Early clearance of leukemic cells during induction therapy of childhood acute lymphoblastic leukemia (ALL) is a basis for treatment optimization. Currently, the most widely used methods for the detection of minute residual malignant cells in the bone marrow and/or peripheral blood, minimal residual disease (MRD), are PCR and flow cytometry (FCM). Immunophenotypic modulation (IM) is a well known factor that can hamper the accurate FCM analysis.Aim: To report the IM detected by 8-color FCM during the BFM-type remission induction in 24 consecutive MRD-positive samples of children with B-cell precursor ALL and the possible implications for MRD detection.Patients and methods: Between 2010 and 2012 we prospectively followed up the MRD on days 15 and 33 of induction treatment in bone marrow (BM) samples and on day 8 in peripheral blood (PB). The IM was assessed by comparative analyses of the changes in the mean fluorescence intensity of 7 highly relevant antigens expressed by the leukemic cells and normal B-lymphocytes.Results: IM occurred, to different extents, in all analyzed day 15 BM and in most day 33 BM samples. Statistically significant changes in the MFI-levels of four CDs expressed by the leukemic blasts were observed: downmodulation of CD10, CD19 and CD34 and upmodulation of CD20. No changes in the expression of CD38, CD58 and CD45 were noticed.Conclusions: Measuring the MRD by standardized 8-color flow cytometry helps improve the monitoring of the disease, leading to better therapeutic results. However, the IM of the different antigens expressed by the leukemic blasts should be taken into consideration and cautiously analyzed.
Neuroblastoma is the most common extracranial solid tumor in children, accounting for 15% of all pediatric cancer deaths. High-risk neuroblastoma (HRNB) is a particularly difficult-to-treat form of the disease that requires aggressive multimodality therapy, including induction chemotherapy, consolidation therapy with high-dose chemotherapy and autologous stem cell transplant, and maintenance therapy with dinutuximab beta. Despite treatment advances, the prognosis of these patients remains poor. As a better response to induction therapy has been associated with prolonged survival in patients with HRNB, we hypothesized that early use of dinutuximab beta—post-induction chemotherapy—may improve patient outcomes. We describe here our experience of administering at least one cycle of dinutuximab beta post-induction and prior to surgery in three children with HRNB who did not demonstrate a complete response to induction chemotherapy. All three patients achieved complete remission. Early use of dinutuximab beta may therefore have the potential to improve outcomes in patients with HRNB.
Lower gastrointestinal bleeding is a rare condition in childhood pathology. The incidence of this disorder in the general population of Bulgarian children is unknown. We report a case of a 7-year-old child with diagnosed hemophilia A and high titer of factor VIII inhibitor; the patient was admitted into the Department of Pediatrics and Medical Genetics for rectorrhagia after falling onto his buttocks while playing. Colonoscopy showed submucosal hematoma 25 cm from the anocutaneous line occluding the intestinal lumen with a lesion of the overlying mucosa as long as 20 mm. If a patient presents with rectorrhagia, timely and carefully planned colonoscopy could identify the source of bleeding, determine the severity of bleeding and the size of hematoma, and assess the need for surgical intervention. The reported case supports the modern view that patients with inhibitor hemophilia should not be denied interventional procedure or surgical intervention for fear of uncontrolled bleeding.
BACKGROUND: Nowadays febrile neutropenia is regarded as heterogeneous syndrome and adequate risk stratification of patients as a basis for tailoring empiric antibiotic therapy is attempted. We conducted this prospective study to determine risk factors for bacteremia in febrile neutropenic children that could be assessed in the first days from the onset of fever and based on logistic regression analysis to construct a risk index score for prediction of bacteremia. MATERIAL AND METHODS: We studied prospectively 199 consecutive episodes of febrile neutropenia for a 4-years period (2000 – 2004), occurring in 80 children with malignancies, treated by conventional chemotherapy. A standardized computer database with a set of variables for each febrile neutropenic episode was used. C-reactive protein was measured at the first fever and depending on its duration - on the 3-rd and 5-th day from the beginning of antibiotic therapy by immunoturbidimetric method. At the onset of fever and before initiation of antibiotic therapy blood cultures were drawn and injected directly into a culture bottle BACTEC Peds plus. RESULTS: By multivariate logistic regression analysis we determined 5 variables as independent risk factors for bacteremia: the underlying malignant disease (leukemia, NHL-gr.IV), chills, perianal cellulitis, presence of central venous catheter and degree of CRP rise ≥34.5 mg/L between the onset of fever and the 3rd day of empiric antibiotic therapy. This logistic regression model has sensitivity of 93%, specificity of 25%, negative predictive value of 89% and positive predictive value of 36% for the presence of bacteremia (Chi-square: 44, −2 log likelihood:200, Cox & Snell R square: 0.2). CONCLUSIONS: The present prospective study is characterized by prevalence of hematologic malignancies and relatively high frequency of bacteremia. We identified a low-risk group of 19.1%, comparable to the reported by other groups. Based on the statistical significance of serial measurement of C-reactive protein, which allows definite risk stratification of febrile neutropenic episodes on the 3rd day from the onset of fever, we would recommend sequential empiric antibiotic approach with switch to oral or even outpatient treatment in the low-risk group on the 3rd day.
Background: During the last four decades the prognosis of childhood acute myeloid leukemia (AML) has been substantially improved due to an increase in complete remission (CR) rates, event-free survival (EFS) and reduced early mortality. The relapsed AML still remains a therapeutic challenge. Aim: To report the AML treatment results of the Bulgarian pediatric oncohematological centers. Materials and methods: Retrospective analysis of the treatment results of children and adolescents (age from 0 to 20 years) with primary AML. Unifi ed AML BFM-backbone type treatment protocol is used. Results: This study included 97 newly diagnosed patients (44 girls and 53 boys) with AML in Bulgaria between 2003 and 2016. The median age at diagnosis was 10.2 years. The most frequent FAB-morphologic subtype was М2 followed by М4. First complete remission (CR1) was achieved in 83 patients (85.6%). The 13-year EFS was 49%, while the overall survival (OS) was 54.6%. Twenty seven (27.8%) patients relapsed, with only 5 of them being still alive towards the end of the study period. Conclusion: The EFS and OS for the children with AML in Bulgaria are comparable with those reported by other European groups. The prognosis of relapsed AML remains still unfavorable for the past 13 years. BACKGROUND
Introduction: Blood-induced joint damage as a hallmark of haemophilia continues to occur despite the widespread prophylaxis. Pre-cise assessment and follow-up of joint status are crucial for tailoring their treatment. Aim: To study the correlation between the bleeding phenotype, the functional joint status, and the magnetic resonance imaging score in pediatric patients with haemophilia. Materials and methods: Eighty-six joints (ankles, knees, and elbows) in patients aged 10.7±0.5 (range 4 - 20) years with severe/moderate haemophilia A, severe haemophilia B and haemophilia A with inhibitors were included in the study. The joints were assessed by Haemophilia Joint Health Score 2.1 (HJHS2.1) one month after the last hemarthrosis in a non-bleeding state. The magnetic reso-nance imaging was performed on 40 (46.5%) of the examined hemophilic joints (16 ankles, 11 knees and 13 elbows). Results: Joint bleeds were present in 37 (38.9%) of the joints with ankles being the most commonly affected. Sixty joints (69.8%) had normal HJHS2.1 score. Only the loss of flexion score differed significantly between the joints and the ankles had highest score. The cumulative number of hemarthrosis in the joint correlated moderately with hemosiderin deposition and strongly with the formation of subchondral cysts on magnetic resonance imaging. The magnetic resonance imaging scores for soft tissue and osteochondral domains correlated moderately with the cumulative number of hemarthrosis in the joint and only with the presence of pain and crepitus of mo-tion from the physical examination. Conclusions: Magnetic resonance imaging is more sensitive than the bleeding phenotype and physical examination in detecting early signs of haemophilic arthropathy.
Citation: Poriazova EG, Boykinova OB, Ivanov IS, Spasova MI, Bozhilov GB, Kevorkyan AK. A technical problem in diagnose clarity of solitary spinal cord neurofi broma in an eleven-month-old boy.
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