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Background/Aim. The cause of eosinophilia often remains unelucidated. The aim of the study was to analyze causes and treatment approaches in children with eosinophilia in pediatric tertiary care hospital. Methods. The medical records of children investigated for eosinophilia (based on the International Classification of Diseases code D72.1) were retrospectively reviewed in the University Children’s Hospital, Belgrade, Serbia, from December 2011 to December 2022. A total of 105 children (62 boys; male:female ratio was 1:4) aged one month to 16.5 years (median 7.7 years) were diagnosed with eosinophilia. After excluding 15 of them due to incorrectly assigned diagnosis based on relative eosinophil number only, the remaining 90 children were grouped according to the severity of eosinophilia (mild, moderate or severe). Results. Serological analysis con-firmed toxocariasis in six (6.7%) patients, while two (2.2%) had a confirmed nematode infestation (Ascaris lumbricoides and Enterobius vermicularis, respectively). Thirty-two (35.6%) children with eosinophilia and three with no true eosinophilia were diagnosed with helminthiasis ex juvantibus. Eosinophilia was ultimately explained by allergic/atopic conditions [19 (21.1%)], drug reactions [four (4.4%)], bacterial infections [nine (8.9%)], hematological problems [five (5.5%)], autoimmune disorders [three (3.3%)], unrelated congenital disorders (one), or as an isolated finding [seven (7.8%)]. In addition, one of the children without an increased absolute eosinophil number was diagnosed with eosinophilic esophagitis. A total of 56 (53.3%) children received anthelminthic treatment: 9 (90.0%) with severe eosinophilia, 19 (51.4%) with moderate, 23 (53.5%) with mild, and 5 (33.3%) children with no true eosinophilia. Most (42) of the children were given mebendazole only, while the remaining 14 (eight with severe, three with moderate, and three with mild) were also initially treated with mebendazole but subsequently shifted to albendazole due to the persistence of eosinophilia. In all treated children, eosinophilia and other relevant findings (if any) subsided in a matter of a few days to a few weeks after initializing treatment. Conclusion. Our results support the recommendation that unexplained eosinophilia of all levels of severity requires a standardized diagnostic approach. The results also provide some support for a potential rational basis for ex juvantibus administration of anthelminthic drugs in a fraction of children with eosinophilia without an obvious etiological explanation.

Introduction/Objective Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory condition characterized by fever, splenomegaly, and cytopenias. Diagnosis of HLH requires at least five of the eight criteria set by the Histiocyte Society and poses a significant challenge to physicians. HLH-2004 criteria include measurement of plasma levels of soluble receptor for interleukin-2 (sIL-2R), an invaluable tool in the diagnosis of HLH, particularly because it can be measured swiftly and inexpensively. Methods We retrospectively analyzed medical records of 45 pediatric patients (28 boys and 17 girls, median age 8.1 years) who were investigated for suspected HLH in University Children’s Hospital in Belgrade, during the period from 2012 to 2022. Results Ten children were diagnosed with HLH, while 35 did not have HLH. All 10 HLH patients had secondary HLH: eight suffered from infection or inflammatory condition, one from an autoimmune disease, and one from malignancy. Level of sIL-2R was above the HLH-2004 cutoff value of 2400 IU/ml in 9/10 patients with HLH (sensitivity 90%) and 9/35 of patients who did not have HLH (specificity 74.2%). Conclusion Soluble IL-2 receptor measurement is valuable in children suspected to have HLH. Sensitivity and specificity of this analysis can be further improved by strict patient selection and a comprehensive diagnostic approach.

Background: Several studies support the evidence of increased incidence of hematological complications in Gaucher disease including monoclonal and polyclonal gammopathies and blood malignancies, especially multiple myeloma. Methods: Serum concentrations of immunoglobulins and PCR analysis of the IGH gene rearrangements were performed. The clonal PCR products were directly sequenced and analyzed with the appropriate database and tools. Serum monoclonal proteins were detected and identified by electrophoresis. Results: Among 27 Gaucher patients, clonal IGH rearrangement was discovered in eight, with 5/8 having also serum monoclonal protein. Elevated immunoglobulins were detected in 9/27 patients. Follow-up data for 17 patients showed that the clonal rearrangement remained the same in four of them, however, in one patient it disappeared after the follow-up period. The remaining 12/17 patients were without previous IGH clonal rearrangement and remained so after the follow-up. Conclusions: Although clonal expansion may occur relatively early in the disease course, at least judging by the IGH gene rearrangements in Gaucher patients, the detected clones may be transient. A careful clinical follow-up in these patients is mandatory, including monitoring for lymphoid neoplasms, especially multiple myeloma.

Introduction. Intensive treatment protocols used for non-Hodgkin lymphoma in children lead to eventfree survival rates ranging from 80% to 90%. However, the results are less successful in developing countries. Lymphoblastic lymphoma (LBL) is the second most frequent type of lymphoma in children, contributing with about one third to all non-Hodgkin lymphoma in childhood. Objective. The aim of the study was to evaluate the results of LBL treatment in University Children’s Hospital (UCH), Belgrade. Methods. A retrospective analysis of patient records at UCH from 1997 to 2015 was carried out in patients aged 0-18 years, in whom the diagnosis of LBL had been established. Twenty-two children were included in the analysis. Results. Mean age at diagnosis was 10 years, with preponderance of male patients. All patients were treated according to Berlin-Frankfurt-Münster-based chemotherapy protocols. With median follow-up of 91.5 months, five-year probability of event-free survival was 79.5% for all patients, while overall survival was 81.8%. Conclusion. Our results, although slightly inferior to those of leading international groups, reflect a good treatment outcome in our patients. [Projekat Ministarstva nauke Republike Srbije, br. 41004]

Introduction. Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory condition characterized by fever, cytopenias, hepatosplenomegaly and hemophagocytosis. HLH may be primary or secondary to infection, autoimmune disease or malignancy. Hypertriglyceridemia is a common abnormality in HLH and one of the HLH-2004 diagnostic criteria. Case Outline. We present an infant with severe hypotonia and hypoproteinemic edema who also had extreme hypertriglyceridemia (21 mmol/l) and was diagnosed with HLH based on six of eight HLH- 2004 criteria (fever, hepatosplenomegaly, bicytopenia, hypertriglyceridemia with hypofibrinogenemia, sIL-2R > 2400 IU/ml, hemophagocytosis). The presence of IgM antibodies to Epstein-Barr virus and cytomegalovirus indicated a probable infectious trigger. The child was cured by the HLH-2004 protocol for secondary HLH (consisting of dexamethasone and cyclosporine). He was also found to have low serum hydroxycobalamin levels, promptly corrected upon hydroxycobalamin administration. Conclusion. The presented case history underlines the need to ascertain the presence or absence of each of the eight HLH-2004 criteria in any patient suspected to suffer from HLH. [Projekat Ministarstva nauke Republike Srbije, br. 41004]

Non-Hodgkin lymphomas of childhood represent a diverse group of neoplasms with different clinical, pathological, immunophenotypical and genetic features. A vast majority of childhood non-Hodgkin lymphomas could be classified into one of the three major histological subgroups: mature B-cell neoplasms, lymphoblastic lymphomas or anaplastic large cell lymphomas. Modern therapeutic strategies lead to cure in more than 80% of patients. Conversely, refractory diseases, as well as disease relapse convey a dismal prognosis. This fact requires much better stratification based on prognostic markers which would ideally recognize distinct groups of patients requiring different therapeutic regimens. Defining novel diagnostic and prognostic markers should improve diagnosis and prognosis as well as patient follow-up. It should also allow introduction of individually tailored treatment regimens in selected groups of patients with non-Hodgkin lymphomas, with the main goal of improving treatment results and decreasing short- and long-term complications. [Projekat Ministarstva nauke Republike Srbije, br. 41004]

Background: There is an apparently increased tendency toward infections in patients with Gaucher disease, possibly due to defective neutrophil function rather than a decreased neutrophil count. Since macrophages are the main cell type affected in Gaucher disease, our aim was to determine the contribution of these cells to the susceptibility of Gaucher patients to infection by studying the respiratory burst capacity of peripheral blood monocytes. Methods: The study was performed in eleven Gaucher type 1 patients and eleven sex and age matched control subjects by measuring peripheral blood monocytes' respiratory burst capacity using flow cytometry. The respiratory burst capacity was measured as dihydrorhodamine-123 median fluorescence in patients and respective controls. Results: There was no statistical difference in the median fluorescence among the patients and respective controls (p>0.05) after phorbol 12-myristate 13-acetate stimulation. Also, statistical difference was not reached among patients treated with enzyme replacement therapy at the time and those untreated. Conclusions: Flow cytometry might represent a more accurate and more reliable measure of respiratory burst compared to the methods of other researchers. Respiratory burst disturbance in monocytes does not seem to contribute to increased susceptibility to infection in Gaucher patients.

Visceral leishmaniasis (VL) is caused by the dissemination of the protozoan parasite Leishmania donovani throughout the reticuloendothelial system. This systemic disease may mimic or lead to several types of hematological disorders including hemophagocytic lymphohistiocytosis (HLH). Infection associated hemophagocytic syndrome caused by Leishmania is very rare and difficult to diagnose. Herein, we describe an infant with HLH associated with VL. The severity of HLH secondary to visceral leishmaniasis ranges from pure biological forms that resolve with antimicrobial therapy to life-threatening emergencies that require specific treatment. Our patient was treated with meglumine antimoniate. The outcome of the disease was favorable with a follow-up of 12 months. [ABSTRACT FROM AUTHOR]

Introduction: During invasive and orthopaedic procedure of the joints, physical therapy is a necessary modality for successful recovery, especially in patients with haemophilia. These patients require concurrent substitution therapy. Recombinant activated factor VII (rFVIIa) has been successfully used in treating haemophilia with inhibitor, especially during preoperative preparation. Nevertheless, optimal substitution therapy during perioperative physical therapy has been poorly defined., Case Outline: In our paper, we review the literature and the course of perioperative rehabilitation after synovectomy in a 16-year-old haemophilic patient with high-titre inhibitor, treated with rFVIIa. Rehabilitation was started on day 3 after surgery, with rFVIIa substitution lasting ten days and followed by intermittent dosages during episodes of pain. After concluding physical treatment, a significantly better function of the operated joint was recorded, which led to the recovery of walking without orthopaedic aid., Conclusion: Patients with inhibitors require individualized approach of a multidisciplinary team in order to achieve optimal results in elective orthopaedic procedures.

Introduction: Particular problem in treating haemophiliacs with serious muscle bleeding such as iliopsoas haematoma, are patients with inhibitor., Case Outline: Our study describes three episodes of psoas haematoma in a patient with haemophilia and inhibitor successfully treated with recombinant activated factor VII (rFVIIa)., Conclusion: Based on our experience, initiating therapy within two hours in our patient, contributed to successful treatment, although small cumulative doses of rFVIIa were used. Therefore, introducing home treatment along with adequate patient and family education similar to developed countries becomes imperative in our environment, not only for providing better quality of life, but also from pharamaco-economic point of view.