Direct oral anticoagulants in patients affected by major congenital thrombophilia
Conclusions: No patient presented hemorrhagic or thrombotic complications during DOAC therapy. DOACs have proven to be effective and safe in this real-life series of patients with major thrombophilia.    (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - June 25, 2019 Category: Hematology Source Type: research

Therapeutic targeting of notch signaling pathway in hematological malignancies
The Notch pathway plays a key role in several processes including stem-cell self-renewal, proliferation, and cell differentiation. Several studies identified recurrent mutations in hematological malignancies making Notch one of the most desirable target in leukemia and lymphoma. The Notch signaling mediates resistance to therapy and controls cancer stem cells supporting the development of on-target therapeutic strategies to improve patients ’ outcome. In this brief review, we outline the therapeutic potential of targeting Notch pathway in T-cell acute lymphoblastic leukemia, chronic lymphocytic leukemia and mantle ce...
Source: Mediterranean Journal of Hematology and Infectious Diseases - June 25, 2019 Category: Hematology Source Type: research

Sickle cell disease and infections in high- and low-income countries
Infections, especially pneumococcal septicemia, meningitis, andSalmonella osteomyelitis, are a major cause of morbidity and mortality in patients with sickle cell disease (SCD). SCD increased susceptibility to infection, while infection leads to SCD-specific pathophysiological changes. The risk of infectious complications is highest in children with a palpable spleen before 6 months of age. Functional splenectomy, the results of repeated splenic infarctions, appears to be an important host-defense defect. Infection is the leading cause of death, particularly in less developed countries. Defective host-defense mechanisms en...
Source: Mediterranean Journal of Hematology and Infectious Diseases - June 25, 2019 Category: Hematology Source Type: research

Azacytidine failure revisited: an appraisal based on real life data from the MDS registry of the Hellenic Myelodysplastic Syndrome Study Group (HMDS).
N/A (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - June 25, 2019 Category: Hematology Source Type: research

Prevalence of blatem, blashv, and blactx-m genes among esbl-producing klebsiella pneumoniae and escherichia coli isolated from thalassemia in iraq
Due to the recent appearance of organisms that are resistant to several drugs (multidrug-resistant) like Enterobacteriaceae that produce extended-spectrum β-lactamase (ESBL, concerns have remarkably increased regarding the suitable treatment of infections. The present study was an investigation into ESBL molecular characteristics among clinical isolates ofKlebsiella pneumoniae andEscherichia coli resulting in UTIs and their pattern of antimicrobial resistance in order to come up with helpful information on the epidemiology of these infections and risk factors accompanied with them. In order to conduct the study, 20K. ...
Source: Mediterranean Journal of Hematology and Infectious Diseases - June 25, 2019 Category: Hematology Source Type: research

Economic evaluation of chelation regimens for β--thalassemia major: a systematic review
Conclusion:In brief, DFP is the best choice, followed by DFO or DFX, when an iron chelator is to be used alone for β-thalassemia major therapy. All studies that compared DFO and DFP combination therapy with DFO (or DFP or DFX) monotherapy established that the combination therapy with DFO and DFP was not cost-effective. However, due to the low number of related studies, more extensive, high-quality research is r equired for further analysis and confirmation of our findings. Moreover, the cost effectiveness is not an absolute issue when in different countries(regions) the results are opposite for other countries(regions...
Source: Mediterranean Journal of Hematology and Infectious Diseases - June 24, 2019 Category: Hematology Source Type: research

Malnutrition, sepsis and tumor lysis syndrome are associated with increased rate of acute mortality in mature b cell non-hodgkin lymphoma in pediatric population- study from tertiary care hospital in pakistan
Conclusion:Rate of acute mortality in B cell NHL is high in our set up and significant riskfactors are tumor lysis syndrome, sepsis and malnourishment at time of presentation. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - June 24, 2019 Category: Hematology Source Type: research

Molecular analysis of non-transfusion dependent thalassemia associated with hemoglobin e- β-thalassemia disease without α--thalassemia
Conclusion: It was found that these SNPs when analyzed in combination could explain the mild phenotypic expression of all cases. These results underline the importance of these informative SNPs on phenotypic expression of Hb E- β-thalassemia patients. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - June 24, 2019 Category: Hematology Source Type: research

Homozygous deletion alfa-thalassemia and hereditary persistence of fetal hemoglobin, two genetic factors predictive the reduction of morbidity and mortality during pregnancy in sickle cell patients . a report from democratic republic of congo
FHb and alpha-thal are two genetic factors that modulate the clinical expression of sickle cell disease.Objective: to determine the beneficial role of FHb and alpha-thal on fetal and maternal morbidity during pregnancy in sickle cell patients.This is a documentary and analytical study that included 960 deliveries of homozygous sickle cell patients. The deliveries were divided into three genotype subgroups: Hb-SS / alpha-thal, HbSS / HPFH and HbSS. The diagnosis of SCD and the quantification of FHb were performed by the capillary electrophoresis technique. The diagnosis of SCD has been confirmed by the molecular test. The d...
Source: Mediterranean Journal of Hematology and Infectious Diseases - June 24, 2019 Category: Hematology Source Type: research

Nucleotide substitutions in hepatitis b viruses derived from chronic hbv patients
Conclusions: Our results showed common mutations within HBsAg, occurring in immune epitopes, a high rate of G1896A mutations in the PC region, and a negative correlation between the emergence of A1762T/G1764A mutation and the G1764T/C1766G mutant in the BCP region.  (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - June 24, 2019 Category: Hematology Source Type: research

Viral load and nucleotide substitutions in hepatitis b viruses derived from chronic hbv patients
Conclusions: Our results showed common mutations within HBsAg, occurring in immune epitopes, a high rate of G1896A mutations in the PC region, and a negative correlation between the emergence of A1762T/G1764A mutation and the G1764T/C1766G mutant in the BCP region.  (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - June 24, 2019 Category: Hematology Source Type: research

Sickle cell disease and pregnancy
Abstract Sickle Cell Disease (SCD) is a group of inherited single-gene autosomal recessive disorders caused by the ‘sickle’ gene, which affects haemoglobin structure. Sickle cell anemia is the most common hemoglobinopathy worldwide. The burden of sickle cell disease in pregnancy has been exponentially increasing with more number of women reaching the reproductive age, and having successful pregnancies. It ha s been proven beyond doubt that SCD in pregnancy poses the pregnant woman and fetus to significantly higher risks than a lady without SCD. SCD is associated with both maternal and fetal complications a...
Source: Mediterranean Journal of Hematology and Infectious Diseases - June 24, 2019 Category: Hematology Source Type: research

A real-world study on Clofarabine and Cytarabine combination in patients with relapsed/refractory acute myeloid leukemia
NA (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - April 29, 2019 Category: Hematology Source Type: research

West Nile virus encephalitis in haematological setting: report of two cases and brief review of the literature
We report on two cases of severe West Nile encephalitis in recently-treated, different-aged, chronic lymphocytic leukemia patients. Both patients developed high-grade fever associated with severe neurological impairment. The younger one harboured germ-line CCR5 delta-32 mutation, which might have played a role in the pathogenesis of its neuroinvasive manifestations. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - April 27, 2019 Category: Hematology Source Type: research

Seroprevalence of Toxocara canis in the city of Catania, Italy.
Toxocariasis is one of the most common helminthiases worldwide. However, there is a lack of data regarding Southern Italy. We have evaluated the seroprevalence and associated environmental factors of toxocariasis in a sample of adults living in the city of Catania.Presence of anti-Toxocara canis IgG antibodies was searched using an ELISA test using excretory/secretory antigens. Environmental risk factors have beene valuated with a face-to-face questionnaire.Two hundred eighty-seven subjects (mean age of 48.1 ±15.6 years) were enrolled, and presence of antiT. canis antibodies was found in 23 participants, of whom 18 ...
Source: Mediterranean Journal of Hematology and Infectious Diseases - April 27, 2019 Category: Hematology Source Type: research

Direct acting antiviral treatment for patients with end stage kidney disease with acute hcv infection
Conclusions: sofosbuvir containing regimens were effective and safe for the treatment of acute HCV in patients with ESKD who were on haemodialysis. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - April 27, 2019 Category: Hematology Source Type: research

Clinical presentation, laboratory analysis and linear growth in 50 neonates and young infants with acute meningitis: one year experience of a single center in qatar
The objective of this study is to describe the clinical presentation of neonates and young infants with acute meningitis with different etiologies andto determine the clinical impact ofthe effect of acute meningitison growth parameters.Material and methods:We analyzed the clinical data and the growth parameters of 50 newborns and young infants (age: 1.6 ± 0.9 month) admitted to our hospital (Al Wakhra Hospital, Department of Pediatrics, Doha ,Qatar), between 1-1-2016 to 1-1-2017, with acute meningitis. Anthropometric measurements included weight, length, and head circumference. Length SDS (L-SDS) and body mass-index...
Source: Mediterranean Journal of Hematology and Infectious Diseases - April 26, 2019 Category: Hematology Source Type: research

Treatment patterns in patients with chronic-phase chronic myeloid leukaemia in routine clinical practice: the simplicity italian population
ConclusionThis analysis provides valuable insights into management and treatment patterns in Italian patients with CML within routine clinical practice. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - April 26, 2019 Category: Hematology Source Type: research

Guest Editor: Raffaella Origa IRON TOXICITY AND HEMOPOIETIC CELL TRANSPLANTATION: TIME TO CHANGE THE PARADIGM.
The issue of iron overload in hemopoietic cell transplantation has been first discussed in the field of transplantation for thalassemia. Thalassemia major is characterized by ineffective erythropoiesis and hemolysis leading to anemia in the majority of patients. Patients require regular blood transfusion therefore they develop iron overload causing organ damage and hematopoietic cell transplantation (HCT) is a consolidated reliably curative option.In this category of patients an important issue for transplant outcome is the iron burden before transplant and in the long-life post-transplant. Nevertheless, today the concept ...
Source: Mediterranean Journal of Hematology and Infectious Diseases - April 25, 2019 Category: Hematology Source Type: research

Iron toxicity and hemopoietic cell transplantation: time to change the paradigm.
The issue of iron overload in hemopoietic cell transplantation has been first discussed in the field of transplantation for thalassemia. Thalassemia major is characterized by ineffective erythropoiesis and hemolysis leading to anemia in the majority of patients. Patients require regular blood transfusion therefore they develop iron overload causing organ damage and hematopoietic cell transplantation (HCT) is a consolidated reliably curative option.In this category of patients an important issue for transplant outcome is the iron burden before transplant and in the long-life post-transplant. Nevertheless, today the concept ...
Source: Mediterranean Journal of Hematology and Infectious Diseases - April 25, 2019 Category: Hematology Source Type: research

Langerans cell histiocytosis: single center experience of 25 years
Objectives: To review a single center outcome of patients with Langerhans Cell Histiocytosis diagnosed at a referral tertiary hospital from Turkey.Methods: The files between 1989 and 2014 of 80 patients with LCH were retrospectively analyzed.Results: During the 25 year period, 80 patients were diagnosed with LCH. The median age at diagnosis was 53 months (2-180 months) and median follow-up time of patients was 10 years and 9 months (24 months-25 years). Bone was the most frequently affected organ (n:60, 75%). Initially, 43 patients (54%) had single system (SS) disease, 20 patients (25%) had multisystem (MS) disease without...
Source: Mediterranean Journal of Hematology and Infectious Diseases - April 25, 2019 Category: Hematology Source Type: research

Guest Editor: Raffaella Origa THYROID DISORDERS IN HOMOZYGOUS β-THALASSEMIA: CURRENT KNOWLEDGE, EMERGING ISSUES AND OPEN PROBLEMS
Abstract.Changes in thyroid function and thyroid function tests occur in patients with β-thalassemia major (TM). The frequency of hypothyroidism in TM patients ranges from 4% to 29 % in different reports. The wide variation has been attributed to several factors such as patients'genotype, age, ethnic variations, treatment protocols of transfusions and chelation, and varying compliance to treatment. Hypothyroidism is the result of primary gland failure or insufficient thy ­roid gland stimulation by the hypothalamus or pituitary gland. The main laboratory parameters of thyroid function are the assessments of serum t...
Source: Mediterranean Journal of Hematology and Infectious Diseases - April 25, 2019 Category: Hematology Source Type: research

Thyroid disorders in homozygous β-thalassemia: current knowledge, emerging issues and open problems
Abstract.Changes in thyroid function and thyroid function tests occur in patients with β-thalassemia major (TM). The frequency of hypothyroidism in TM patients ranges from 4% to 29 % in different reports. The wide variation has been attributed to several factors such as patients'genotype, age, ethnic variations, treatment protocols of transfusions and chelation, and varying compliance to treatment. Hypothyroidism is the result of primary gland failure or insufficient thy ­roid gland stimulation by the hypothalamus or pituitary gland. The main laboratory parameters of thyroid function are the assessments of serum t...
Source: Mediterranean Journal of Hematology and Infectious Diseases - April 25, 2019 Category: Hematology Source Type: research

Final height and endorine complications in patients with β-thalasssemia intermedia: our experience in non-transfused versus infrequently transfused patients and correlations with liver iron content
Conclusions: A significant number of TI patients have high LIC, short stature and endocrine disorders. Patients who require occasional transfusions have more liver iron overload and higher hepatic dysfunction. Females appear to attain better final adult height and have higher IGF1- SDS versus males. Our data emphasize the need for long term surveillance for identification of organ-specific risk factors and early disease manifestations.We also recommend a close monitoring of endocrine and other complications, according to the international guidelines. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - April 25, 2019 Category: Hematology Source Type: research

Familial mediterranean fever: assessing the overall clinical impact and formulating treatment plans
Recurrent self-limited attacks of fever and short-lived inflammation in the serosal membranes, joints and skin are the leading features of familial Mediterranean fever (FMF), the most common autoinflammatory disorder in the world, transmitted as autosomal recessive trait caused by MEFV gene mutations. Their consequence is an abnormal function of pyrin, a natural repressor of inflammation, apoptosis and release of cytokines. FMF-related mutant pyrins are hypophosphorylated following RhoA GTPases ’ impaired activity and show a propensity to relapsing uncontrolled systemic inflammation with inappropriate response t...
Source: Mediterranean Journal of Hematology and Infectious Diseases - April 25, 2019 Category: Hematology Source Type: research

Comparison of the power of procalcitonin and c-reactive protein to discriminate between different aetiologies of fever in prolonged profound neutropenia: a single-centre prospective observational study.
Management of fever in prolonged profound neutropenia remains challenging with many possible infectious and non-infectious causes. We investigated whether procalcitonin (PCT) is superior to C-reactive protein (CRP) in discriminating between different etiologies of fever in this setting.CRP and PCT were tested daily during 93 neutropenic episodes in 66 patients. During this study period 121 febrile episodes occurred and were classified into four categories based on clinical and microbiological findings: microbiologically documented infection (MDI); clinically documented infection (CDI); proven or probable invasive fungal di...
Source: Mediterranean Journal of Hematology and Infectious Diseases - February 26, 2019 Category: Hematology Source Type: research

Survival probability in patients with sickle cell anemia using the competitive risk statistical model
The objective of this study is to analyze the causes of death and the effect of sex and age on survival of Brazilian patients with SCA. Data of patients with SCA who were seen and followed at HEMORIO for 15 years were retrospectively collected and analyzed. Statistical modeling was performed using survival analysis in the presence of competing risks estimating the covariate effects on a sub distribution hazard function. Eight models were implemented, one for each cause of death. The cause ‐specific cumulative incidence function was also estimated. Males were most vulnerable for death from chronic organ damage (p = 0.0005...
Source: Mediterranean Journal of Hematology and Infectious Diseases - February 26, 2019 Category: Hematology Source Type: research

H1n1 infection in pregnancy; a retrospective study of feto-maternal outcome and impact of the timing of antiviral therapy
Conclusions:H1N1 influenza A infection in pregnancy is associated with adverse maternal and perinatal outcomes. Medical and public awareness, low threshold for testing suspected pregnant patients, very early initiation of antiviral therapy, and multidisciplinary approach in our series decreased the overall adverse effects of this infection.  (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - February 26, 2019 Category: Hematology Source Type: research

Pregnancy in thalassemia
Therapeutic advances, including the availability of oral iron chelators and new non-invasive methods for early detection and treatment of iron overload, have significantly improved the life expectancy and quality of life of patients with thalassemia, with a consequent increase in their reproductive potential and desire to have children. Hundreds of pregnancies have been reported so far, highlighting that women carefully managed during preconception usually carry out a successful gestation and labour, both in case of spontaneous conception and use of assisted reproductive techniques. A multidisciplinary team including a car...
Source: Mediterranean Journal of Hematology and Infectious Diseases - February 26, 2019 Category: Hematology Source Type: research

Differences in ex-vivo chemosensitivity to anthracyclines in first line acute myeloid leukemia
DISCUSSION:A third of the patients could benefit of the use of this test in the first line induction therapy selection, although it should be confirmed in a clinical trial specifically designed. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - February 26, 2019 Category: Hematology Source Type: research

Novel deleterious sequence change in the NLRP12 gene in a child with autoinflammatory syndrome, joint hypermobility and cutis laxa from India.
An otherwise healthy male child of 9 years presented with paroxysmal fever and diffuse abdominal pain along with loss of appetite and nausea lasting for 3-4days every 4-6 weeks for last 2 years. He also has stretchable skin and hypermobile joint which he inherited from his mother who never suffered any paroxysmal attack of the kind.  Work up for acute intermittent porphyria, lead poisoning and familial mediterranean fever was negative. A novel harmful sequence change in NLRP12 gene was detected and a diagnosis of NLRP12 associated autoinflammatory syndrome was made. This sequence change with disease has not yet been r...
Source: Mediterranean Journal of Hematology and Infectious Diseases - February 26, 2019 Category: Hematology Source Type: research

Spectrum and immunophenotypic profile of acute leukemia: a tertiary center flow cytometry experience
Conclusion:In this study we document the spectrum; correlate the immunophenotype with genetic data of all leukemias, especially with respect to T-ALL where the data from India is scarce. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - February 26, 2019 Category: Hematology Source Type: research

Prognostic impact of immunohistochemical p53 expression in bone marrow biopsy in higher risk mds: a pilot study
Conclusions:The ICH p53 expression in bone marrow biopsy in higher risk MDS was confirmed to have prognostic value.  These results indicate more than 10% expression as the best cut off value. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - February 26, 2019 Category: Hematology Source Type: research

Can base excess be used for predict to neonatal sepsis in preterm newborns?
In this study a total of 118 infants were enrolled. The infants were classified into two groups: group 1 (sepsis, n=49) and group 2 (control, n=69). Blood gas analysis investigated for screening of neonatal sepsis.Results: A total of 49 newborns with neonatal sepsis and 69 healthy controls were enrolled. A comparison of markers of sepsis revealed C-reactive protein, interleukin-6 level to be significantly higher and pH, pCO2, HCO3 and base excess values to be significantly lower in newborns with sepsis compared healthy controls (p
Source: Mediterranean Journal of Hematology and Infectious Diseases - February 25, 2019 Category: Hematology Source Type: research

Cord blood platelet lysate: in vitro evaluation to support the use in regenerative medicine.
In this study, we compared cytokine profile and mesenchymal cell growth supporting the ability of platelet lysate obtained from adult and cord blood. Ourin vitro results strongly back the utilization of CB platelet lysatein vivo, as an efficacious, safe and inexpensive alternative to promote damaged tissue healing when the autologous PRP is contraindicated. Moreover, the policy of manufacturing CB platelet lysate can limit the current disposal of many collected CB units not suitable for transplant due to their low nucleated cell count.   (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - February 25, 2019 Category: Hematology Source Type: research

Relapsed/refractory chronic lymphocytic leukemia: chemoimmunotherapy, treatment until progression with mechanism-driven agents or finite-duration therapy?
Treatment of relapsed/refractory (R/R) chronic lymphocytic leukemia (CLL) has dramatically improved thanks to the development of mechanism-driven agents including drugs that inhibit kinases in the BCR pathway or BCL2. The treating physician has now the opportunity to decide i) which patient can be still offered chemoimmunotherapy as salvage treatment, ii) which patient is a candidate to receiving at relapse continuous treatment with ibrutinib, idelalisib and rituximab or venetoclax and iii) which patient may benefit from a fixed-duration treatment using the BCL2 antagonist venetoclax in association with rituximab.Ibrutinib...
Source: Mediterranean Journal of Hematology and Infectious Diseases - January 1, 2019 Category: Hematology Source Type: research

New therapeutic options for the treatment of sickle cell disease
Sickle cell disease (SCD; ORPHA232; OMIM # 603903) is a chronic and invalidating disorder distributed worldwide, with high morbidity and mortality.  Given the disease complexity and the multiplicity of pathophysiological targets, development of new therapeutic options is critical, despite the positive effects of hydroxyurea (HU), for many years the only approved drug for SCD.New therapeutic strategies might be divided into (1) pathophysiology-related novel therapies and (2) innovations in curative therapeutic options such as hematopoietic stem cell transplantation and gene therapy. The pathophysiology related novel th...
Source: Mediterranean Journal of Hematology and Infectious Diseases - January 1, 2019 Category: Hematology Source Type: research

Chronic kidney disease amongst sickle cell anaemia patient at the university of maiduguri teaching hospital, north eastern nigeria: a study of prevalence and risk factors
ConclusionThe present study has revealed a high prevalence of CKD amongst patients with SCA in this region. Various clinical and laboratory predictors of eGFR were also identified. Monitoring and detection of early stages of these groups of patients may allow for interventions which may delay progression into advance stages and ESRD. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - January 1, 2019 Category: Hematology Source Type: research

High prevalence of Hepatitis C Virus associated B-cell lymphoma in Mansoura Region (Egypt), ANRS 12263 study
In conclusion:  B-cell lymphomas are highly associated with HCV infection in Egypt. Further developments are needed to give access to antiviral treatment for those patients in Delta region.  (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - January 1, 2019 Category: Hematology Source Type: research

Increase in Candida parapsilosis candidemia in cancer patients
This study aimed to identify the risk factors of candidemia and asses possible clinically significant differences betweenCandida parapsilosis and other candida species among cancer patients.Methods: A retrospective study was conducted in a Chinese tertiary cancer center over a 6-year period. A total of 323 cancer patients were enrolled and analyzed from 2012 to 2018. Data about demographic characteristics, underlying diseases, and risk factors of candidemia were collected. Univariate and multivariate logistic regression models were used to identify the risk factors associated with the development of candidemia.Results: Amo...
Source: Mediterranean Journal of Hematology and Infectious Diseases - January 1, 2019 Category: Hematology Source Type: research

Soluble st2 and cd163 as potentialbiomarkers to differentiate primary hemophagocytic lymphohistiocytosis from macrophage activation syndrome
Conclusion: Patients with pHLH and MAS show some differences in cytokine profiles. The elevated levels of IFN- γ, IL-10, IL-18 and TNF-α can contribute to the diagnosis of HLH, but may not discriminate pHLH from MAS. Levels of sST2 and sCD163 may serve as markers to distinguish pHLH from MAS. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - January 1, 2019 Category: Hematology Source Type: research

Soluble ST2 and CD163 as Potential Biomarkers to Differentiate Primary Hemophagocytic Lymphohistiocytosis from Macrophage Activation Syndrome
Conclusion: Patients with pHLH and MAS show some differences in cytokine profiles. The elevated levels of IFN- γ, IL-10, IL-18 and TNF-α can contribute to the diagnosis of HLH, but may not discriminate pHLH from MAS. Levels of sST2 and sCD163 may serve as markers to distinguish pHLH from MAS. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - January 1, 2019 Category: Hematology Source Type: research

Bone mineral density and vitamin d receptor genetic variants in egyptian children with beta thalassemia on vitamin d supplementation
This study was conducted on forty children with βTM and forty unrelated healthy sex and age-matched controls. Serum calcium, phosphorus, ALP, ferritin and vitamin D were measured. VDR genetic variants (BsmI,TaqI, andFokI) were genotyped by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). BMD was measured by dual-energy X-ray densitometry (DEXA) of the lumbar spine.Results: In βTM patients, 22.5% had deficient, 50% had insufficient and only 27.5% had sufficient levels of vitamin D. BMD Z score was significantly lower in βTM patients compared to controls (p
Source: Mediterranean Journal of Hematology and Infectious Diseases - January 1, 2019 Category: Hematology Source Type: research

Respiratory viral infections in children and adolescents with hematological malignancies
This study evaluated the clinical characteristics of RVI and established strategies for the PCR test in children and adolescents with hematological malignancies.Methods: This study included children and adolescents with underlying hematological malignancies and respiratory symptoms, in whom a multiplex PCR test was performed. Patients in whom RVI was identified and not identified were categorized into Groups I and II, respectively. Group I was sub-divided into patients with upper and lower respiratory infections. The medical records of the enrolled patients were retrospectively reviewed.Results: A total of 93 respiratory i...
Source: Mediterranean Journal of Hematology and Infectious Diseases - January 1, 2019 Category: Hematology Source Type: research

The use of hplc as a tool for neonatal cord blood screening of haemoglobinopathy - a validation study
Conclusions: The study validated the earlier observation by 100% concordance with results of CBC and HPLC. Presence of Hb Bart ’s at birth does not always mean the presence of alpha thalassemia, as subjects with Hb Bart’s was below 1% by quantitation, were shown to be normal by molecular studies. Key Words: Neonatal, screening, HPLC validation, haemoglobinopathy, sickle cell disease, thalassaemia  (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - January 1, 2019 Category: Hematology Source Type: research

Detection of CALR mutations using high resolution melting curve analysis (HRM-A); application on a large cohort of Greek ET and MF patients
ConclusionsOverall, our method could rapidly and cost-effectively detect the mutation status in a representative cohort of Greek patients; the mutation make-up in our group was not different from what has been published for other national groups. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - January 1, 2019 Category: Hematology Source Type: research

Hiv and lymphoma: from epidemiology to clinical management
Patients infected with human immunodeficiency virus are at increased risk for developing both non Hodgkin ’s lymphoma (NHL) and Hodgkin’s lymphoma (HL). Even if this risk has decreased for NHL after the introduction of combination antiretroviral therapy (cART), they remain the most common AIDS-related cancer in the developed world. They are almost always of B-cell origin, and some specific lymphoma types are more common than others. Some of these lymphoma types can occur in both HIV-uninfected and infected patients, while others preferentially develop in the context of AIDS. HIV-associated lymphoma differ from ...
Source: Mediterranean Journal of Hematology and Infectious Diseases - January 1, 2019 Category: Hematology Source Type: research

In Vivo Emergence of UL56 C325Y Cytomegalovirus Resistance to Letermovir in a Patient with Acute Myeloid Leukemia after Hematopoietic Cell Transplantation
CMV associated tissue-invasive disease is associated with a considerable risk of morbidity and mortality after allogeneic hematopoietic stem cell transplantation (HSCT). Recently, the terminase inhibitor letermovir (LMV) has been approved for prophylaxis of CMV infection in HSCT. We hereby report a 60-year-old female experiencing CMV reactivation after HSCT in a CMV seronegative donor-constellation. Due to ongoing elevated CMV viral load and drug-associated myelosuppression, which prevented ganciclovir therapy, treatment was replaced by foscarnet. Due to nephrotoxicity, foscarnet was switched to LMV. The patient developed ...
Source: Mediterranean Journal of Hematology and Infectious Diseases - January 1, 2019 Category: Hematology Source Type: research

International multicenter experience in the treatment of invasive aspergillosis in immunocompromised cancer patients
CONCLUSIONS:Based on international experience in immunocompromised cancer patients with IA, primary therapy with voriconazole-containing regimen is associated with improved response and survival compared with non-voriconazole amphotericin B based regimen. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - January 1, 2019 Category: Hematology Source Type: research

Bortezomib maintenance for the treatment of Monoclonal Gammopathy of Renal Significance
Monoclonal gammopathy of renal significance (MGRS) defines renal disease resulting from monoclonal proteins that are secreted from clonal B cells, that does not meet criteria for lymphoma or multiple myeloma. Recognizing MGRS in clinical practice is important because renal outcomes are poor and treatments targeting the underlying clonal disease have been associated with improved renal survival. In this case report, we present a case of a patient with membranoproliferative glomerulonephritis (MPGN) with IgG-kappa deposition who underwent clone directed treatment in a phased approach with induction and maintenance to achieve...
Source: Mediterranean Journal of Hematology and Infectious Diseases - January 1, 2019 Category: Hematology Source Type: research