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1.  Inflammatory response following neutrophil recovery postchemotherapy in acute myeloid leukemia cases without evidence of infection: role of homing of neutrophils 
Neutropenic sepsis is a common clinical entity occurring in postchemotherapy patients. Infection may not be the cause of fever in such patients after neutrophil-count recovery. Herein, we present two patients who developed fever during the neutropenic phase of induction chemotherapy and were treated with broad-spectrum antibiotics until they were no longer febrile and had recovered their neutrophil count. Being off antibiotics, they redeveloped fever within 48–72 hours. These fevers seemed to be secondary to postinfectious inflammatory response and not infection, supported by the fact that adequate antibiotic treatment was given and the collected fluid contained neutrophils but the cultures were negative. We hypothesize an explanation for this phenomenon based on the “homing of neutrophils” to bone marrow, which involves chemoattraction of CXC chemokine receptor (CXCR)-4 expressed on neutrophils towards the chemokine stromal cell-derived factor (SDF)-1 (CXCL12) expressed constitutively by bone marrow. Literature has shown that elevation of SDF-1 levels at injured/inflamed sites might create a similar gradient. This gradient results in the migration of neutrophils to the sites of previous injury/inflammation, leading to the formation of sterile abscesses. Based on our cases, we also conclude that antibiotics do not prevent the formation or treat such sterile “abscesses”; however, the drainage of these “abscesses” and treatment with anti-inflammatory agents are useful in such cases.
doi:10.2147/JBM.S53616
PMCID: PMC3956695  PMID: 24648782
SDF-1; CXCR4; postinfectious inflammatory response; homing of neutrophils
2.  Applying spatial epidemiology to hematological disease using R: a guide for hematologists and oncologists 
“Spatial statistics” is an academic field that deals with the statistical analysis of spatial data, and has been applied to econometrics and various other policy fields. These methods are easily applied by hematologists and oncologists using better and much less expensive software. To encourage physicians to use these methods, this review introduces the methods and demonstrates the analyses using R and FleXScan, which can be freely downloaded from the website, with sample data. It is demonstrated that spatial analysis can be used by physicians to analyze hematological diseases. In addition, applying the technique presented to the investigation of patient prognoses may enable generation of data that are also useful for solving health policy-related problems, such as the optimal distribution of medical resources.
doi:10.2147/JBM.S57944
PMCID: PMC3949695  PMID: 24624001
leukemia; malignant lymphoma; Tango’s index; spatial regression model
3.  Clinical utility of rivaroxaban in stroke prevention associated with nonvalvular atrial fibrillation – patient considerations 
The direct factor Xa inhibitor rivaroxaban was the first within the group of orally available direct factor Xa inhibitors to gain clinical approval for oral anticoagulation in patients with nonvalvular atrial fibrillation in 2011. The –xabans, as drugs from the group of oral direct factor Xa inhibitors are often referred to, comprise currently three drugs (apixaban, edoxaban, and rivaroxaban) with clinical approval for a variety of clinical indications that require oral anticoagulation therapy. The number of patients with nonvalvular atrial fibrillation requiring long-term oral anticoagulation therapy for the prevention of stroke and systemic embolism is predicted to increase up to five times by 2050. Many of these patients will be within the elderly and aging population that is at a higher risk of stroke and also at a higher risk for bleeding events. This requires novel options for efficient and safe oral anticoagulation, and rivaroxaban is one of the novel oral anticoagulants that have been shown to be at least as effective as vitamin K antagonists in patients with nonvalvular atrial fibrillation. Furthermore, like all of the novel oral anticoagulants, rivaroxaban provides a significant reduction in intracerebral hemorrhage compared with vitamin K antagonists such as warfarin. The clinical utility of oral anticoagulation with rivaroxaban in patients with nonvalvular atrial fibrillation is discussed here, along with special patient considerations, including impaired renal function, switching from a vitamin K antagonist, and patients with concomitant acute coronary syndrome.
doi:10.2147/JBM.S32957
PMCID: PMC3938442  PMID: 24591854
rivaroxaban; factor Xa; stroke; intracranial bleeding; vitamin K antagonist; atrial fibrillation; acute coronary syndrome; triple therapy; BAY 59-7939; DX9065a; DU-176b
4.  Management of thrombotic thrombocytopenic purpura: current perspectives 
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy which causes significant morbidity and mortality unless promptly recognized and treated. The underlying pathogenesis of TTP is a severe deficiency in ADAMTS13 activity, a metalloprotease that cleaves ultralarge von Willebrand factor multimers. This deficiency is either autoantibody mediated (acquired TTP) or due to deleterious mutations in the gene encoding ADAMTS13 (congenital TTP). The elucidation of this disease mechanism has reinforced the rationale and place of current therapies (eg, plasma exchange) as well as providing a basis for the prospective evaluation of immunotherapy with rituximab in addition to classic immunosuppression (eg, corticosteroid) in autoantibody-mediated TTP. This review discusses the current evidence base for therapeutic interventions in acquired and congenital TTP as well as providing a practical approach to the other aspects of investigation and management for which a firm evidence base is lacking. Novel agents that are currently being evaluated in prospective trials and future directions of therapy are also discussed which are expected to make an important contribution to improving outcomes in patients with TTP.
doi:10.2147/JBM.S46458
PMCID: PMC3921093  PMID: 24523598
TTP; rituximab; ADAMTS13
5.  Toward a patient-based paradigm for blood transfusion 
The current “manufacturing paradigm” of transfusion practice has detached transfusion from the clinical environment. As an example, fresh whole blood in large-volume hemorrhage may be superior to whole blood reconstituted from multiple components. Multicomponent apheresis can overcome logistical difficulties in matching patient needs with fresh component availability and can deliver the benefits of fresh whole blood. Because of the different transfusion needs of patients in emerging economies and the vulnerability of these blood systems to emerging infections, fresh whole blood and multicomponent apheresis can better meet patient needs when compared with transplants of the “manufacturing paradigm”. We propose that patient blood management, along with panels of repeat, paid, accredited apheresis and fresh whole-blood donors can be used in emerging economies to support decentralized blood services. This alternative transfusion–medicine paradigm could eventually also be adopted by established economies to focus transfusion medicine on local patient needs and to alleviate the problem of the aging volunteer donor base.
Video abstract
doi:10.2147/JBM.S55769
PMCID: PMC3917954  PMID: 24520208
indications; emerging countries; patient blood management
8.  A common signaling pathway is activated in erythroid cells expressing high levels of fetal hemoglobin: a potential role for cAMP-elevating agents in β-globin disorders 
Journal of Blood Medicine  2013;4:149-159.
Background
Although erythroid cells prepared from fetal liver, cord blood, or blood from β-thalassemia patients are known to express fetal hemoglobin at high levels, the underlying mechanisms remain elusive. We previously showed that cyclic nucleotides such as cAMP and cGMP induce fetal hemoglobin expression in primary erythroid cells. Here we report that cAMP signaling contributes to high-level fetal hemoglobin expression in erythroid cells prepared from cord blood and β-thalassemia.
Methods
The status of the cAMP signaling pathway was investigated using primary erythroid cells prepared from cord blood and the mononuclear cells of patients with β-thalassemia; erythroid cells from adult bone marrow mononuclear cells served as the control.
Results
We found that intracellular cAMP levels were higher in erythroid cells from cord blood and β-thalassemia than from adult bone marrow. Protein kinase A activity levels and cAMP-response element binding protein phosphorylation were higher in erythroid cells from cord blood or β-thalassemia than in adult bone marrow progenitors. Mitogen-activated protein kinase pathways, which play a role in fetal hemoglobin expression, were not consistently activated in cord blood or β-thalassemia erythroid cells. When cAMP signaling was activated in adult erythroid cells, fetal hemoglobin was induced at high levels and associated with reduced expression of BCL11A, a silencer of the β-globin gene.
Conclusion
These results suggest that activated cAMP signaling may be a common mechanism among erythroid cells with high fetal hemoglobin levels, in part because of downregulation of BCL11A. Activation of the cAMP signaling pathway with cAMP-elevating agents may prove to be an important signaling mechanism to reactivate fetal hemoglobin expression in erythroid cells.
doi:10.2147/JBM.S54671
PMCID: PMC3862583  PMID: 24353450
cAMP; signaling; fetal hemoglobin; β-globin disorders; cord blood; β-thalassemia
9.  Contribution of blood platelets to vascular pathology in Alzheimer’s disease 
Journal of Blood Medicine  2013;4:141-147.
Cerebral amyloid angiopathy (CAA) is a critical factor in the pathogenesis of Alzheimer’s disease (AD). In the clinical setting, nearly 98% AD patients have CAA, and 75% of these patients are rated as severe CAA. It is characterized by the deposition of the β-amyloid peptide (mainly Aβ40) in the walls of cerebral vessels, which induces the degeneration of vessel wall components, reduces cerebral blood flow, and aggravates cognitive decline. Platelets are anuclear cell fragments from bone marrow megakaryocytes and their function in hemostasis and thrombosis has long been recognized. Recently, increasing evidence suggests that platelet activation can also mediate the onset and development of CAA. First, platelet activation and adhesion to a vessel wall is the initial step of vascular injury. Activated platelets contribute to more than 90% circulating Aβ (mainly Aβ1-40), which in turn activates platelets and results in the vicious cycle of Aβ overproduction in damaged vessel. Second, the uncontrolled activation of platelets leads to a chronic inflammatory reaction by secretion of chemokines (eg, platelet factor 4 [PF4], regulated upon activation normal T-cell expressed and presumably secreted [RANTES], and macrophage inflammatory protein [MIP-1α]), interleukins (IL-1β, IL-7, and IL-8), prostaglandins, and CD40 ligand (CD40L). The interaction of these biological response modulators with platelets, endothelial cells, and leukocytes establishes a localized inflammatory response that contributes to CAA formation. Finally, activated platelets are the upholder of fibrin clots, which are structurally abnormal and resistant to degradation in the presence of Aβ42. Thus, opinion has emerged that targeting blood platelets may provide a new avenue for anti-AD therapy.
doi:10.2147/JBM.S45071
PMCID: PMC3825710  PMID: 24235853
cerebral amyloid angiopathy; Aβ40; chronic inflammatory; cerebral vessel
10.  Relative efficacy of bivalirudin versus heparin monotherapy in patients with ST-segment elevation myocardial infarction treated with primary percutaneous coronary intervention: a network meta-analysis 
Journal of Blood Medicine  2013;4:129-140.
In the absence of head-to-head clinical data, the objective of this study was to indirectly compare the efficacy and safety of a bivalirudin-based anticoagulation strategy with that of heparin monotherapy in patients with ST-elevation myocardial infarction (STEMI) intended for primary percutaneous coronary intervention. A systematic literature review was performed to identify randomized controlled trials to build a network of bivalirudin and heparin monotherapy strategies in STEMI patients using heparin, with glycoprotein IIb/IIIa inhibitor as a common reference strategy. At 30 days, the bivalirudin-based strategy was expected to result in lower mortality rates than heparin monotherapy (odds ratio [OR], 0.55; credible limit [CrL], 0.32–0.95). This relationship was sustained at 1 year. At 30 days, the risk for stroke (OR, 0.88; CrL, 0.37–2.13), myocardial infarction (OR, 0.79; CrL, 0.40–1.55), and thrombolysis in myocardial infarction major and minor bleedings (OR, 0.66; CrL, 0.45–0.98) tended to be numerically reduced with bivalirudin in comparison with heparin monotherapy. For patients with STEMI intended for primary percutaneous coronary intervention, bivalirudin is associated with lower mortality rates in comparison with heparin monotherapy. This study suggests that bivalirudin is more effective and safer than heparin monotherapy and should therefore be preferred over heparin monotherapy.
doi:10.2147/JBM.S50595
PMCID: PMC3794970  PMID: 24124401
primary angioplasty; STEMI; pharmacology
11.  Association of vitamin D deficiency and hyperparathyroidism with anemia: a cross-sectional study 
Journal of Blood Medicine  2013;4:123-128.
Vitamin D deficiency and anemia are common in the Middle East, and vitamin D deficiency and hyperparathyroidism have been reported to be associated with an increased prevalence of anemia. In this study, the hypothesis that vitamin D deficiency and hyperparathyroidism may be associated with anemia in a Bahraini population was tested. Association of hyperparathyroidism and vitamin D levels (deficiency and insufficiency) with anemia was investigated in 421 Bahrainis (213 males and 208 females). In females, the prevalence of anemia was significantly associated with vitamin D deficiency independent of parathyroid hormone levels (odds ratio: 2.9; 95% confidence interval: 2.3–10.5; P = 0.001). In females, the prevalence of anemia appeared to be significantly associated with hyperparathyroidism (odds ratio: 2.1; 95% confidence interval: 1.2–3.7; P = 0.01); however, this significant association disappeared when adjusted for vitamin D deficiency (odds ratio: 1.6; 95% confidence interval: 0.75–6.5; P = 0.154). Results from this study suggest that vitamin D deficiency is independently associated with anemia in females but not males. Further studies to determine whether vitamin D supplementation could be used to treat anemia are warranted.
doi:10.2147/JBM.S47171
PMCID: PMC3770718  PMID: 24039460
vitamin D; deficiency; hyperparathyroidism; anemia
12.  Review of current classification, molecular alterations, and tyrosine kinase inhibitor therapies in myeloproliferative disorders with hypereosinophilia 
Journal of Blood Medicine  2013;4:111-121.
Recent advances in our understanding of the molecular mechanisms underlying hypereosinophilia have led to the development of a ‘molecular’ classification of myeloproliferative disorders with eosinophilia. The revised 2008 World Health Organization classification of myeloid neoplasms included a new category called “myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1.” Despite the molecular heterogeneity of PDGFR (platelet-derived growth factor receptor) rearrangements, tyrosine kinase inhibitors at low dose induce rapid and complete hematological remission in the majority of these patients. Other kinase inhibitors are promising. Further discoveries of new molecular alterations will direct the development of new specific inhibitors. In this review, an update of the classifications of myeloproliferative disorders associated with hypereosinophilia is discussed together with open and controversial questions. Molecular mechanisms and promising results of tyrosine kinase inhibitor treatments are reviewed.
doi:10.2147/JBM.S33142
PMCID: PMC3747024  PMID: 23976869
hypereosinophilia; classification; myeloproliferative disorders; molecular alterations; tyrosine kinase inhibitor
13.  Deferasirox: appraisal of safety and efficacy in long-term therapy 
Journal of Blood Medicine  2013;4:101-110.
Deferasirox is a once-daily, oral iron chelator that is widely used in the management of patients with transfusional hemosiderosis. Several Phase II trials along with their respective extension studies as well as a Phase III trial have established the efficacy and safety of this novel agent in transfusion-dependent patients with β-thalassemia, sickle-cell disease and bone marrow-failure syndromes, including myelodysplastic syndrome and aplastic anemia. Data from various clinical trials show that a deferasirox dose of 20 mg/kg/day stabilizes serum ferritin levels and liver iron concentration, while a dose of 30–40 mg/kg/day reduces these parameters and achieves negative iron balance in red cell transfusion-dependent patients with iron overload. Across various pivotal clinical trials, deferasirox was well tolerated, with the most common adverse events being gastrointestinal disturbances, skin rash, nonprogressive increases in serum creatinine, and elevations in liver enzyme levels. Longer-term extension studies have also confirmed the efficacy and safety of deferasirox. However, it is essential that patients on deferasirox therapy are monitored regularly to ensure timely management for any adverse events that may occur with long-term therapy.
doi:10.2147/JBM.S35478
PMCID: PMC3743529  PMID: 23966805
deferasirox; iron overload; thalassemia; sickle-cell disease; myelodysplastic syndrome
14.  Current strategies to minimize the bleeding risk of warfarin 
For many decades, the vitamin K antagonist warfarin has been the mainstay of treatment for various conditions that require anticoagulation, including atrial fibrillation. Although the efficacy of warfarin in both prevention and treatment of thrombosis has been demonstrated in numerous randomized clinical studies, one of the major concerns that remains is the risk of bleeding. Although the net benefit of warfarin has been demonstrated in large clinical trials, physicians and patients alike are often reluctant to use warfarin because of the bleeding risk. Bleeding in patients on warfarin is generally minor requiring no intervention, but the development of a major bleeding complication is associated with significant morbidity and can even be fatal. Numerous risk factors that increase the probability of having a hemorrhage while on warfarin have been identified, and bleeding risk scores have been developed. Various strategies to reduce bleeding risks have been developed and have become more important, since the use of warfarin and other anticoagulants continues to increase. This paper provides a concise review of bleeding risk factors, while outlining recommendations both physician and patients can incorporate to help reduce the risk of bleeding.
doi:10.2147/JBM.S41404
PMCID: PMC3760283  PMID: 24019755
hemorrhage; warfarin; thrombosis; anticoagulants; dabigatran; vitamin K antagonist
16.  The protective effects of lafutidine for bortezomib induced peripheral neuropathy 
Peripheral neuropathy (PN) caused by bortezomib is an important complication of multiple myeloma. Subcutaneous injection of bortezomib reduced PN, but 24% of cases were grade 2 PN and 6% of cases were grade 3 PN. PN higher than grade 2 was not resolved by subcutaneous injection. PN higher than grade 3 has serious dose limiting toxicity and is the cause of discontinuing bortezomib treatment. Lafutidine is an H2-blocker with gastroprotective activity and is thought to function by increasing mucosal blood flow via capsaicin sensitive neurons. The same activity of lafutidine is considered to improve glossodynia and taxane induced PN. We hypothesized that lafutidine prevents or improves PN that is caused by bortezomib. In the current study, bortezomib was administered in the usual manner (intravenous administration of bortezomib 1.3 mg/m2, twice a week for 2 weeks, followed by 1 week without treatment) for up to four cycles to compare our data with other studies. Lafutidine was administered orally at a dose of 10 mg twice daily. In our eight evaluated cases, the total occurrence of PN was four out of eight patients (50%). There were only grade 1 PN (4 out of 8) cases, and no cases higher than grade 2. We conclude that (1) the total occurrence of PN was not improved, (2) there was no PN after the first course, (3) there were only grade 1 cases and there were no cases higher than grade 2, and (4) no cases discontinued bortezomib treatment because of PN. This is the first report showing that lafutidine is useful for the amelioration of bortezomib induced PN.
doi:10.2147/JBM.S44127
PMCID: PMC3712744  PMID: 23874126
bortezomib induced peripheral neuropathy; lafutidine; capsaicin sensitive neurons; calcitonin gene-related peptide; transient receptor potential 1; multiple myeloma
17.  Iron stores in regular blood donors in Lagos, Nigeria 
Background
Apart from challenging the bone marrow to increase its red cell production, thereby producing more blood for the donor, regular blood donation has been shown to have several benefits, one of which is preventing accumulation of body iron which can cause free radical formation in the body. This study was carried out to assess body iron stores in regular blood donors.
Methods
A total of 52 regular (study) and 30 first-time (control) volunteer blood donors were studied prospectively. Twenty milliliters of venous blood was drawn from each subject, 5 mL of which was put into sodium ethylenediamine tetra-acetic acid specimen bottles for a full blood count, including red blood cell indices. The remaining sample was allowed to clot in a plain container, and the serum was then retrieved for serum ferritin, serum iron, and serum transferrin receptor measurement by enzyme-linked immunosorbent assay.
Results
Mean hemoglobin and packed cell volume in the study group (13.47 ± 2.36 g/dL and 42.00 ± 7.10, respectively, P = 0.303) were not significantly higher than in the control group (12.98 ± 1.30 g/dL and 39.76 ± 4.41, respectively, P = 0.119). Mean serum ferritin was 102.46 ± 80.26 ng/mL in the control group and 41.46 ± 40.33 ng/mL in the study group (P = 0.001). Mean serum ferritin for women in the study group (28.02 ± 25.00 ng/mL) was significantly lower than for women in the control group (56.35 ± 34.03 ng/mL, P = 0.014). Similarly, men in the study group had a lower mean serum ferritin (48.57 ± 45.17 ng/mL) than men in the control group (145.49 ± 87.74 ng/mL, P = 0.00). The mean serum transferrin receptor value was higher in the study group (1.56 ± 0.88 μg/mL) than in the control group (1.19 ± 0.38 μg/mL, P = 0.033).
Conclusion
These findings suggest that hemoglobin concentration, packed cell volume, and serum iron levels are not significantly affected by regular blood donation and that regular blood donors appear to have reduced iron stores compared with controls.
doi:10.2147/JBM.S42210
PMCID: PMC3685448  PMID: 23807865
blood donors; hemoglobin; serum ferritin; serum iron; transferrin receptors
18.  New developments in the management of congenital Factor XIII deficiency 
Congenital Factor XIII (FXIII) deficiency is a rare, inherited, autosomal recessive coagulation disorder. Most mutations of this condition are found in the A-subunit with almost half these being missense mutations. Globally, approximately one in three million people suffer from this deficiency. Factor XIII deficiency is associated with severe life threatening bleeding, intracranial hemorrhage, impaired wound healing, and recurrent pregnancy losses. FXIII is known to have a potential role in mediating inflammatory processes, insulin resistance, bone metabolism, neoplasia, and angiogenesis. The algorithm provided for FXIII diagnosis and classification will enable prompt identification and early intervention for controlling potential life threatening complications. Prophylactic replacement therapy using blood products containing FXIII such as fresh frozen plasma, cryoprecipitate, or using FXIII concentrate remains the mainstay for the management of FXIII deficiency. In most parts of the world, cryoprecipitate and plasma transfusions are the only treatments available. Management developments have revealed the effectiveness and safety of recombinant FXIII concentrate for prophylaxis and treatment. The aim of this review is to provide an overview of advancements made in the management of FXIII deficiency from the time it was first detected, highlighting novel developments made in recent years. Greater research is warranted in identifying novel approaches to manage FXIII deficiency in light of its underlying pathophysiology.
doi:10.2147/JBM.S32693
PMCID: PMC3674014  PMID: 23761984
Factor XIII; Factor XIII deficiency; treatment; diagnosis; inherited coagulative disorders
19.  Serum ferritin levels in adults with sickle cell disease in Lagos, Nigeria 
Background
Serum ferritin is considered to be one of the most important tools in the measurement of iron balance in steady-state sickle cell disease. Increased gastrointestinal absorption of iron has been reported in sickle cell disease because of the associated chronic hemolysis, and it is also thought that repeated red cell transfusion consequent to chronic hemolysis and anemia causes excessive iron levels. The aim of this study was to determine overall and gender-specific mean ferritin levels in patients with steady-state sickle cell disease in order to establish the prevalence of iron deficiency and overload.
Methods
This was a cross-sectional study in homozygous patients with sickle cell disease attending the sickle cell clinic at Lagos State University Teaching Hospital, Ikeja. A 5 mL blood sample was collected in plain bottles from consenting participants during steady-state periods. The serum was separated and analyzed for ferritin by enzyme-linked immunosorbent assay. Another 5 mL sample was collected for a full blood count, done on the same day of collection, to determine red blood cell indices, ie, mean cell volume, mean cell hemoglobin concentration, and mean corpuscular hemoglobin concentration. The Pearson Chi-square test was used for statistical analysis. The differences were considered to be statistically significant when P was <0.05.
Results
In total, 103 patients were recruited for this study and comprised 58 women (56.40%) and 45 men (43.70%). The overall mean ferritin concentration was 93.72 ± 92.24 ng/mL. The mean ferritin concentration in the women was 92.00 ± 88.07 ng/mL and in men was 96.41 ± 99.80 ng/mL. Only eight (7.76%) of the 103 patients had a serum ferritin level < 15 ng/mL, while two subjects (1.94%) had a serum a ferritin level > 300 ng/mL. Ninety-three subjects (90.29%) had serum ferritin within the normal reference range of 15–300 ng/mL.
Conclusion
In this study, 90% of subjects with sickle cell disease had normal iron stores; serum ferritin was higher in men than in women, and iron deficiency was more common than overload in the disease.
doi:10.2147/JBM.S42212
PMCID: PMC3666661  PMID: 23723723
serum ferritin levels; sickle cell disease
20.  Treatment of patients with von Willebrand disease [Erratum] 
doi:10.2147/JBM.S35154
PMCID: PMC3665495
21.  Barriers and perceived limitations to early treatment of hemophilia 
Early treatment of bleeds in hemophilia patients, both with and without inhibitors, has been shown to be of immense benefit in the overall clinical outcome. Despite the advantages of treating the bleeding episodes early, significant barriers and limitations remain. The aim of this review is to highlight the various barriers and perceived limitations to early therapy of bleeding episodes, especially in patients who have developed inhibitors to factor VIII. The peer-reviewed literature was searched for articles on hemophilia patients, with and without inhibitors, and early treatment, to identify the barriers to early treatment and potential impact on patient outcomes. The most important barrier is the educational barrier, which involves lack of awareness among patients regarding the signs of a bleed, as well as importance of early therapy. It is also common for parents or caregivers of school-age children to exhibit inconvenience and scheduling barriers. Distance to the treatment center can also play a role here. Some patients experience financial barriers related to cost of clotting factor products, insurance coverage, or insurance caps and out-of-pocket costs. Rarely, there can also be problems related to venous access or home infusion. Lastly, multiple psychosocial barriers can prevent adherence to treatment regimens. Identification and addressing these individual barriers will result in improved compliance rates, prevent joint damage, be more cost-effective, and lead to better overall health of these patients.
doi:10.2147/JBM.S43734
PMCID: PMC3660133  PMID: 23700376
hemophilia A; hemophilia B; inhibitors; outcomes; quality of life; cost of care
22.  Severe hypernatremia and hyperchloremia in an elderly patient with IgG-kappa-type multiple myeloma 
A 77-year-old male was admitted to hospital after suffering a pelvic bone fracture in a road traffic accident and was incidentally found to have IgG-kappa-type multiple myeloma with hypercalcemia. The patient was also noted to be hypokalemic and had low HCO3−, with possible damage to the distal tubules in the kidneys. When the treatment was begun with bortezomib/dexamethasone/elcatonin and sodium bicarbonate (NaHCO3) in normal saline (equivalent to a daily sodium dose of 200 millimoles per liter [mmol/L]), the patient was in a state of poor oral fluid intake. The patient developed hypernatremia and hyperchloremia, with a peak serum sodium and chloride levels of 183 mmol/L and 153 mmol/L, respectively, at the sixth day after the start of treatment. Following the switch of the intravenous infusions from normal saline to soldem 1 and soldem 3 solutions, these high-electrolyte levels gradually returned to normal over the next 7 days. Although the patient showed disturbed consciousness (Japan Coma Scale = JCS-I-3) during the period of electrolyte abnormality, he eventually fully recovered without sequelae. In this patient, we successfully managed the severe hypernatremia/hyperchloremia, caused by the combined effects of intravenous saline burden in a state of poor oral fluid intake, during the treatment for IgG-kappa type multiple myeloma.
doi:10.2147/JBM.S44091
PMCID: PMC3660129  PMID: 23700375
hypernatremia; hyperchloremia; multiple myeloma; IgG-kappa; metabolic acidosis; renal tubular damage
23.  Lipid profile of regular blood donors 
Introduction
A few reports have linked regular blood donation to the lowering of parameters of lipid profile. Estimating the lipid profile is an accepted method of assessing an individual’s risk for coronary heart disease, particularly if there is evidence of lipid peroxidation. Regular blood donation may lower iron stores, and this in turn lowers lipid peroxidation. This study was carried out to determine the effect of blood donation on lipid profile.
Materials and methods
Eighty-two participants consented to participate and were enrolled into the study, 52 of whom were regular blood donors (study group) and 30 were non-donors (control group). Venous blood (10 mL) was drawn from each subject into new plain screw-capped disposable plastic tubes. This was allowed to clot and the serum was used to determine total cholesterol, triglycerides, low-density lipoprotein, and high-density lipoprotein.
Results
The mean total cholesterol (4.66 ± 0.86 mmol/L), triglycerides (1.22 ± 0.64 mmol/L), and low-density lipoprotein (2.32 ± 0.73 mmol/L) were significantly lower in the regular blood donors than the control group (5.61 ± 1.26 mmol/L, 1.77 ± 2.9 mmol/L, and 3.06 ± 0.89 mmol/L, respectively; P < 0.05 in all cases). Also, while 42% of the study group had a low/high-density lipoprotein ratio of at least three, about 57% of the control group had a ratio of at least three (P = 0.21).
Conclusion
Regular blood donation may be protective against cardiovascular disease as reflected by significantly lower mean total cholesterol and low-density lipoprotein levels in regular blood donors than in non-donors.
doi:10.2147/JBM.S42211
PMCID: PMC3663474  PMID: 23717053
donor; total cholesterol; low-density lipoprotein
24.  Public banking of umbilical cord blood or storage in a private bank: testing social and ethical policy in northeastern Italy 
Background
In northeastern Italy, according to Italian legislation, authorized public facilities can accept the donation and preservation of cord blood stem cells (CB-SC). Attitudes and knowledge in pregnant women differs between the local and immigrant (non-European Union [EU]) population. In this study we assessed the choices that pregnant women have with respect to the public and private harvesting system and the main reasons driving their decisions. We examined the ethnic origin of the families and compared tests for syphilis screening and leukocyte (WBC) counts in the CB-SC bags that are required for validation of the collection.
Methods
Out of a population of 3450 pregnant patients at the Institute for Maternal and Child Health of Trieste, northeast Italy, 772 women agreed to cord blood harvesting and the associated lab tests. Of these, 221 women (28.6%) were from immigrant families of non-EU countries. Their ethnic affiliation was recorded, and tests were performed for syphilis screening and for nucleated red blood cell (NRBC) interference with the WBC count in CB-SC bags to assess cellularity and to determine if storage was appropriate.
Results
Of the 772 pregnant women, 648 (84.0%) accessed the public collection system, which is free of charge, and 124 (15.0%) accessed the private fee-based system. One woman from the non-EU group opted for the private fee-based system. Of the 3450 pregnant women screened for syphilis at the Institute for Maternal and Child Health, the Treponema pallidum hemagglutination (TPHA) and Venereal Disease Research Laboratory (VDRL) tests were the main tests performed (66.0% of total cases) because many gynecologists in the public harvesting system apply the Italian regulations of the 1988 Decree, while the private system requires tests on syphilis and leaves the option to the lab physicians to select the best determination method. We found that the chemiluminescence method was more specific (97.0%) than the TPHA (83.0%) and nontreponemal rapid plasma reagin VDRL (75.0%) tests (P < 0.05, χ2 test). The specificity link between the two automatic methods versus microscopes for WBC dosing and NRBC interference was r2 = 0.08 (ADVIA 120) and r2 = 0.94 (XE-2100). The public system does not include human T-cell lymphotropic virus testing; this is reserved for the population from endemic zones.
Conclusion
In northeastern Italy current legislation prevents the establishment of private fee-based banks for storage of CB-SC. The cryopreservation, for future autologous personal or family use, is possible only by sending to foreign private banks, with a further fee of €300. These regulations confirm that Italian legislation tries to increase the anonymous allogenic donations and the number of CB-CS bags stored in the free-cost public system, that are available to anyone with therapeutic needs. Private banking is used almost exclusively by the wealthier local population. In the public system, many physicians continue to use older Italian laws regarding syphilis diagnosis, and NRBC interference on WBC count may have an impact on cord blood harvesting. Our findings suggest that in the EU there is no consensus policy on donor management. The value of storage for potential use within the family is useful only with collaboration between the public and the private systems.
doi:10.2147/JBM.S41532
PMCID: PMC3628527  PMID: 23610532
cord blood collection; public system; private system; pregnant women’s choice
25.  Biomarkers for the differentiation of anemia and their clinical usefulness 
The World Health Organization defines anemia as the point at which the amount of hemoglobin in the circulation falls below World Health Organization cutoffs for specific age and sex groups. Anemia is a worldwide problem of complex etiology and is associated with many factors. The purpose of this review was to describe the biomarkers used to identify the nature of anemia in patients and in the community. The important biomarkers are the automated red cell counts, tests for nutritional deficiencies, hemoglobinopathies, and inflammation. Diseases are important potential initiators of anemia, but biomarkers of specific diseases are not included in this review, only the underlying feature common to all disease – namely, inflammation.
doi:10.2147/JBM.S29212
PMCID: PMC3610441  PMID: 23687454
iron deficiency; biological markers; blood cell count; inflammation; avitaminosis; hemoglobinopathies

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