characteristics of index donations and follow-up observations.

Nucleic acid testing (NAT) was implemented in Poland in 1999 for screening of plasma for fractionation and for all blood donors in 2002. To analyze seronegative NAT-positive samples representing hepatitis C virus (HCV) window-period (WP) in the years 2000 to 2016 and to determine infection outcome. We analyzed results of 17 502 739 donations screened in minipools (6-48) or individually.