Gábor Ottóffy

Immunogenicity of a 2009 pandemic influenza virus A H1N1 vaccine, administered simultaneously with the seasonal influenza vaccine, in children receiving chemotherapy.

No examination of simultaneous vaccination against pandemic H1N1 and the seasonal influenza virus strains, in children with cancer receiving chemotherapy, are yet published. We investigated the immunogenicity of a whole‐virion, inactivated, adjuvanted pandemic H1N1, and seasonal influenza vaccines administered simultaneously to children with cancer undergoing chemotherapy.

Angiomatoid Fibrous Histiocytoma: Pleomorphic Variant Associated with Multiplication of EWSR1-CREB1 Fusion Gene

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor which exceptionally occurs in visceral organs or bones. Histologically this is a bland, monomorphic tumor and only occasionally shows pleomorphism. Vast majority of the soft tissue cases share the same translocation and the resulting EWSR1-CREB1 gene fusion as background pathogenetic alteration. Here we report a 10-year-old boy with subcutaneous tumor of the right shoulder. Histological, immunohistochemical and FISH analyses of the case revealed pleomorphic phenotype, characteristic immunophenotype and multiplication of the EWSR1-CREB1 fusion gene in the nuclei of the tumor cells. The possible explanation of the fusion gene multiplication, its relation to the morphology and the clinical outcome are discussed in the context of the published literature.

Detection of early precursors of t(12;21) positive pediatric acute lymphoblastic leukemia during follow-up†

DNA‐, RNA‐, and cell‐based methods provide different biologic information for determining the presence of minimal residual disease (MRD). We monitored the responses of patients with pediatric acute lymphoblastic leukemia (pALL) using DNA markers, TEL/AML1 expression, and scanning fluorescence microscopy (SFM). Using SFM, 36% of patients exhibited 1.5–3.1 log and 2.9–4.2 log higher MRD levels compared with those based on DNA and RNA markers, respectively. CD10+ ancestor cells with germline antigen receptors, but silent TEL/AML1 expression, may reside in the lymphoid stem cell compartment of treated t(12;21)‐positive patients and might act as a potential source of cells for late relapses. Pediatr Blood Cancer 2010; 54:158–160.

Sequential and hierarchical chromosomal changes and chromosome instability are distinct features of high hyperdiploid pediatric acute lymphoblastic leukemia

Pathogenesis of the non‐random accumulation of extra chromosomes in the low and high hyperdiploid (HeL, HeH) pre‐B pediatric acute lymphoblastic leukemia (B‐pALL) is largely unknown, and has been clarified with respect only to tetrasomic chromosomes. We analyzed the hierarchy of changes in chromosome number and chromosomal instability, as well as clonal heterogeneity and evolution, in the untreated bone marrow cell samples from 214 B‐pALL patients.

Korszakváltás a gyermekkori szerzett csontvelő-elégtelenséggel járó kórképek kezelésében Magyarországon

INTRODUCTION Acquired bone marrow failures are rare but fatal diseases in childhood. Since 2013, Hungary has been participating as a full member in the work of the European Working Group on uniform diagnostics and therapy in patients with acquired bone marrow failure syndromes. Hypocellular refractory cytopenia of childhood has been emphasized as a frequent entity, transplanted by reduced intensity conditioning with excellent outcomes. AIM To analyse and compare the results of treatment before and after our joining. METHOD A total of 55 patients have been treated in the 8 centres of the Hungarian Pediatric Oncology Network during 5 years between 2013 and 2017 (severe aplastic anemia: 9, myelodysplastic syndrome: 41, juvenile myelomonocytic leukemia: 5 patients). Allogeneic hematopoietic stem cell transplantation was performed in severe aplastic anemia in 7 cases, while antithymocyte globulin was administered in one case and one patient died before diagnosis. In patients with myelodysplastic syndromes, watch and wait strategy was applied in 4, while transplantation in 37 cases. Reduced intensity conditioning was used in 54 percent of these cases. Transplantation was the treatment of choice in all 5 patients with juvenile myelomonocytic leukemia. RESULTS In the whole patient cohort, the time from diagnosis to treatment was median 92 (3-393) days, while in severe aplastic anemia median 28 (3-327) days only. Grade II-IV acute graft versus host disease occurred in 22.6%, grade III-IV in 6.8% and chronic in 11.2%. All the patients treated with severe aplastic anemia are alive and in complete remission (100%). The overall estimated survival rate is 85.1% in myelodysplastic syndrome, while 75% in juvenile myelomonocytic leukemia. The median follow-up was 30.4 (1.1-62.5) months. There was a remarkable increase in overall survival comparing the data before (1992-2012) and after (2013) joining the international group, 70% vs. 100% (p = 0.133) in severe aplastic anemia and 31.3% vs. 85.1% (p = 0.000026) in myelodysplastic syndrome. CONCLUSION Due to a change in the paradigm of the conditioning regimen in hypocellular refractory cytopenia of childhood, the overall survival rate has significantly increased. Orv Hetil. 2018; 159(42): 1710-1719.

X kromoszómához kötött limfoproliferatív betegség (XLP): Az Epstein–Barr-vírus (EBV) találkozása egy ritka génhibával

Absztrakt: Az X kromoszomahoz kotott limfoproliferativ betegseg (XLP) ritka immundeficienciaval jaro szindroma, melyet az SH2D1A gen mutacioja okoz. A betegseget az Epstein–Barr-virusfertőzes aktiv...

Case 3: A neonate with an abdominal mass.

A male neonate was born at 39 wk gestation by vaginal delivery after 16 h of ruptured membranes. His birthweight was 3200 g, and Apgar scores were 9 and 10 at 1 and 5 min, respectively. Findings of the first physical examination revealed no abnormalities. The vaginal culture of the mother was positive for Candida sp. Because of the maternal history and the 16 h of ruptured membranes, a blood sample was drawn from the newborn and marginally elevated C-reactive protein (15.3 mg/l; reference range B/10 mg/l), elevated indirect bilirubin level (15.6 mg/dl, direct fraction 0.58 mg/dl) and normal WBC count (9.8 /10/mm) were found. Ampicillin and phototherapy were therefore initiated. On ‘‘routine’’ ultrasound (US) screening, bilateral adrenal haemorrhage was observed, which was supported by an elevated D-dimer level (2086 mg/l; reference range B/500 mg/l). Despite the ampicillin treatment, CRP elevated further (54 mg/l) and leukocytosis (17.3 /10/mm) also appeared. Antibiotic therapy was therefore changed to cefuroxime. Cultures of blood, cerebrospinal fluid, throat, urine and stool were negative. The baby had no fever and tolerated oral feeding well. Physical examination revealed a transiently palpable abdominal mass in the subcostal region on the right side. Because the serum CRP level was invariably elevated (64 mg/l), the antimicrobial therapy was modified, and a combination of ampicillin, cefotaxime and gentamycin was started. This regime was applied for an 8-d period. However, the size of the right adrenal mass increased further (51 /34 /35 mm), but it had been absorbed on the left side. On the 18th day of life, the baby was transferred to our NICU. US showed a central hyperreflective zone surrounded by an 8 12-mm-thick hypoechogenic area. Blood flow was not observed in the mass. In the 3rd week of life, the antibiotic treatment was terminated. The baby was continuously afebrile and had normal weight gain, although infection markers remained elevated (CRP 112 mg/l, ESR 180 mm/h, WBC 12 /10/mm with 56% neutrophils, 38% lymphocytes and 6% monocytes). Repeated measurements of urinary vanillylmandelic acid (VMA) showed normal levels; however, consequently elevated neuron-specific enolase (NSE) values (26, 30 and 29.6 mg/l; normal value B/12.5 mg/l) were detected. Abdominal CT scan demonstrated a large homogenic mass on the upper part of the right kidney, having a 2 3-mm-thick capsule separating it from the liver and the kidney (Figure 1). The right suprarenal gland was dislocated medially by this mass. After injection contrast medium, the picture was not changed. Because of the possibility of anaerobic infection on the basis of elevated infectious markers, clindamycin therapy was initiated. At this point, the baby was pale and his haemoglobin level had decreased to 7.4 g/dl (haematocrit of 22%), so a blood transfusion was given. The size and homogeneity of the mass did not change during the clindamycin treatment. At 6 wk of age, an operation was carried out to reveal the diagnosis.