Hematology Clinic Research Articles

Third-degree atrioventricular block and pacemaker implantation in a man with severe hemophilia A: A case report.

Hemophilia A (HA) is a rare bleeding disorder characterized by reduced factor VIII (FVIII) activity and consequently spontaneous bleeding. Since the introduction of prophylaxis with safer FVIII concentrates, people with HA are ageing. Interestingly, they are developing cardiovascular diseases as their non‐hemophilia counterparts. We describe a 48‐year‐old patient with severe HA who presented a third‐degree atrioventricular block. A DDDR pacemaker was implanted under supervision of the Hematology Clinics. There were no adverse events during the procedure. The procedure was safe, and it should be performed under the supervision of a hemophilia expert.

Acute Severe Thrombocytopenia Event Associated with Trimethoprim/Sulfamethoxazole Use.

Objective:To report a case of life-threatening thrombocytopenia associated with the use of trimethoprim/sulfamethoxazole (TMP/SMX) therapy.Report of the case:50-year-old woman with no significant past medical history who presented with one day of petechial rash on her arms, chest and legs. Patient reports that she had just completed a 7- day course of TMP/SMX (1-double strength tablet twice a day) for uncomplicated UTI by her PMD. On admission, the patient was hemodynamically stable, and complete blood cell count revealed a platelet count of 2000/uL. TMP/SMX was believed to be the most likely cause of thrombocytopenia. After discontinuation of TMP/SMX and treatment with 2 units of platelets, 1gm intravenous immunoglobulin (IVIG) and oral dexamethasone, repeat CBC showed a stable platelet count of 90,000/uL. Patient was successfully discharged on hospital day 3 with outpatient follow up with the hematology clinic for further monitoring.Conclusion and Discussion:Differential diagnosis of severe thrombocytopenia include drug induced thrombocytopenia (DITP), thrombotic thrombocytopenic purpura (TTP), post transfusion purpura (PTP), immune thrombocytopenic purpura (ITP), heparin induced thrombocytopenia (HIT), or catastrophic antiphospholipid antibody syndrome (APS). Drug-dependent antibodies are an unusual class of antibodies that bind firmly to specific epitopes on platelet surface glycoproteins only in the presence of the sensitizing drugs. DITP typically has an abrupt onset of severe thrombocytopenia, usually less than 20,000/uL. Thrombocytopenia usually begins to recover within 1-2 days after the offending drug is discontinued and platelet levels usually normalize within one week as demonstrated in our case report. Pharmacological treatment can include platelet transfusions in case of severe, overt bleeding, corticosteroids or IVIG administration. In most cases, however, discontinuation of the offending drug is sufficient.

Addressing unmet basic needs for children with sickle cell disease in the United States: clinic and staff perspectives

BackgroundThe purpose of this study was to assess pediatric hematology clinic staff’s perspectives regarding barriers and facilitators in addressing unmet basic needs for children with sickle cell disease (SCD).MethodologySix focus groups were held at four urban pediatric hematology clinics in the Northeastern region of the United States from November to December 2019. Discussion questions were developed to align with the integrated Promoting Action on Research Implementation in Health Services (i-PARIHS) implementation science framework, focusing on the domains of context and recipient and how clinics address adverse social determinants of health (SDoH) in their patient populations. A summative content analytical approach was taken to identify major themes in the data.ResultsWe discerned the following themes: (1) families of children with SCD experience numerous unmet basic needs; (2) clinic staff believed they had a role to play in addressing these unmet basic needs; (3) staff felt their ability to address families’ unmet basic needs depended upon caregivers’ capacity to act on staff’s recommendations; and (4) clinic staff’s ability to address these needs was limited by organizational and systemic factors beyond their control.ConclusionsThese findings have important implications for how best to address adverse SDoH for this vulnerable pediatric population so that urban-based pediatric hematology clinics can more equitably support families.

Determination of the relationship between mortality and SOFA, qSOFA, MASCC scores in febrile neutropenic patients monitored in the intensive care unit.

PurposeFebrile neutropenia (FN) is a hematological emergency. It is challenging and confusing for the clinicians to make the decision of the febrile neutropenic patients under chemotherapy to be monitored at intensive care unit (ICU). The aim of this study was to define the factors supporting decision-making for the critical patients with febrile neutropenia.MethodsThe data of 60 patients, who were taken to the ICU while they were under treatment in the Hematology Clinic with a diagnosis of febrile neutropenia, were analyzed retrospectively, in order to identify clinically useful prognostic parameters.ResultsThe ICU mortality rate was 80%. Mortality was significantly associated with higher sequential organ failure assessment score (SOFA), quick sequential organ failure assessment score (qSOFA), and hematological SOFA (SOFAhem) scores on admission. All cases having SOFA score 10 and above and qSOFA score 2 and above died. In multivariate analysis, qSOFA score was found to be statistically significant in predicting mortality in regard to ICU admission (p = 0.004).ConclusionMortality of febrile neutropenic patients admitted to ICU is high. It would be appropriate to determine the extent of organ dysfunction instead of underlying disease, for making the decision of ICU admission. It should be noticed that the risk mortality is high for the FN cases with SOFA score 10 or above, qSOFA score 2 or above, and in need of mechanical ventilation and positive inotropic support; hence, early intervention is recommended. In our study, the most significant parameter in predicting ICU mortality was found to be qSOFA.

Assessment of serum zinc and copper levels in Egyptian children with β-thalassemia major

Background Thalassemia, with about 60,000 individuals born annually, is a severe inherited anemia, arising from the failure of hemoglobin synthesis. Objective This cross-sectional study aimed at evaluating the levels of serum zinc and copper in Egyptian children with β-thalassemia major on two different oral iron chelators, and its relation to clinical and routine laboratory parameters. Materials and Methods This cross-sectional study was conducted on sixty Egyptian children, with β-thalassemia major, who were recruited from the Hematology Clinic in New Children Hospital. All of them were below 18 years old. The hemoglobin electrophoresis of each patient was revised as a diagnostic investigation. The patients were classified into two groups: Group I included 30 patients on oral deferasirox, while group II included 30 patients on oral deferiprone. Thirty, age-and-sex-matched children were included in the study as controls. Both cases and controls were subjected to laboratory investigations, which are complete blood count, bilirubin (total and direct), alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, calcium and phosphorous, and zinc and copper levels in serum. Samples were analyzed spectrophotometrically on Beckman AU 680 (Beckman Coulter International, Nyon, Switzerland) using dedicated manufacturer reagents. Serum zinc and copper levels were measured by atomic absorption spectrometry (Perkin Elmer, Buckinghamshire, United Kingdom). Results Thirteen percent of the studied cases had low serum zinc level, while none of the control children had low serum zinc level. There was only one case that had high serum zinc level. Thirty five percent of the studied cases had high serum copper level, while none of the controls had high serum copper level, and only one case had low serum copper level. Comparing both types of oral chelators, there was no significant difference in them, regarding the zinc and copper levels. Conclusions Serum levels of zinc and copper were significantly altered in the thalassemic cases more than the controls. These levels were not related to the type of oral chelator used or their serum ferritin levels.

Inverstigation of corneal and lens densitometry in eyes of patients with primary myelofibrosis

Aim: To investigate the corneal-lens densitometry and endothelial cells of Primary myelofibrosis patients.Materials and Methods: We evaluated 49 eyes of 49 primary myelofibrosis patients on follow up in hematology clinic and compared to 46 eyes of 46 age and sex-matched control group . The Scheimpflug images of 90–270 degrees by Pentacam HR (Oculus Optikgerate GmbH, Wetzlar, Germany) were used for measurement. After pupil dilatation, the corneal apex was marked by pentacam automatically and the diameter of central 6 mm around marked point was analyzed. PNS (Pentacam Nuclear Staging) software was used for densitometric analysis of the lens from a cylindrical region in the nucleus center. Specular Microscope CEM-530 (Nidek, Japan) was used for endothelial cell analysis. Results: There was no statistically significant difference between groups for age and gender(Table1). Corneal density and corneal volume were higher in the patient group but it was not statistically significant (respectively p=0.390, p=0.078). Lens density was found as statistically significantly higher in the patient group compared to the control group(p0.001). For the corneal endothelial analysis by specular microscopy, there was no statistically significant difference for cell density, the coefficient of variant and endothelial hexagonality (respectively, p=0.546, 0.671, 0.103).Conclusion: Primary myelofibrosis does not affect corneal densitometry and corneal endothelial cells. However, there is a higher lens densitometric measurement in Primary myelofibrosis compared to the control healthy group.

The Role of Helicobacter Pylori Infection in Idiopathic Thrombocytopenic Purpura in Children

Background: Many studies had found obvious relation between helicobacter pylori infection and chronic idiopathic thrombocytopenic purpura; several studies have reported remission of immune thrombocytopenic purpura (ITP) after eradication of a coexistent Helicobacter pylori infection in adults, data in children are limited. Objective: To detect the association of H. pylori infection and idiopathic thrombocytopenic purpura in children and the effect of eradication of the infection on platelet count in these patients. Patients and methods: This study was conducted on 83 chronic idiopathic thrombocytopenic purpura children with age range from 3 to 13 years. They were 46 males 37 females. Also the study included 104 healthy individual with the same range of age. They were 58 males and 46 females as a control group. This was between October 2018 and September 2019 in the Pediatric Hematology and GIT Clinics or inpatients wards of Pediatric Department. Results: Clinical benefit occurred in 31 cases of the 83 chronic ITP (cITP) patients screened; nevertheless, we have a simple, inexpensive, non‐invasive screening method; the adverse events of eradication therapy are negligible, together with an affordable cost, all uncommon attributes among the specific treatments currently available for cITP; therefore it is worthwhile to test for and treat H. pylori infection. Conclusion: Our data showed a prevalence of H. pylori infection in cITP pediatrics that was similar to other studies. We found that successful H. pylori eradication induced platelet response in 37.3% of 83 cases included in our study.

The Role of Helicobacter Pylori Infection in Idiopathic Thrombocytopenic Purpura in Children

Background: Many studies had found obvious relation between helicobacter pylori infection and chronic idiopathic thrombocytopenic purpura; several studies have reported remission of immune thrombocytopenic purpura (ITP) after eradication of a coexistent Helicobacter pylori infection in adults, data in children are limited. Objective: To detect the association of H. pylori infection and idiopathic thrombocytopenic purpura in children and the effect of eradication of the infection on platelet count in these patients. Patients and methods: This study was conducted on 83 chronic idiopathic thrombocytopenic purpura children with age range from 3 to 13 years. They were 46 males 37 females. Also the study included 104 healthy individual with the same range of age. They were 58 males and 46 females as a control group. This was between October 2018 and September 2019 in the Pediatric Hematology and GIT Clinics or inpatients wards of Pediatric Department. Results: Clinical benefit occurred in 31 cases of the 83 chronic ITP (cITP) patients screened; nevertheless, we have a simple, inexpensive, non‐invasive screening method; the adverse events of eradication therapy are negligible, together with an affordable cost, all uncommon attributes among the specific treatments currently available for cITP; therefore it is worthwhile to test for and treat H. pylori infection. Conclusion: Our data showed a prevalence of H. pylori infection in cITP pediatrics that was similar to other studies. We found that successful H. pylori eradication induced platelet response in 37.3% of 83 cases included in our study.

Early mortality and overall survival in acute promyelocytic leukemia – a single-center experience

Acute promyelocytic leukemia (APL) is a subtype of acute leukemia (AL) with distinct cytogenetics, clinical and biological characteristics. APL was considered as one of the most rapidly lethal forms of acute myeloblastic leukemia (AML), but recently, with the introduction of all-trans retinoic acid (ATRA) it has become the most curable subtype of AL. The main difficulty with APL is early death (ED), defined as death because of any cause within 30 days after diagnosis, and it has emerged as the most important cause of treatment failure. Our retrospective-prospective study was realized at the University Clinic for Hematology from January 2004 until December 2020. It included 46 patients with APL, according to FAB and WHO classification with confirmed molecular diagnosis. The following patients’ risk stratification factors were analyzed: age, Sanz risk score, WBC, PL, clinical presentation of the disease, levels of fibrinogen and D-dimers. During the study period, APL was diagnosed in 46 patients, 24 females (52.2%) and 22 males (47.8%), with mean age of 45 years. The overall survival showed that 24 patients (52.1%) were alive and 22 (47.8%) had lethal outcome. Regarding treatment, five patients (10.9%) died before starting the chemo-treatment. But, still, ED was observed in 13 patients (59%), and in 9 patients (40.9%) death occurred 30 days after establishing the diagnosis. The main reasons of mortality were also analyzed. To prevent ED prior to treatment, suspected APL patients should be immediately hospitalized and treated as medical emergency. Keywords: acute promyelocytic leukemia, early death, all-trans retinoic acid

Citomegalovirusna reaktivacija kod pacijenata u procesu alogene transplantacije matičnh ćelija hematopoeze

Introduction: Opportunistic CMV reactivation is the most common viral complication after allogenic hematopoietic stem cell transplantation (allo-HSCT). Aim: The aim of our study is to evaluate the frequency of CMV reactivation in relation to the serostatus od the donor and the recipient, and the correlation with the day of leukocyte (Le) and thrombocyte (Tr) engraftment. We compared the frequency of CMV reactivation in myeloablative conditioning (MAC) versus reduced intensity conditioning (RIC), as well as in match related donor (MRD) versus match unrelated donor (MUD) allo-HSCT. We analyzed whether CMV reactivation affected the overall survival (OS) after allo-HSCT. Materials and methods: In a retrospective cohort study, we inspected 42 patients over the age of 18 years, who were treated at the Clinic for Hematology of the Clinical Center of Serbia, from December 2017 to November 2019. Results: Most CMV reactivations were noticed if the recipient (R) was seropositive, and the donor (D) was seronegative (R+/D= 60.0%). The number of CMV DNA copies corelated with the day of leukocyte engraftment of (p = 0.031), but not of thrombocyte engraftment (p = 0.598). The frequency of reactivation in patients treated with RIC was 25.0%, and it was 63.5%, if they were treated with MAC. The intensity of the conditioning regimen corelated with the number of CMV DNA copies (p = 0.025%). There was no correlation found between the type of transplantation (MRD or MUD) and CMV reactivation (p = 0.515). OS after allo-HSCT was 36.39 months (95% CI 26,0 - 46,78). The mean OS in patients with CMV reactivation was 7.39 months (95% CI 5,72 - 9,06), but we did not prove that CMV reactivation had an impact on OS (p = 0.527). Conclusion: CMV reactivation was most common in the R+/Dgroup. CMV reactivation did not affect OS after allo-HSCT in our group of patients.

Myeloma multiplex treatment and overall survival

The Multiple myeloma (MM) is a plasma cell malignancy in which monoclonal plasma cells proliferate in bone marrow, resulting in an overabundance of monoclonal paraprotein (M protein), destruction of bone, and displacement of other hematopoietic cell lines. This retrospective-prospective study was conducted at the University Clinic for Hematology in Skopje, North Macedonia, in the period between January 2009 and December 2019. Patients younger than 65 years, without comorbidities, fit for autologous peripheral blood stem cell transplantation (PBSCT), were treated with Cyclophosphamide-Thalidomide-Dexamethasone (CyThalDex) protocol divided into two daily doses which were maintained until complete remission. Patients over 65 years of age, unfit for more aggressive treatment options like peripheral blood stem cells (PBSCT) with comorbidities and renal failure, were treated with Melphalan-Prednisone-Thalidomide (MPT) protocol. The third group of patients was treated without new immunomodulators such as thalidomide, but instead a salvage therapy was given consisted of chemotherapy and corticosteroids. The use of thalidomide can lead to more undesirable effects such as deep vein thrombosis and renal neuropathy. The results obtained in our study showed no high percentage of these effects. However, a better survival rate was registered along with a longer period without progression of the underlying disease (PFS). Moreover, a higher percentage of complete remission (CR) was achieved and a very good partial response (VGPR) in general. Myeloma multiplex is still incurable disease with pattern of regression and remission followed by multiple relapses rising from the residual myeloma cells, but in the future still many unsolved questions has to be answered. Keywords: myeloma multiplex, autologous stem cell transplantation, thalidomide

Uticaj antigljivične profilakse na pojavu gljivičnih infekcija kod bolesnika u programu alogene transplantacije

Introduction: Patients with hematologic malignancies, such as acute myeloid leukemia and acute lymphoblastic leukemia (AML/ALL), myelodysplastic syndrome (MDS), and those undergoing allergenic stem cell transplantation (alloSCT) are at the highest risk of invasive fungal infections (IFI). The most common causative agents are Candida spp. and Aspergillus spp. Among the strategies for preventing IFIs is the adequate implementation of antifungal prophylaxis recommended by the NCCN (National Comprehensive Cancer Network). Aim: The aim of the study was to analyze the occurrence of IFIs in these patients, as well as to analyze the impact and importance of timely antifungal prophylaxis with regards to the development of these infections. Materials and methods: The retrospective study included 42 patients, of the average age of 35 years, who underwent the allo-SCT program, between 2017 to 2019, and received antifungal prophylaxis at the Clinic for Hematology of the Clinical Center of Serbia (CCS). Based on information obtained from medical histories, databases were formed. Statistical analysis included descriptive statistical methods that were performed in the SPSS program. Results: Nineteen (45.2%) patients presented with the clinical manifestation of oral candidiasis. Invasive pulmonary aspergillosis developed in only 3 (7.1%) patients. There was a statistically significant association between clinically manifest aspergillosis (7.1%) and the presence of antigens (Galactomannan) in these patients (p <0.001). There was also a statistically significant association between clinically manifest aspergillosis and graft weakness: 2 (66.6%) vs. 1 (33.3%), (p = 0.016). Conclusion: The use of adequate antifungal prophylaxis significantly reduces the incidence of IFIs in patients undergoing the allo-SCT program, and this contributes to the reduction of morbidity and mortality.

The Relationship Between Thrombo-Inflammatory Biomarkers and Cellular Indices of Inflammation in Lymphoma Patients.

IntroductionThrombo-inflammatory biomarkers play an important role in the pathogenesis of lymphoma. We aimed to characterize the interrelationship of thrombo-inflammatory biomarkers and blood cellular indices in lymphoma patients.Materials and MethodsNinety-eight lymphoma patient samples were collected from Lymphoma Center of Clinic of Hematology, University of Belgrade, Serbia. Normal controls (n = 50) represented plasma from healthy individuals. Plasminogen activator inhibitor (PAI-1), D-Dimer, factor XIII, C-reactive protein (CRP), microparticles (Mp), Von Willebrand factor (vWF), total protein S, urokinase-type plasminogen activator (uPA), tumor necrosis factor (TNFα), β2-glycoprotein I (β2GPI), and fibronectin levels were measured utilizing commercially-available ELISA methods. Thrombin generation profile (TGA) was measured using a fluorometric kinetic assay. Platelets, leukocytes, lymphocytes, and neutrophils were measured in conjunction with the complete blood profile.ResultsStatistically significant differences were noted in levels of PAI-1, D-Dimer, factor XIII, CRP, microparticles, vWF, uPA, TNFα, β2GPI, fibronectin, and TGA when compared to normal (all P values < .001). Platelet to leukocyte ratio (PLA) correlated to TNFα and fibronectin (R = −0.31 and −0.53, respectively) and the platelet to neutrophil ratio (PNR) correlated to factor XIII and β2GPI (R = 0.40 and 0.40, respectively).ConclusionPlasma samples from lymphoma patients demonstrated a significantly altered thrombo-inflammatory biomarker profile that has notable correlations to blood cellular indices.

Učestalost infekcija tokom primene visokodozne hemioterapije sa autolognom transplantacijom matičnih ćelija

Introduction: Autologous stem cell transplantation (AHSCT) is a well-established therapy for hematologic malignancies. Changes in transplantation strategies and improvement in supportive care have significantly altered the incidence and pattern of infections in these patients. Aim: Evaluating the frequency of infections in the first 30 days after AHSCT, as well as the possible influence of the number of CD34 + stem cells in the graft and of the engraftment parameters: ALC500_20 (absolute lymphocyte count 0.5x109 / L per day + 20), ANC500_11 (absolute neutrophil count 0.5x109 / L per day + 11), and PLT20_13 (platelets 20x109 / L per day + 13), on the occurrence of infections. Materials and methods: The retrospective cohort study examined 80 patients above the age of 20 years, diagnosed with multiple myeloma (MM), non-Hodgkin's (NHL) or Hodgkin's lymphoma (HL), treated at the Clinic for Hematology of the Clinical Center of Serbia, in the period between July 2006 and December 2017. All episodes of fever and/or documented infection during neutropenia have been reported. Results: The average survival after AHSCT was 34.5 months. A total of 54 patients (67.5%) had a documented infection. Gram-positive infections were five times more common than gram-negative. In gram-positive isolates, coagulase-negative staphylococcus - CoNS was the most common (37.0%) pathogen, followed by Streptococcus a haemolyticus (12.4%). Among gram-negative isolates, Escherichia coli was present in 62.5% of the cases, while Klebsiellaspp. and Ralstonia pickettii were represented with an equal frequency of 12.5%. Fungal infections were rare (Candida spp., 10.0%). Viral infections were verified in 5 (6.3%) patients (Herpes zoster virus 3.8% and H1N1 2.5%). Conclusion: The number of CD34+ stem cells in the graft, as well as the rate of hematopoietic reconstitution, i.e., the achievement of ALC500_20, ANC500_11, and PLT20_13, were not statistically significant for the development of infections in the early phase after AHSCT.

Karakteristike bolesnika sa sekundarnom eritrocitozom u odnosu na bolesnike sa policitemijom verom

Introduction: Erythrocytosis represents elevated hemoglobin and hematocrit levels above the range of normal values. Primary erythrocytosis - polycythemia vera, is characterized by increased erythrocyte production, due to a disorder at the level of the multipotent stem cell in the bone marrow. On the other hand, secondary erythrocytosis (SE) is the result of bone marrow stimulation by an external factor. Aim: The aim of our study was to determine parameters which are significant in differentiating SE from primary erythrocytosis - polycythemia vera (PV). Materials and methods: This is a retrospective study involving 108 patients with SE and 111 patients with PV, who were diagnosed and treated at the Clinic of Hematology of the Clinical Center of Serbia (CCS), in the period: December 2005 - November 2018.From the patient records, the following data were extracted: demographic characteristics, laboratory parameters, spleen size, total red cell mass, serum erythropoietin (EPO) level, and spontaneous growth of the BFU-E colony. Results: Patients with SE were younger, with a predominance of the male gender and with significantly higher serum EPO values than patients with PV. Patients with PV had significantly higher values of BFU-E, leukocyte and platelet count, spleen size, and LDH level than patients with SE. Total red cell mass analysis did not show a differential diagnostic significance. Conclusion: Findings of normal spleen size, normal leukocyte and platelet count, normal serum LDH level, and elevated EPO, in patients, refer to the diagnosis of secondary erythrocytosis, while the findings of splenomegaly, leukocytosis, thrombocytosis, elevated serum LDH level, decreased EPO, and the presence of spontaneous BFU-E colony speak in favor of the diagnosis of polycythemia vera.

Diseminovana intravaskularna koagulopatija u akutnoj nepromijelocitnoj mijeloidnoj leukemiji - učestalost, kliničko-laboratorijske karakteristike i prognostički značaj

Introduction: Acute promyelocytic leukemia (APL) has the highest risk for overt disseminated intravascular coagulopathy (DIC), with reported incidence of DIC of up to 90%, as compared to 10-40% in other AML types. The influence of DIC on early death in non-APL AML patients has not been evaluated so far. Aim: The aim of our study was to analyze the incidence of DIC, its clinical and laboratory characteristics, and the impact on the survival and early death of patients with non-APL AML. Materials and methods: A total of 176 patients with non-APL AML, diagnosed and treated at the Clinic for Hematology of the Clinical Center of Serbia, between 2015 and 2020, were evaluated retrospectively. The diagnosis of DIC was made on the basis of ISTH (International Society on Thrombosis and Haemostasias) criteria. Results: The mean age of our patients was 53.8 ± 14.6 years, with 99/176 patients being men (56.2%). DIC was present in 74/176 patients (42.05%), who had a significant prevalence of the hemorrhagic syndrome (p = 0.01). The risk factors for overt DIC were the following: older age (p &lt;0.01), comorbidities (p = 0.01), leukocytosis (p &lt;0.001) and a high level of LDH (p &lt;0.001). The FAB (French, American and British) type of non-APL AML, the cytogenetic risk group, and CD56 (cluster of differentiation) had no influence on overt DIC (p &gt; 0.05). No difference was found in early mortality, outcome, and the survival of non-APL AML patients, with and without DIC (p &gt; 0.05). Conclusion: Older age at diagnosis, comorbidities, leukocytosis, and high LDH concentrations are found to be adverse risk factors for overt DIC in non-APL AML patients. If treated promptly, with immediate, adequate and intensive use of blood derivates and components, DIC has no negative impact on early mortality, outcome, and survival.

Prognostički značaj vrednosti serumskog feritina pri inicijalnoj dijagnozi kod bolesnika sa akutnom mijeloidnom leukemijom

Introduction/Aim: Acute myeloid leukemia (AML) is a heterogenous malignant disease whose course and outcome are influenced by a number of prognostic factors. Serum ferritin (SF) is often elevated in oncology patients, and it has been shown that it strongly influences an unfavorable outcome in various malignancies. The aim of this study is to assess the effect of high SF values on overall survival and disease-free survival, as well as to assess the correlation of SF values with other prognostic markers, such as clinical and laboratory parameters. Methods: Retrospective analysis included 108 patients diagnosed with AML at the Clinic for Hematology of the Clinical Center of Serbia (CCS), in Belgrade, in the period 2017 - 2019. Patients with acute promyelocytic leukemia, acute mixed lineage leukemia, secondary AML and patients treated with palliative therapy were excluded from the study. Patients were grouped based on the SF cutoff value of 800 µg/L. Results: Patients with higher SF values had a significantly higher incidence of early death (p = 0.020), sepsis in the induction phase of therapy (p &lt; 0.010), and significantly lower initial hemoglobin levels (p = 0.040), as compared to patients with lower SF values. SF at diagnosis appeared to be a significant independent predictive factor of overall survival (p = 0.019) and of disease-free survival (p = 0.040). Conclusion: Our study showed a significant association of high SF values with sepsis in induction, early death, mean hemoglobin, overall survival, and disease-free survival. Identification of SF as an independent prognostic factor and a potential target site of the action of new drugs could contribute to a better prognosis of AML patients.

Role of circulating myeloid-derived suppressor cells in pathogenesis of immune thrombocytopenia in children and adolescents

Context Regulatory T cells have an immunosuppressive function on T-cell activation. They are involved in pathophysiology and treatment of immune thrombocytopenia (ITP). Circulating myeloid-derived suppressor cells (MDSCs) are involved in immune dysregulation in ITP. Aim To determine the mean level of MDSCs in acute, persistent, and chronic ITP and its effect on treatment modalities and prognosis. Patients and methods A total of 41 patients with ITP were recruited from the pediatric hematology clinic of Ain Shams University. They were classified into acute, persistent, and chronic and were compared with 20 age-matched and sex-matched healthy controls. All patients were subjected to history taking with emphasis on age of presentation, disease duration, and treatment modalities, as well as thorough clinical examination. Mean values of C-reactive protein, alanine aminotransferase, and serum creatinine were collected from the patients’ files. All study participants underwent complete blood count (Coulter) and MDSCs by flow cytometry. Secondary thrombocytopenia was excluded. Results Acute ITP was detected in 29%, 24% had persistent, and 46% had chronic ITP. Their age ranged from 1 to 16 years at the study entry, and 51.2% were male. Active disease was found in 58.5%, whereas 41.4% in remission. No treatment was offered to 53%, whereas 24% of patients were on steroids. MDSCs decreased significantly in patients with ITP compared with the control group (P&lt;0.001), whereas there was no significant difference among the patient groups regarding MDSCs level (P=0.325) or with different treatment modalities. Conclusion Reduced numbers of MDSCs play a role in pathogenesis of ITP. Yet, MDSCs did not differ according to disease duration or treatment modalities.

Perception to hydroxyurea therapy in patients with sickle cell disease: Report from 3 centers.

Background:Hydroxyurea (HU) is an hemoglobin F inducing agent used in the treatment of sickle cell disease (SCD).Aim:The aim of this study is to determine the perception of HU by people living with SCD.Materials and Methods:A pretested questionnaire was self-administered to known cases of SCD attending pediatrics and adult hematology clinics in three participating centers. Mothers of children <18 years responded on their behalf.Results:There were 101 responders, 49 (48.5%) males and 52 (51.5%) females, of which 24 (23.8%) were children <18 years and 77 (76.2%) were adults. The majority (n = 73, 72.3%) knew their phenotype. Up to 63 (62.4%) had crises in the past 3 months. Only 35 (34.7%) had heard of HU, many through their doctor (n = 16, 45.7%), 8 (22.9%) through online resources, and 7 (20%) from friends. Only 12 (11.9%) had been exposed to HU therapy, of which 5 (41.7%) had discontinued therapy mostly due to side effects (n = 2, 40%). The seven patients (58.3%) on continuous HU therapy for a duration of 6 months to over 5 years, all reported reduced hospital admissions and frequency of crises as benefits of the drug, whereas 4 (57.1%) had stopped requiring blood transfusion since starting therapy. Of those who had never taken HU, 53 (52.5%) believed that HU should be used in treating SCD and majority (n = 32, 60.4%) would want to be commenced on the drug. However, 8 (15.1%) would decline therapy (mostly due to perceived associated side effects; n = 4; 50%). Six (11.3%) were unsure if they would want the drug and 7 (13.2%) would have to discuss the decision first with their family. There were 8 (8.9%) responders who did not think HU will be beneficial in SCD and would decline treatment, while 26 (29.2%) were unsure of both the benefits of the drug or of commencing therapy.Conclusion:The findings from this study suggest that HU is beneficial for patients with SCD; however, the awareness of this medication among SCD patients is still low in our environment. Some SCD patients would decline the use of HU due to perceived side effects. We recommend that more awareness on HU be created and coordinated multi-center studies on the efficacy of HU in the Nigerian population be carried out.

Factors influencing vaccination status against influenza and pneumococcus in sickle cell anemia patients

Aim: The aim of the present study is to investigate the factors which influence vaccination status against seasonal influenza and pneumococcus in patients with sickle cell anemia which is known to be the most common hereditary hematologic disease worldwide.Materials and Methods: This prospective, cross-sectional study was conducted with the volunteer patients above 18 years who were admitted to Hematology Clinic of Baskent University Adana Research and Training Center due to sickle cell anemia during 01 December – 31 December 2018.Results: Of 38 patients, 22 (57.9%) were females. Ratio of vaccination against pneumococcus was found to be higher among the patients who had vaccination cards (p0.05). Ratio of vaccination against pneumococcus and influenza was found to be higher among the patients who were coming for regular controls and the difference was statistically significant (p0.05). The reason for not getting vaccinated was detected to be “forgetting vaccination” (37.5%), “not believing the necessity of vaccination” (25%) and “not being recommended by health authorities” (25%).Conclusion: It was concluded that vaccination rates could be increased through using vaccination cards, inviting the patients for regular controls and health authorities’ recommending vaccination.