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ABSTRACT While first-line induction therapy for patients with multiple myeloma has changed over the years, autologous hematopoietic stem cell transplantation still plays a significant role, improving both depth of response and progression-free survival of myeloma patients. Our 25-year experience in mobilizing hematopoietic stem and progenitor cells for 472 transplant-eligible myeloma patients was retrospectively reviewed. Patients were stratified according to the remission induction therapy received, and the outcomes were compared among the cohorts that received vincristine, adriamycin and dexamethasone (VAD) (n = 232), bortezomib and dexamethasone (BD) (n = 86), cyclophosphamide, bortezomib and dexamethasone (CyBorD) (n = 82) and other regimens (n = 67). Cyclophosphamide plus granulocyte colony-stimulating factor was the predominant mobilization regimen given. A greater number of CD34+ cells (9.9 × 10E6/kg, p = 0.026) was collected with less hospital admissions in BD patients (13%, p = 0.001), when compared to those receiving VAD (7.5 × 10E6/kg, 29%), CyBorD (7.6 × 10E6/kg, 19%), or other regimens (7.9 × 10E6/kg, 36%). Induction therapy did not influence the overall rate of unscheduled visits or the length of hospitalization because of complications following mobilization. The myeloma response was not significantly deepened following the cyclophosphamide administered for mobilization. This analysis demonstrates the importance of monitoring the impact of initial treatment on downstream procedures such as stem cell mobilization and collection.

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ABSTRACT Introduction: Multiple myeloma (MM) is a common hematologic malignancy with variable degrees of immunodeficiency. Disease- and treatment-related compromise of the immune system predisposes patients to infections, which are a major cause of morbidity and mortality. Objective: We aimed to establish the incidence and main characteristics of infections in MM patients treated at our center over a 10-year period. Method and results: Of the 412 patients retrospectively analyzed, 154 (37.4%) were documented to have at least one episode of infection and were included in this study. A total of 244 infectious episodes were documented. The most common site of infection was the lung, followed by the genitourinary system. The most common infections were bacterial, followed by viral. Escherichia coli were the most common organism. In 160 (65.5%) episodes, the organism was not isolated. Thalidomide with dexamethasone was the most common treatment regimen, followed by melphalan with dexamethasone. Infection was the main cause of death in 26 (6.3%) out of all 412 patients. Conclusion: Infections are a notable cause of morbidity and mortality in the clinical course of MM patients. By considering patient and disease characteristics, a risk-adapted selection of the MM treatment should be employed, with special attention toward patient age and disease-associated organ dysfunction. Patient education, access to healthcare and physician vigilance are also essential. Vaccination and antimicrobial prophylaxis may be considered prior to or during therapy.

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ABSTRACT Background: Despite advances in health care for sickle cell disease patients, as well as in the improvement in reproductive issues mainly in women with the disease, pregnancy is still a challenge, both for the mother and the child, with high rates of maternal and fetal morbidity and mortality. Besides their chronic hemolytic status and vaso-occlusive events that confer systemic complications, pregnant women also have higher rates of pain episodes, infections, abortion, intrauterine growth retardation, pre-term births, eclampsia, stillbirth and the hemolysis, elevated liver enzymes and low platelets syndrome. The physiologic mechanisms of the disease in pregnancy are still unknown and chronic inflammatory responses may interfere in the adverse outcomes. The cytokine and chemokine profiles in pregnancy with sickle cell disease remain unknown. The aim of this study was to evaluate the cytokine profile of the inflammatory response of pregnant women with sickle cell disease. Method: Blood samples from 20 pregnant women with sickle cell disease, 24 women with sickle cell disease in steady state, 16 healthy pregnant women and a control group with 9 women at childbearing age were assayed for interleukin-6. Main results: Pregnant women with sickle cell disease presented high serum levels of interleukin-6, compared to healthy pregnant women (p = 0.0115). Conclusion: These data suggest that the increased production of interleukin-6 may occur during pregnancy with sickle cell disease and that the role of this cytokine in the sickle cell disease pathophysiology and pregnancy complications should be further studied.

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ABSTRACT Introduction: Although there is a vast literature regarding the association between inherited thrombophilia, obstetric complications and the effect of low molecular weight heparin (LMWH), these are controversial and we have not found publications related to additional risk factors other than thrombophilia.Our objectives were to assess the prevalence of miscarriage, placenta-mediated pregnancy complications and fetal loss in pregnant women with IT, establishing associated risk factors and the effect of LMWH. Materials and methods: A retrospective cohort of pregnant women with IT was formed. Risk factors considered were: high-risk IT, age ≥35 years, body mass index ≥25 and ≥30, assisted reproductive technology, antiphospholipid antibodies, autoimmune disease, first-degree family history of obstetric complications and personal history of venous or arterial thromboembolic disease, the outcomes being M, FL and PMPC. Results and conclusions: Data from 250 pregnancies in 88 women were obtained.There were 112 (45%) Ms, 13 (5.2%) FLs and 25 (10%) PMPCs.High-risk IT was associated with FL (OR = 4.96; 95% CI, 1.42-17.3). Antiphospholipid antibodies and family history of obstetric complications were associated with PMPC (OR = 7.12; 95% CI, 1.89-26.74, OR = 3.88; 95% CI, 1.18-12.78, respectively). The LMWH presented a benefit in the combined outcome (any obstetric complication, OR = 0.25; 95% CI, 0.12-0.54) and M (OR = 0.41; 95% CI, 0.20-0.82).We conclude that obstetric complications are common in women with IT. Antiphospholipid antibodies, family history of obstetric complications and high-risk IT might be additional risk factors. The LMWH has an apparent protective effect against obstetric complications, which is consistent with some previous studies.

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ABSTRACT Background: Assessing trends in the rate of transfusion-transmissible infections (TTIs) in blood donors is critical to the monitoring of the blood supply safety and the donor screening effectiveness. The objective of this study was to conduct a trend analysis of TTIs and associated demographic factors of donors at a public blood bank in the central Brazil. Methods: A retrospective analysis (2010-2016) of blood donation data was performed to determine the prevalence of markers for TTIs. Multinomial and multivariate logistic regression were used to verify the association between the explanatory variables and TTIs. The trend was evaluated with the Prais Winsten's regression analysis. Results: The prevalence of TTIs was 4.04% (5,553 donors) among 137,209 donors, with a steady trend in the analyzed period. The seroprevalence for the hepatitis B virus (HBV), syphilis, hepatitis C virus (HCV), human immunodeficiency virus (HIV), Chagas disease, and human T-lymphotropic virus (HTLV) were 1.63%, 0.87%, 0.46%, 0.21%, 0.21% and 0.09%, respectively. The prevalence of HBV decreased (b = −0.021, p < 0.001), while syphilis increased (b = 0.112; p = 0.001), during the period investigated. The probability for a positive test for TTI was higher among donors with a low level of education, aged ≥30 years old, without stable marital status, and first-time donors. Conclusions: Even with the reduction in HBV, the increased rate of syphilis may have contributed to the fact that the overall rate of TTIs did not decrease in the evaluated period.

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ABSTRACT Background: Blood transfusion-transmitted infections in individuals suffering from beta-thalassemia have been reported in Pakistan, but the information on their sociodemographic and clinical determinants is lacking. This study aims to describe the prevalence, as well as the factors, contributing in blood transfusion-transmitted infections. Method: Between December 2011 and December 2013, in a non-probable sampling, 350 thalassemia patients were recruited in Lahore, Multan, Karachi and Peshawar, Pakistan. Subjects were screened for transfusion-transmitted infections. Results: A seropositive rate of 36.5% was observed; males (94, 73.4%) and females (34, 26.6%). Among several risk factors associated with transfusion-transmitted infections, province (p = 0.001), gender (p = 0.003), age (p < 0.03), education (p < 0.00), degree of consanguinity (p = 0.05), age at fetal blood test (p = 0.005), fetal hemoglobin levels (p = 0.005), death due to thalassemia (p = 0.001) and iron-related complications (p = 0.04) showed significant correlation. Participants with an age >10 years were significantly more prone to seropositivity than those aged ≤10 years. Moreover, the ferritin level was also significantly higher in those aged >10 years than in those ≤10 years. It was observed that males had a higher seroprevalence rate (94, 73.4%) than females (34, 26.6%). The most prevalent transfusion-transmitted infections was the hepatitis C virus, with 115 cases (89.8%). Conclusion: A high prevalence rate of HCV in subjects with transfusion-dependent thalassemia is linked with insufficient facilities, poor management and compromised socioeconomic status. Therefore, more multicenter studies covering cities from different regions of the country are needed in order to develop preventive measurements at the regional and national level.

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ABSTRACT Introduction: The serological screening of blood donors has been instituted by the Brazilian Ministry of Health and is mandatory in the research on several diseases transmissible through blood transfusion. Blood banks need to establish a screening service capable of reducing associated transfusions risks. Objective: The objective of this study was to establish the prevalence of transfusion-transmissible infection markers in donors at a hemotherapy service located in southwest Bahia, Brazil. Methods: A retrospective study was performed between 2010 and 2016. Variables, such as the characterization of donors who were suitable and unsuitable for donation (in clinical screening), stratification by gender and age group and unsuitable samples for reactive test results (in serological screening) by aspecific antibodies against hepatitis B virus (anti-HBc and HBsAg), hepatitis C virus (anti-HCV), human T-lymphotropic virus (anti-HTLV I/II), HIV virus (anti-HIV I/II), chagas disease, and syphilis markers, were evaluated. Results: Collected data showed that 3.13% of the donors were considered unsuitable for serological screening and that the prevalence of reactive test results was higher in donors aged between 30 and 39 years and in males. The means of the serological markers was 1.09% for syphilis reagents, 0.63% for anti-HIV I/II, 0.51% for anti-HBc and anti-HCV, 0.15% for HBsAg, 0.14% for HTLV I/II and 0.10% for Chagas disease. Conclusion: These results reflect the importance of awareness campaigns on sexually transmitted diseases and transfusion safety measures taken by hemotherapy services.

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ABSTRACT Introduction: The incidence of grade 3-4 anemia was reported to be 3% with imatinib therapy for newly diagnosed chronic myeloid leukemia (CML) in the chronic phase (CP). However, there are few data regarding the causes and the development of anemia after long-term treatment. This study aimed to evaluate the incidence of anemia after at least two years of imatinib treatment of CML patients in the CP and to identify other contributing causes of anemia in this population. Patients and methods: We performed a retrospective analysis of 97 CML patients in the CP treated with imatinib for at least two years. We analyzed the hemoglobin (Hb) levels of CML patients at diagnosis, upon initiation of treatment with imatinib and after two years of imatinib treatment, and investigated other causes of anemia in this population. Results: Most of the patients presented Hb levels below the normal range (80.4%) after the second year of treatment, 17.9% grade 2 and 1.3% grade 3. In 13 cases (16.7%), anemia was attributed to resistance and in 13 cases (16.7%) the following causes were identified: iron deficiency (n = 5), hypothyroidism (n = 2), vitamin B12 deficiency (n = 3), acquired immune deficiency syndrome (AIDS) (n = 1), pulmonary tuberculosis (n = 1) and renal toxicity (n = 1). In 52 patients (66.6%), there were no other factors contributing to anemia, except imatinib treatment. Conclusion: Regular follow-up is required to identify the causes of anemia not related to CML or imatinib toxicity. The importance of investigating secondary causes of anemia should be emphasized, especially in patients with good adherence to treatment and satisfactory therapeutic response.

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ABSTRACT Introduction: Hemophilia is a coagulopathy caused by a deficiency in coagulation factors VIII (hemophilia A) or IX (hemophilia B). It is a chronic disease and, hence, impairs the quality of life (Qol) of the patients. This study aimed to evaluate the Qol of patients with hemophilia using the WHOQOL-bref and the Haemo-A-Qol instruments, its relation to the clinical condition and its sociodemographic variables. Methods: This is a cross-sectional, epidemiological study, comprising 17 patients with hemophilia, registered at the hemocenter, who met the eligibility criteria. Data were collected using three questionnaires: a semi-structured clinical evaluation form, the WHOQOL-bref and the Haem-A-Qol. Results: The average age was 30 years old, and most participants declared themselves to be single (58.82%), without children (64.70%) and employed (58.82%). Hemophilia A was observed in 14 patients and the most severe form of the disease was more prevalent (64.70%). The average score of Qol, estimated by the WHOQOL-bref questionnaire was 74.3; being "social relations" the domain with the highest average. The Haem-A-Qol measured an average of 36.2 and the domain with the highest result was "Family Planning". Conclusion: Hemophilia had a higher negative impact upon the physical, sports and leisure features in the sample subjects. The analysis of the questionnaires did not reveal statistical agreement between them. Based on this, the Haem-A-Qol is considered the most recommended to evaluate the Qol, as it addresses factors more specifically related to the disease. No statistical significance was observed between the scores of Qol, as for the presence of comorbidities, gravity of the hemophilia and positive serology for infections.

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ABSTRACT Background: Recently, a small peptide called Hepcidin, was found to have an important role in regulating the iron metabolism in anemia of chronic disease (ACD) patients. Hepcidin is regulated by a variety of conditions at the transcriptional level. Therefore, our study aims to predict the level of hepcidin serum using inflammation markers and iron indicators in patients afflicted with ACD and observe how this severity of inflammation separated the level of interleukin-6 (IL-6), as well the as hepcidin level. Methods: A cross-sectional data analysis was conducted on 80 ACD adult patients treated at the Sanglah Teaching Hospital in Bali, Indonesia. We used hepcidin serum and several markers, such as the hemoglobin level, inflammation markers, renal function tests, IL-6, and iron indicators, to predict the hepcidin level. Results: This study recruited 80 ACD patients, comprising 45 men (56.3%) and 35 women (43.7%). The mean age of the participants was 43 ± 16.5 years. Only IL-6, ferritin and serum creatinine correlate significantly with serum hepcidin from seven variables that were previously eligible to enter the analysis. This study found the model to predict the hepcidin level using IL-6 ferritin and the creatinine level as the hepcidin level (predicted) = −23.76 + 0.396 (IL 6) + 0.448 (ferritin) + 0.310 (creatinine). Conclusion: This study has revealed that the creatinine level, ferritin and IL-6 can be used to predict the hepcidin level in patients with anemia of chronic disease. It is to be hoped that further cohort studies can validate our formula to predict the hepcidin level.

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ABSTRACT Introduction: Hemophilia is well known in males, but poorly recognized in hemophilia carriers, who may have a hemorrhagic tendency, and the symptoms may be frequent and severe. Few studies have been done evidencing this bleeding in female carriers of the hemophilia gene. Objectives: To verify the prevalence of hemorrhagic symptoms in HC, compared to women in the general population. Material and method: The articles published between October 1996 and November 2016 were searched in the PubMed, Scielo, Lilacs, Web of Science, Scopus and Cochrane Central databases. Results and discussion: Seventy-five articles were found in electronic databases and 2 additional articles, through manual search in journal summaries and bibliographical references of other review articles. There is a limitation as to the number of studies that explore the association between the risk of hemorrhagic events and HC A or B. Among the few existing studies, there is a methodological difference, evidenced by control groups with distinct recruitments, divergent questionnaires and non-standardized concepts. Conclusion: This review verified the existence of a higher prevalence of hemorrhagic symptoms in the HC in some outcomes, however, due to the limitations of the few studies found, there is still insufficient evidence to state that the HC has a greater hemorrhagic tendency in relation to the general population.

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ABSTRACT Introduction: The myelodysplastic syndromes (MDS) are a group of heterogeneous clonal hematopoietic stem cell disorders that results in peripheral blood (PB) cytopenias and bone marrow (BM) dysplasia. Dysplasia is the hallmark of the disorder, and must exceed the threshold of 10%. Conventional karyotype (KT) has a role in the classification and prognostication of subtypes. In daily practice, many cases are diagnosed in face of exuberant clinical complains, but cases with dismal evidences pose real difficulties to definitively conclude the case. Material and methods: The objective of this study is to detect cases in which no morphology evidence of dysplasia or increased blasts were observed but KT was decisive for MDS diagnosis. 666 cases were admitted to rule out MDS. Results: There were found 5 (0.75%) cases who presented no evident dysplasia morphology or whose dysplasia was borderline but the karyotype was decisive because showed clonal evidence. The karyotype was: case 1: 46,XY,del(5q)(q13q33),del(11)(q13q23)[7]/46,XY[13]; case 2: 46,XX,del(11)(q21q23)[20]; case 3: 46,XX,del(7)(q22q34)[4]/46,XX[8]; case 4: 47,XX,del(5)(q13q33),+mar[12]/46,XX[8] and case 5: 46,XXt(2;11)(p21;q24),del(4)(?q25),del(21)(q22)[14]/46,XX[6]. Conclusion: Patients with cytopenia and insufficient or borderline evidence of dysplasia may experience a long journey before a MDS diagnosis is made. Cytogenetics studies may abbreviate this pathway when clonal aberrations considered presumptive of MDS are detected. This study shows that karyotype should still be considered as a diagnostic tool.