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Chronic Hydroxyurea Therapy in Children with Sickle Cell Anemia: Mechanisms of Action, Systemic Effects, and Long-Term Safety.

Sickle cell disease (SCD) is the most common monogenic disorder worldwide and remains a major cause of morbidity and mortality. Sickle cell anemia (SCA), the homozygous HbSS genotype, represents the most severe and frequent form within the spectrum of SCD. Hydroxyurea (HU), a ribonucleotide reductase inhibitor, represents the first and most widely used disease-modifying therapy for SCA. This review summarizes current evidence on the mechanisms of action, clinical efficacy, systemic effects, and long-term safety of chronic HU therapy in patients with SCA. A comprehensive literature search was c

Characterization of limitations of life-sustaining therapy decisions and their impact on outcomes in adults with septic shock - Insights from a retrospective cohort.

PURPOSE: In-ICU and in-hospital deaths in critically ill patients are often subsequent to decisions of limitations of life-sustaining therap … re significantly associated with comorbidities, duration of organ-support, in-ICU complications and platelet transfusion. The overall in-ICU mortality rate was 37.6 % and reached 75.9 % among patients with LLST. CONCLUSION: Among septic shock patients, LLST are frequent, increasingly decided soon after ICU admission, vary during the ICU stay and carry a negative impa …

Development and validation of a nomogram to predict bronchopulmonary dysplasia in very preterm, very low birth weight infants.

OBJECTIVES: This study aimed to identify early predictors of bronchopulmonary dysplasia (BPD) in very preterm, very low birth weight infants and to construct and externally validate a nomogram that quantifies individual BPD risk shortly after birth to guide proactive clinical management. METHODS: We retrospectively analyzed 304 preterm infants admitted to our hospital between 2019-2024. The cohort comprised 113 infants diagnosed with BPD and 191 non-BPD controls. Clinical data, including maternal characteristics, neonatal parameters, and hematological indices measured at 14 days of postnatal

Early administration of caplacizumab combined with plasma exchange for thrombotic microangiopathy due to malignant hypertension: a case report.

Both thrombotic thrombocytopenic purpura (TTP) and malignant hypertension (MHT) present with thrombotic microangiopathy (TMA). Combination therapy with caplacizumab, anti-von Willebrand factor (VWF) A1 domain antibody, and plasma exchange (PE) has recently been highlighted as a novel therapeutic option for TTP. We treated a 51-year-old woman who showed severe hypertension, retinopathy, and acute kidney injury. Level of consciousness was clear on admission, but low-grade fever was observed. Laboratory tests showed normocytic anemia, thrombocytopenia, renal dysfunction, and a slight decrease in

Efficacy and safety of biosimilar Peg-filgrastim after autologous stem cell transplant in myeloma and lymphoma patients: a comparative study with biosimilar Filgrastim, Lenograstim, and originator Peg-filgrastim.

Data about biosimilar Peg-filgrastim (bioPEG) in autologous stem cell transplant (ASCT) are still scarce. The aim of this study has been to assess efficacy and safety of bioPEG among lymphoma and myeloma patients undergoing ASCT, comparing these data with historical controls receiving other G-CSFs. Furthermore, an economic evaluation has been included to estimate the savings by using bioPEG. This is a prospective cohort study comparing lymphoma and myeloma patients undergoing ASCT and receiving bioPEG (n = 73) with three historical consecutive cohorts collected retrospectively who received oth

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… Red blood cell (RBC) and platelet transfusion were administered for hemoglobin level < 8 g/dL and platelet count < 10 × 10 9 /L or in patients with symptomatic anemia or hemorrhagic syndrome. … In contrast, our data showed a significant lower RBC and platelet transfusion rate in patients receiving both biosimilar and originator Peg-filgrastim, when compared with the other two groups ( p < 0.001). …

Hepatitis E associated determinants and diagnostic biomarkers during pregnancy and its prenatal consequences in Multan, Punjab tertiary care setting (Pakistan).

OBJECTIVE: Hepatitis E virus (HEV) is the most common cause of acute viral hepatitis in the world. Hepatitis E infection is commonly widespread by the fecal oral routes and contaminated water. This study was designed to explore the prevalence and risk factors of hepatitis E infection in pregnant women of the Multan district, Pakistan. METHODS: The study comprised of a total of 500 enrolled patients, among which, 105 pregnant females with hepatitis E infection fulfilled the criteria for anti-HEV antibodies. Pregnant women without significant complications and without hepatitis E infection were

Hemostatic disorders in patients with infective endocarditis undergoing urgent surgical valve replacement - Rethinking current beliefs.

BACKGROUND: Although infective endocarditis (IE) represents a unique model of thrombo-inflammatory disease, the most frequent early complications of surgical valve replacement (SVR) in IE population are coagulopathy and bleeding. The hemostatic capacity and procedure-related coagulation disorders of IE patients undergoing SVR are unknown. The aims of this study were to test periprocedural hemostasis in IE patients undergoing urgent SVR, and to assess the association between disorders of hemostasis and early bleeding as well as with thromboembolic events. METHODS: A prospective, two-center, hy

Endoscopic resection of early esophageal tumors in patients with cirrhosis or portal hypertension: a multicenter observational study.

BACKGROUND: Liver cirrhosis and esophageal cancer share several risk factors, such as alcohol intake and excess weight. Endoscopic resection … underwent endoscopic band ligation (EBL) before resection, 15 received vasoactive drugs, 8 received platelet transfusion, and 9 underwent EBL during the resection procedure. Rates of complete macroscopic resection, en bloc resection, and curative resection were 92 %, 86 %, and 63 %, respectively. Adverse events included 3 perforations, 8 delayed bleedings, 8 sepsis, 6 …

Perioperative ventricular arrhythmias are increased with hemoperitoneum and are associated with increased mortality in dogs undergoing splenectomy for splenic masses.

OBJECTIVE: To identify risk factors for intra- and postoperative ventricular arrhythmias (VAs) and in-hospital mortality in dogs undergoing splenectomy for splenic masses. ANIMALS: 308 dogs. METHODS: Records from 2010 through 2018 were reviewed for dogs undergoing splenectomy for a splenic mass. Clinical and laboratory findings on admission, diagnostic imaging, anesthesia, surgery and pathology reports, treatment records, and in-hospital mortality were evaluated with logistic regression. RESULTS: VAs occurred in 138 (44.8%) dogs (126/308 [40.9%] postoperative, 51/308 [16.6%] intraoperative,

Rapidly progressing severe coagulopathy and thrombocytopenia in extreme anorexia nervosa patient with small bowel strangulation: A case report.

INTRODUCTION: Anorexia nervosa (AN) is characterized by severe dietary restriction or other weight loss behaviors motivated by a strong fear of body weight gain and a disturbed body image. In this paper, we report a case of an extreme AN patient, in whom severe coagulopathy and thrombocytopenia rapidly progressed with strangulation obstruction. PRESENTATION OF CASE: A female patient in her 20's with previous history of AN came to our hospital complaining acute onset abdominal pain. Her body mass index at presentation was 12.1 kg/m2; thus, she was classified as an "extreme" AN (BMI <15) accord

The David Operation Offers Shorter Hemostasis Time Than the Bentall in Case of Acute Aortic Dissection Type A.

Background The aim of the present study was to compare the clinical outcome of the David operation and the Bentall operation in patients with Stanford type A acute aortic dissection (AADA) from the viewpoint of hemostasis. Methods Between April 2016 and April 2020, 235 patients underwent emergent surgery for AADA. Of them, 38 patients required aortic root replacement (ARR: The David operation 17, the Bentall operation 21). The mean age was 59.3±12.6 years. In the present series, the David operation was the first choice for relatively young people, and the Bentall operation was performed for re

Comparative analysis of hematological parameters of first-time and repeat blood donors: Experience of a blood bank in southern Nigeria.

INTRODUCTION: The safety of a blood transfusion practice is anchored on safe blood from a healthy donor, while further protecting the donor from future harm. This study aimed to evaluate the hematological parameters of blood donors in view of their donor category to aid in assessing the safety threshold in terms of donation frequency. METHODS: This study made use of the descriptive cross-sectional design. The blood donors were bled and their samples analyzed using a hematology autoanalyzer. RESULTS: A total of 178 male blood donors were recruited. Most of the donors were aged 18 to 39 years

Assessment of serum iron stores in regular plateletpheresis donors.

BACKGROUND: Iron deficiency anaemia is the most common nutritional deficiency disorder in the world. Iron deficiency is a potential complication in repeated apheresis donation. The present study was aimed to evaluate serum iron stores in regular plateletpheresis donors. MATERIALS AND METHODS: A total of 60 donors were included in this study, which included 30 regular plateletpheresis donors as cases and controls were 30 first time donors. The donor samples were collected before donation for complete hemogram, transfusion transmissible infections screening and serum iron, total iron binding ca

A mini-review on aplastic anemia, illustrated by a case report on bone marrow hot pockets mimicking sclerotic bone metastases.

A 61-year-old female presented with pancytopenia with a hemoglobin of 7.6 g/dL, platelet count of 26.000/µL and neutrophil count of 525/µL. Bone marrow aspirate showed moderately cellular marrow with a dysplastic erythroid lineage and poor megakaryo- and granulopoiesis without excessive blast count. Trephine biopsy revealed profoundly hypocellular marrow with rare hematopoietic elements. Conventional karyotyping was normal and next generation sequencing revealed no mutations. These findings were compatible with transfusion dependent, non-severe aplastic anaemia (AA) with grade 3 thrombopenia a

Differential diagnostic and treatment difficulties in a patient with acquired von Willebrand syndrome.

BACKGROUND: Acquired von Willebrand syndrome (AVWS) is a rare, frequently underdiagnosed and underestimated bleeding disorder. Careful personal and family history and late-onset mucocutaneous bleeding could help clarify the etiology of bleeding deficiency. CASE PRESENTATION: An 82-year-old male patient was admitted to our clinic with a severe nosebleed on 30.05.2018. Laboratory results revealed thrombocytosis, elevated white blood cell count and high LDH. Basic coagulation parameters were normal. He was referred to our clinic, where a bone marrow biopsy was taken. His personal and family hist

Outcomes of allogeneic transplantation for hemoglobin Bart's hydrops fetalis syndrome in Hong Kong.

BACKGROUND: Hemoglobin Bart's hydrops fetalis syndrome (BHFS) was once considered a fatal condition universally. Medical advances over the past three decades have resulted in increasing numbers of BHFS survivors. This retrospective review summarized local territory-wide experience and outcomes of BHFS patients who received allogeneic hematopoietic stem cell transplantation (HSCT) in Hong Kong. METHODS: All BHFS patients who underwent allogeneic HSCT in Hong Kong, either in one of the two former pediatric transplant centers (Queen Mary Hospital and Prince of Wales Hospital) on or before 2019 o

Single-agent carboplatin AUC10 in metastatic seminoma: A multi-centre UK study of 216 patients.

BACKGROUND: Radiotherapy and cisplatin-based combination chemotherapy are accepted standard-of-care treatments for metastatic seminoma with excellent survival outcomes but with established short- and long-term morbidity. Carboplatin monotherapy may be a less toxic alternative; however early historic studies at AUC7 showed inferior outcomes. OBJECTIVES: To evaluate multi-institutional data on and toxicity and longer-term survival for metastatic seminoma patients treated with the single-agent carboplatin AUC10. METHODS: We undertook a multi-institutional analysis incorporating all men with the

Basic principles of viscoelastic testing.

BACKGROUND: Viscoelastic testing is a method of hemostatic analysis that provides a real-time, holistic view of ex vivo clotting. It allows for examination of both cellular and plasma protein contributions to clotting including platelet number and function, fibrin(ogen) function, and coagulation factor function. The method assesses physical clot properties during the transition of blood from a liquid to a gel state, either by measurement of clot shear modulus using physical force transduction or by measurement of clot resonance frequency using sonometric interrogation. Results are reported in

Clozapine-induced anemia: A case-report
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Clozapine, an atypical antipsychotic, can cause potentially life-threating side effects such as agranulocytosis. Our case presents a picture of severe anemia without any depression of the white cells or platelet lines. A 36-year-old man with treatment-resistant schizophrenia was admitted to the Psychiatric Unit for therapy assessment. After admission, he was gradually switched to clozapine treatment, 400 mg/d. General laboratory test results were normal, with a hemoglobin (Hb) level of 15.2 g/dL. The Hb level gradually decreased to 7.1 g/dL 10 weeks after switching to clozapine, when the patie

Intravesical application of platelet-rich plasma in patients with persistent haemorrhagic cystitis after hematopoietic stem cell transplantation: a single-centre preliminary experience.

PURPOSE: Haemorrhagic cystitis (HC) after allogeneic transplantation (HSCT) is a condition characterized by diffuse inflammation and bleeding from the bladder mucosa. Treatment of HC is not standardized and clinical Guidelines are elusive. The aim of this study was to evaluate the safety and efficacy of intravesical treatment with platelet-rich plasma (PRP) in patients with HC after allogenic HSCT. METHODS: Data from ten consecutive patients with BK virus-induced HC between 2013 and 2017 were collected. HC was classified into four grades. Inclusion criteria were (a) grade 3 or 4 BKV-induced H