The primary motivating factor for blood donation is altruism and its derivatives, such as empathy and social responsibility. However, no conclusive answers were obtained from the analysis of these articles, indicating the need for further studies focused on this topic. In Brazil, blood donation is voluntary, unpaid, and anonymous. Despite being a reference in Latin America for improving voluntary donation rates and expanding the age range of potential donors, there are still many challenges related to this practice, as only 1.9% of the Brazilian population are blood donors. This article aims to identify, through a literature review, the main motivations for blood donation. For this purpose, a systematic review was conducted, including studies published in English, Portuguese, and Spanish between 2013 and 2023, from the PubMed, SciELO, and LILACS databases, using MeSH queries with the descriptors “blood donors,” “donor motivation,” and “motivation.” Original articles and review articles were included. A total of 207 articles were identified in February and March 2023, and after applying the inclusion criteria, 25 articles were selected for the study. This study was registered in the International Prospective Register of Systematic Reviews (PROSPERO- CRD42023431808) and concluded that the main motivator for blood donation was altruism and its “derivatives,” such as empathy and social responsibility. No conclusive answers were obtained from the analysis of these articles, which necessitates further studies on this topic

Monoclonal Gammopathy of Undetermined Significance (MGUS) and Smoldering Myeloma (SMM) describe pre-malignant conditions that are defined by the presence of monoclonal immunoglobulin production. They are found in up to 3% of individuals over the age of 50 with a rate of progression to multiple myeloma or other related malignancy of approximately 1% per year. Accurate diagnosis and risk-stratification are paramount to prevent end-organ damage and determine follow-up intervals. With the data currently available, treatment for MGUS is not recommended. Patients with newly diagnosed high- risk SMM should be referred to a centre and therapy with lenalidomide plus/minus dexamethasone for 2 years or participation in a clinical trial should be discussed.

Background: Sickle Cell Diseases (SCDs) are inborn and catastrophic processes on vascular endothelium, particularly at the capillaries. Methods: All patients with the SCDs were included. Results: We studied 222 males and 212 females with similar mean ages (30.8 vs 30.3 years, p>0.05, respectively). Beside Chronic Obstructive Pulmonary Disease (COPD) (25.2% vs 7.0%, p<0.001), smoking (23.8% vs 6.1%, p<0.001), alcohol (4.9% vs 0.4%, p<0.001), transfused red blood cells (RBCs) in their lives (48.1 vs 28.5 units, p=0.000), disseminated teeth losses (5.4% vs 1.4%, p<0.001), ileus (7.2% vs 1.4%, p<0.001), cirrhosis (8.1% vs 1.8%, p<0.001), leg ulcers (19.8% vs 7.0%, p<0.001), digital clubbing (14.8% vs 6.6%, p<0.001), coronary heart disease (CHD) (18.0% vs 13.2%, p<0.05), chronic renal disease (CRD) (9.9% vs 6.1%, p<0.05), and stroke (12.1% vs 7.5%, p<0.05) were all higher, and autosplenectomy (50.4% vs 53.3%, p<0.05) and mean age of mortality were lower in males, significantly (30.2 vs 33.3 years, p<0.05). Conclusion: The hardened RBCs-induced capillary endothelial damage initiates at birth, and terminates with multiorgan failures even at childhood in the SCDs. Parallel to the COPD, all of the atherosclerotic risk factors or consequences including smoking, alcohol, disseminated teeth losses, ileus, cirrhosis, leg ulcers, digital clubbing, CHD, CRD, and stroke were higher, and autosplenectomy and mean age of mortality were lower in males which can not be explained by effects of smoking and alcohol alone at the younger age. So COPD may have an atherosclerotic background, and low-dose aspirin plus low-dose warfarin may be life-saving treatment regimens in severe COPD.

In patients with inherited mild haemophilia treated with clotting factor concentrates, the risk of inhibitor development is lower than in severe haemophilia. We report the observation of a 38-year-old man, with mild haemophilia A, previously treated for mucosal bleeding, who presented no major immunization risk factors except an exon 23 missense mutation p.Arg2178Cys in the F8 gene in the C1 domain. He developed an inhibitor against Octocog alfa. The implication of the mutation in this particular immunization process is well known. Characterization of this inhibitor has been extended as a type I inhibitor with classical epitope mapping and solely directed against exogenous factor VIII. Due to the occurrence of an anamnestic response associated with a high titre inhibitor, we had recourse to Eptacog alfa during a critical bleeding situation.

A 60-year-old Surinamese woman was admitted to the department of Hematology because of refractory hypercalcemia and weight loss. Three months earlier she presented with aphasia and ataxia due to hypercalcemia (4.32 mmol/L). She was treated with intravenous fluids, calcitonin and pamidronate, resulting in normocalcemia. Serum Parathyroid Hormone (PTH) was 1.93 pmol/L. At that time, a bone marrow and Peripheral Blood (PB) examination and Fluorodeoxyglucose-Positron Emission Tomography (FDG-PET) scan did not show any abnormalities. Physical examination revealed multiple papules and diffuse scaling (Figure 1A). Current PB analysis demonstrated anemia (10 g/dL), thrombocytopenia (103 x 109/L), leukocytosis (20.5x 109/L; 38% lymphocytes), hypercalcemia (3.58 mmol/L) and elevated LDH (652 U/L). FDG-PET/CT at this time point showed ascites and splenomegaly.

Intravascular large B-cell lymphoma is a B-cell lineage neoplasm, which is typically recognized as a high-grade neoplasm with poor outcomes. Though rare, the cutaneous variant can present with multiple lesions of the skin and negative systemic staging. We present a case of intravascular large B-cell lymphoma first identified on cutaneous biopsy, with pancytopenia, in addition to negative bone marrow biopsy and serum protein electrophoresis. The patient is a 71-year-old male who presented with upper body pruritis and pancytopenia, as well as weight loss and fatigue. A full body skin exam incidentally revealed a well-demarcated, firm, bound down atrophic lichenified plaque on the right anterior thigh. A punch biopsy revealed large, atypical lymphoid cells exclusively localized within the lumina of variably sized blood vessels, immunoreactive for CD20, BCL6, MUM1, and CD5, but negative for CD3, CD10, cyclin D1, and TdT. A diagnosis of intravascular large B-cell lymphoma was made, and he was referred to oncology for further treatment. Full body skin exam is a mainstay of dermatologic practice, and it is essential to pursue biopsy with uncharacteristic lesions, regardless of the presenting concern.

The lectin pathway proteases (MASPs) are diverse in regard to their substrate selectivity and also have substrates outside the complement pathway. These proteases have been reported to cleave proteins of the coagulation cascade. MASP-1 has thrombin-like activity and therefore cleaves fibrinogen, fibrin and prothrombin. MASP-2 also participates in coagulation by cleaving prothrombin. Thus, these proteases are key players in the process of thrombosis and thrombolysis, therefore have implications in thrombotic vascular diseases. The cleaved fragments of fibrinogen and fibrin, consequently produced during coagulation and fibrinolysis, are important in the repair process and hence may have involvement in inflammatory and fibro-proliferative diseases. Also, altered levels of MBL and its associated serine proteases reported to be involved in different diseases such as cardiovascular diseases, diabetes, cancer, sepsis, stroke, COVID-19 etc. may have a role in thrombosis and thrombolysis. Upregulated lectin pathway-associated proteins predispose towards autoimmune disease susceptibility and may also exhibit off-target activities resulting in clot formation. Thus, the lectin pathway and its associated proteases are of great importance in normal circumstances where it plays a role in the maintenance of hemostasis and homeostasis, playing a preventive role in infections. Modulation in the level of MASPs proteases could be developed as a promising approach to treat a variety of infections and diseases.

Introduction: Rare Bleeding Disorders (RBDs), a group of inherited coagulation factor deficiencies, are more common in areas with a high rate of consanguineous marriages. Data on the frequency and outcome of RBDs among children in Oman are limited. Aim: This study evaluated the clinical profile and bleeding outcomes of paediatric RBDs at the Royal Hospital, the largest referring centre in Oman, from 2010 to 2020. Methods: This retrospective descriptive cohort study includes all Omani children, less than 13 years of age, diagnosed with RBDs, and followed at the Royal Hospital. The patient’s data was retrieved from the electronic chart system. Results: Forty-one patients (24 males and 17 females) were included. Based on factor level at diagnosis, 34(83%) patients have factor VII deficiency, four (9.7%) patients have factor XIII deficiency, one (2.4%) patient has fibrinogen deficiency, and one patient (2.4%) has factor X deficiency. Four (9.4%) patients required blood transfusion for active bleeding, 15(36.6%) patients were treated on demand with tranexamic acid, and 3(7.3%) patients were on regular prophylaxis with tranexamic acid. The mean period of follow-up was 3.15 years. The majority (87.8%) of patients had no active bleeding, and only two (4.9%) patients developed severe bleeding. The mean annual bleeding rate outcome was 0.2, 0.5, and 7 for patients with FVII, FXIII, and FX, respectively. Conclusions: Omani children with rare bleeding disorders are commonly diagnosed incidentally. They usually do not require active treatment because their bleeding outcome is favourable.

The effect caused by medium acidification to pH 6.5 on the agglutinating ability of seven anti-A MAbs (2-8, 2-17, 2-19, 2-22, 2-23, 2-28 from Workshop IV and BRIC-145) and their inhibition by glycoconjugates obtained from the membranes of A erythrocytes by enzymatic treatment and chloroform-methanol method, followed by ion-exchange gel chromatography, was evaluated. Medium acidification most significantly reduced the agglutination of a erythrocytes by IgM MAbs 2-17, 2-19, 2-22 and 2-28 and had a weaker manifestation in more alkaline IgM MAbs 2-8, 2-23 and BRIC-145. The inhibition of the lipid isotypes a lp-00, A lp-0, and Alp-3 (with the isoionic pH points of 8.1, 8.0 and 6.55) and the protein ones A pr-1 and A pr-3 (with the isoionic pH points of 7.15 and 6.45) was assessed in scores. Acidification to pH value 6.5 for MAbs 2-28 and 2-17 caused a considerable reduction in inhibition with acid Alp-3 and Apr-3 with slightly increased inhibition with alkaline A lp-00; MAbs 2-22 and 2-19 insignificantly and selectively altered the inhibitory capacity by more alkaline types of glycoconjugates. MAb 2-8 hardly changed inhibition. All the above illustrates a significant role played by both the charge of glycotopes and antibodies and the specificity - the selective avidity of MAbs to certain isotypes of a glycotopes.

Background: Sensitivity of classical coagulation assays by using mammalian plasmas to pro- and anticoagulant compounds including venom or toxins occurs on a microscale level (micrograms). Although it improves responses to agonists, recalcification triggers a relatively fast thrombin formation process. The Recalcification Time (RT) of factor XII- deficient Chicken Plasma (CP) is comparatively long (=1800 seconds) when compared to human plasma or others. Our objective was to compare its sensitivity with that presented by human plasma samples to Unfractionated Heparin (UH), a prototype anticoagulant compound, under similar conditions through rotational thromboelastometry. Methods: To find doses of UH sufficient enough to prolong the Clotting Time (CT) parameter of these activated plasmas to values within their normal RT ranges. Results: In total, 0.0065±0.0009 IU of UH (n=6) was detected in 260μL of CP samples, but only 0.125±0.012 IU of UH was sufficient to induce a similar effect in activated human plasma samples. Conclusion: The higher sensitivity of CP to anticoagulants could be useful for (a) detection of anticoagulant compounds in substances of unknown origin; (b) purification procedures of anticoagulant toxins from crude animal venoms and (c) determination of relative potencies of agonists and their selective antagonists such as pharmaceutical agents, antivenoms or natural inhibitors of venom toxins with a better result in kinetic clothing parameters. Keywords: Coagulation process; Chicken; Factor XII deficiency; Animal models; Biomedical research; Rotational thromboelastometry