Immunogenicity profile after COVID-19 vaccination in patients with onco-hematological diseases.

Objective: To evaluate the influence of onco-hematological pathologies on seroconversion to COVID-19 vaccines, in addition to the effects of chemotherapy treatment on this response.

Methods: The present study evaluated the immunogenic response of 76 patients with onco-hematological diseases to multiple vaccine platforms compared to 25 control individuals.

Results: Our results showed positive response rates of 74.

Posterior Circulation Evaluation in Patients with Sickle Cell Anemia.

Background: The role of transcranial Doppler (TCD) ultrasonography in identifying children with sickle cell anemia (SCA) at risk for stroke is well known; however, the major studies that evaluated TCD velocities in children with SCA did not report posterior circulation evaluation data. The objective of our study was to describe the pattern of blood flow velocities in the posterior circulation of patients with SCA and to examine their relationship with findings on magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA).

Methods: All adult patients with SCA followed in the outpatient clinic of our hospital were evaluated with TCD and MRI/MRA.

Haptoglobin gene polymorphisms and interleukin-6 and -8 levels in patients with sickle cell anemia.

Background: Haptoglobin genotypes, and interleukin-6 and -8 participate in the pathophysiology of sickle cell anemia. The expression of cytokines is regulated by genetic mechanisms however the effect of haptoglobin polymorphisms on these cytokines is not fully understood. This study aimed to compare the frequency of haptoglobin genotypes and the interleukin-6 and -8 concentrations in sickle cell anemia patients and controls to investigate the association between haptoglobin genotypes and cytokine levels.

Interleukin-1β and interleukin-6 gene polymorphisms are associated with manifestations of sickle cell anemia.

Sickle cell anemia (SCA), a disorder characterized by both acute and chronic inflammation, exhibits substantial phenotypic variability. Interleukin-1 beta (IL-1β) and IL-6 are important in acute and chronic diseases, and their single nucleotide polymorphisms (SNPs) have been considered as predictors of prognosis in several inflammatory conditions. This study aims at exploring possible association of IL-1β and IL-6 SNPs as potential genetic modifiers and or predictors of SCA clinical and laboratory phenotypes.

Exercise-Induced Abnormal Increase of Systolic Pulmonary Artery Pressure in Adult Patients With Sickle Cell Anemia: An Exercise Stress Echocardiography Study.

Background: Pulmonary hypertension (PH) at rest is a risk factor for death in patients with sickle cell anemia (SCA). Exercise echocardiography (EE) can detect latent PH. We sought to investigate the occurrence of exercise-induced abnormal response of systolic pulmonary artery pressure (SPAP) in adult patients with SCA and normal SPAP at rest, and to identify the independent predictors of this abnormal response.

Priapism is associated with sleep hypoxemia in sickle cell disease.

Purpose: We assessed penile rigidity during sleep and the relationship of sleep abnormalities with priapism in adults with sickle cell disease.

Materials And Methods: This was a case-control study of 18 patients with sickle cell disease and a history of priapism during the previous year, and 16 controls with sickle cell disease. Participants underwent overnight polysomnography and RigiScan® Plus recording to detect penile rigidity oscillations.

Molecular analysis of β-thalassemia patients: first identification of mutations HBB:c.93-2A>G and HBB:c.114G>A in Brazil.

The various clinical phenotypes in β-thalassemias have stimulated the study of genetic factors that could modify the manifestations of these diseases. We examined 21 patients with β-thalassemia (β-thal) in order to identify some genetic modifying factors: β-thalassemia mutations, HBG2:g.-158C>T polymorphism, α-globin gene deletions and (AT)xNz(AT)y motif within the hypersensitive site 2-locus control region (HS2-LCR).

Bone marrow and peripheral white blood cells number is affected by sleep deprivation in a murine experimental model.

Sleep deficit and related disorders are becoming increasingly prevalent in modern life and an extensive literature has documented that acute or chronic sleep deprivation can lead to several physiological consequences. Here, we evaluated the effects of sleep deprivation on hematopoietic composition of either bone marrow or peripheral blood. Mice were subjected to paradoxical sleep deprivation (PSD) for 72 h by modified multiple platform method, with or without an additional sleep recovery (SR) period of 10 days.

Absence of association between TNF-α polymorphism and cerebral large-vessel abnormalities in adults with sickle cell anemia.

Stroke is a serious complication of sickle cell anemia (SCA) affecting children and adults. Recent reports suggested that tumor necrosis factor-α (TNF-α) (-308) polymorphism is an important risk factor for stroke in children with SCA. The role of TNF-α polymorphism in the frequency of brain magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) abnormalities in adults with SCA is still uncertain.

Brain magnetic resonance imaging abnormalities in adult patients with sickle cell disease: correlation with transcranial Doppler findings.

Background And Purpose: Brain imaging abnormalities were reported in up to 44% of children with sickle cell disease (SCD). The prevalence of neuroimaging abnormalities in adult patients with SCD and their relationship to transcranial Doppler is still unclear. Our objectives were to study the frequency of MRI and MR angiography abnormalities in adults with SCD and to define what transcranial Doppler velocities are associated with intracranial stenoses detected by MR angiography.

Multiple primary choledocholithiasis in sickle cell disease.

Choledocholithiasis can be caused by either primary or secondary bile duct stones. Although patients with sickle cell disease (SCD) are at high risk of development of pigmented gallstones due to chronic hemolysis, primary choledocholithiasis in SCD is very uncommon. The delay in the diagnosis of biliary tract pathology can occur in patients who had a prior cholecystectomy.

XmnI polymorphism is associated with fetal hemoglobin levels in hypoplastic syndromes.

Context And Objective: Acquired fetal hemoglobin (HbF) elevation has been implicated as a prognostic factor in dyserythropoietic disorders. Our objectives were to examine acquired HbF increases in aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH) patients, and to evaluate whether there is an association between the presence of XmnI and 5' hypersensitive site locus control region (LCR-HS2) polymorphisms and the HbF levels.

Design And Setting: Cross-sectional study at the Hematology and Blood Transfusion Service of Universidade Federal de São Paulo - Escola Paulista de Medicina.

Transcranial Doppler in adult patients with sickle cell disease.

Background: Transcranial Doppler (TCD) is the key test in determining the need for prophylactic blood transfusion to prevent stroke in children with sickle cell disease (SCD). The role of TCD in assessing stroke risk in adults with SCD as well as the pattern of TCD velocities in these patients are still undetermined.

Methods: The authors compared TCD velocities in SCD adults (n=56) with those of healthy controls (n=56).

Effects of hydroxyurea in a population of Brazilian patients with sickle cell anemia.

Fetal hemoglobin (HbF) inhibits the polymerization of sickle hemoglobin, modulating the clinical features of sickle cell anemia (SCA). Hydroxyurea (HU) therapy can increase the HbF level, although its production can be influenced by genetic determinants. Twenty-two Brazilian SCA patients were evaluated over 5 years before and after HU use.

Acquired hemoglobin H disease in a patient with aplastic anemia evolving into acute myeloid leukemia.

Context: The prognosis of severe aplastic anemia has improved since the introduction of bone marrow transplantation and treatment with antithymocyte globulin. In contrast to the success of these protocols, studies with long term follow-up have shown the occurrence of clonal diseases such as paroxysmal nocturnal hemoglobinuria, myelodysplastic syndrome and acute leukemia in aplastic anemia.

Case Report: We report the first case of a Brazilian patient with aplastic anemia who developed myelodysplastic syndrome and acute myeloid leukemia showing acquired hemoglobin H and increased fetal hemoglobin.

Myonecrosis in sickle cell anemia: case report and review of the literature.

Vascular occlusion is responsible for most of the severe complications of sickle cell anemia (SCA). The involvement of muscle and fascia is uncommon in SCA, but myonecrosis may occur in SCA crisis. The data accumulated in the literature is limited to only a few reports describing mainly adult patients presenting with severe muscular pain.