The human pegivirus: A new name for an "ancient" virus. Can transfusion medicine come up with something new?

Human pegivirus (HPgV, formerly called GB virus C/hepatitis G virus) is a poorly understood RNA virus of the Flaviviridae family. The HPgV infection is common worldwide and the virus is likely transmitted by blood products. At this time, no causal association between HPgV and human diseases has been identified. While waiting for new findings to better understand the Pegivirus genus, the aim of our narrative review is to discuss the currently available information on HPgV focusing on its prevalence in blood donors and its potential threat to transfusion safety.

Patient blood management: a fresh look at a fresh approach to blood transfusion.

The overall use of allogeneic blood transfusions in clinical practice remains relatively high and still varies widely among centres and practitioners. Moreover, allogeneic blood transfusions have historically been linked with risks and complications: some of them (e.g. transfusion reactions and transmission of pathogens) have been largely mitigated through advancements in blood banking whereas some others (e.g. immunomodulation and transfusion-related acute lung injury) appear to have more subtle etiologies and are more difficult to tackle. Furthermore, blood transfusions are costly and the supply of blood is limited. Finally, evidence indicates that a great number of the critically ill patients who are being transfused today may not be having tangible benefits from the transfusion. Patient blood management is an evidence-based, multidisciplinary, multimodal, and patient-tailored approach aimed at reducing or eliminating the need for allogeneic transfusion by managing anaemia, perioperative blood conservation, surgical haemostasis, and blood as well as plasma-derivative drug use. From this point of view, the reduction of allogeneic blood usage is not an end in itself but a tool to achieve better patient clinical outcome. This article focuses on the three-pillar matrix of patient blood management where the understanding of basic physiology and pathophysiology is at the core of evidence-based approaches to optimizing erythropoiesis, minimising bleeding and tolerating anemia. Anesthesiologists and critical care physicians clearly have a key role in patient blood management programmes are and should incorporate its principles into clinical practice-based initiatives that improve patient safety and clinical outcomes.

A case of subacute cutaneous lupus erythematosus in a patient with mixed connective tissue disease: successful treatment with plasmapheresis and rituximab.

A 30-year-old woman affected by Mixed Connective Tissue Disease with scleroderma spectrum developed a facial eruption, a clinical and histological characteristic of subacute cutaneous lupus erythematosus (SCLE). Speckled anti-nuclear antibodies, high-titer anti-ribonucleoprotein1, anti-Sm, anti-Cardiolipin (aCL) IgG/IgM, and anti-Ro/SSA antibodies were positive. SCLE was resistant to Azathioprine, Hydroxychloroquine, and Methotrexate while Mycophenolate Mofetil was suspended due to side effects. Subsequently, the patient was treated with three cycles of therapeutic plasma exchange (TPE) followed, one month after the last TPE, by the anti-CD20 antibody Rituximab (RTX) (375 mg/m(2) weekly for 4 weeks). Eight and 16 months later the patient received other two TPE and RTX cycles, respectively. This therapeutic approach has allowed to obtain a complete skin healing persistent even after 8-month follow-up. Moreover, mitigation of Raynaud's phenomenon, resolution of alopecia, and a decline of aCL IgG/IgM and anti-Ro/SSA antibodies were observed.

Models for institutional and professional accreditation of haemophilia centres in Italy.

The Health Commission of the Conference between the Italian State and Regions recognized the need to establish an institutional accreditation model for Haemophilia Centres (HCs) to be implemented by 21 Regions in order to provide patients with haemophilia and allied inherited coagulations disorders with high and uniform standards of care. The Italian National Blood Centre, on behalf of the Commission, convened a panel of clinicians, patients, experts, representatives from Regions and Ministry of Health. The agreed methodology included: systematic literature review and best practice collection, analysis of provisions and regulations of currently available services, priority setting, definition of principles and criteria for the development of recommendations on the optimal requirements for HCs. The result was the formulation of two recommendations sets. Two sets of recommendations were produced. The first concerns regional policy planning, in which the following aspects of comprehensive haemophilia care should be considered for implementation: monitoring and auditing, multidisciplinary approach to clinical care, protocols for emergency management, home treatment and its monitoring, patient registries, drug availability and procurement, recruitment and training of health care professionals. The second set concerns the accreditation process and lists 23 organizational requirements for level 1 HCs and 4 additional requirements for level 2 HCs. These recommendations help to provide Italian Regional Health Authorities with an organizational framework for the provision of comprehensive care to patients with inherited coagulation disorders based on current scientific evidence.

Polymorphism of the Duffy erythrocyte chemokine receptor in Italian patients with Behçet's disease.

This study examined the hypothesis that the polymorphism of Duffy antigen receptor for chemokines (DARC) predisposes to and/or influences the clinical manifestations of Behçet's disease. The serum levels of IL-8 and monocyte chemotactic peptide (MCP)-1, two DARC-binding chemokines, were investigated and related to this polymorphism. Twenty-eight patients with Behçet's disease and 30 healthy blood donors were included in the study. No null phenotypes were found among the patients studied, and the frequencies of the other phenotypes (Fy((a+b-)), Fy((a+b+)), and Fy((a-b+))) did not significantly differ from those found in the blood donor group or reported in the general Caucasian population. No difference was found between the single phenotypes in terms of IL-8 and MCP-1 serum levels, and no relevant association between the clinical characteristics, Behçet's disease-associated human leukocyte antigen (HLA)-B51, and single phenotypes was observed. This investigation indicates that DARC is not a genetic trait significantly associated with or predisposing to Behçet's disease, at least in Caucasian Italians. However, the role of this polymorphism in the development and in the clinical course of the disease awaits further investigation.

Lymphocyte T subsets and natural killer cells in Italian and Philippino blood donors.

BACKGROUND AND OBJECTIVES: The characterization of lymphocyte subsets in blood donors has been utilized to determine the normal ranges that can be related to race. A study was performed in blood donors from two racial groups - Caucasian (Italians) and Asian (Philippinos) - to define respective T-lymphocyte subsets and levels of cytokines. MATERIALS AND METHODS: Ninety-two blood donors (46 Italians and 46 Philippinos) were enrolled. Blood count and immunophenotyping of lymphocytes by flow cytometry were carried out, and cytokine production was tested in six blood donors of each group. RESULTS: Philippino blood donors showed a significantly higher mean value of leucocytes (P = 0.01) and lymphocytes (P < 0.001) than Italians. The mean absolute count of lymphocyte subsets CD3- CD16+ CD56+ and CD3+ CD8+ were both significantly higher in Philippino than in Italian subjects, respectively, P < 0.01 and P < 0.0001. Philippinos showed a statistically significant higher frequency of lymphocytes producing interferon-gamma (IFN-gamma) compared to Italians (P = 0.02). CONCLUSIONS: T-lymphocyte subsets in Italian and Philippino blood donors seem to be correlated to ethnic background. The higher levels of CD3+ CD8+ T cells, natural killer (NK) cells and IFN-gamma-producing cells found in Philippinos suggest leucoreduction in Asian blood donors.

Induction of alkaline phosphatase activity in chronic myeloid leukemia cells: in vitro studies and speculative hypotheses.

Three patients with Ph chromosome + chronic myeloid leukemia (CML) in chronic phase suffered from intercurrent pleuritis of undefined origin. At that time, leucocyte alkaline phosphatase activity (LAPA) score was low for circulating neutrophils, but high for those from pleural effusion. LAP negative circulating CML granulocytes were incubated with the pleural liquid: after 40-70 hr, almost all were intensely LAP positive. This finding suggests that the low LAPA score in resting CML neutrophils is attributable to the absence of appropriate stimuli rather than to an incapacity to synthesize the enzyme.