Posterior reversible encephalopathy syndrome (PRES) induced by intrathecal methotrexate administration in a patient with acute lymphoblastic leukaemia

A patient with B-cell precursor acute lymphoblastic leukaemia was diagnosed with posterior reversible encephalopathy syndrome (PRES) after an intrathecal administration of methotrexate during induction chemotherapy. PRES presented with headache, epilepsy, unconsciousness, blurred vision, hypertension, and vomiting. Also, characteristic lesions of the central nervous system were revealed by magnetic resonance imaging of the head, especially in the white matter of the posterior lobes

Low seroprevalence and low incidence of infection with "Toxoplasma gondii" (Nicolle et Manceaux, 1908) in pediatric hematopoietic cell transplantation donors and recipients : polish nationwide study

Czyzewski, Krzysztof, Fraczkiewicz, Jowita, Salamonowicz, Malgorzata, Pieczonka, Anna, Zajac-Spychala, Olga, Zaucha-Prazmo, Agnieszka, Gozdzik, Jolanta, Styczynski, Jan (2019): Low seroprevalence and low incidence of infection with Toxoplasma gondii (Nicolle et Manceaux, 1908) in pediatric hematopoietic cell transplantation donors and recipients: Polish nationwide study. Folia Parasitologica (019) 66: 1-6, DOI: 10.14411/fp.2019.019, URL: http://dx.doi.org/10.14411/fp.2019.01

Evaluation of PET quantitation accuracy among multiple discovery IQ PET/CT systems via NEMA image quality test

Introduction: Quantitative imaging biomarkers are becoming usual in oncology for assessing therapy response. The harmonization of image quantitation reporting has become of utmost importance due to the multi-center trials increase. The NEMA image quality test is often considered for the evaluation of quantitation and is more accurate with a radioactive solid phantom that reduces variability. The goal of this project is to determine the level of variability among imaging centers if acquisition and imaging protocol parameters are left to the center's preference while all other parameters are fixed including the scanner type. Methods: A NEMA-IQ phantom filled with radioactive Ge-68 solid resin was imaged in five clinical sites throughout Europe. Sites reconstructed data with OSEM and BSREM algorithms applying the sites' clinical parameters. Images were analyzed according with the NEMA-NU2-2012 standard using the manufacturer-provided NEMA tools to calculate contrast recovery (CR) and background variability (BV) for each sphere and the lung error (LE) estimation. In addition, a F-18-filled NEMA-IQ phantom was also evaluated to obtain a gauge for variability among centers when the sites were provided with identical specific instructions for acquisition and reconstruction protocol (the aggregate of data from 12 additional sites is presented). Results: The data using the Ge-68 solid phantom showed no statistical differences among different sites, proving a very good reproducibility among the PET center models even if dispersion of data is higher with OSEM compared to BSREM. Furthermore, BSREM shows better CR and comparable BV, while LE is slightly reduced. Two centers exhibit significant differences in CR and BV values for the F-18 NEMA NU2-2012 experiments; these outlier results are explained. Conclusion: The same PET system type from the various sites produced similar quantitative results, despite allowing each site to choose their clinical protocols with no restriction on data acquisition and reconstruction parameters. BSREM leads to lower dispersion of quantitative data among different sites. A solid radioactive phantom may be recommended to qualify the sites to perform quantitative imaging

Zespół tylnej odwracalnej encefalopatii wyindukowany dokanałowym podaniem metotreksatu u pacjenta z ostrą białaczką limfoblastyczną

U pacjenta z ostrą białaczką limfoblastyczną o różnicowaniu B-common rozpoznano zespół tylnej odwracalnej encefalopatii (PRES, posterior reversible encephalopathy syndrome) po dokanałowym podaniu metotreksatu w trakcie chemioterapii indukcyjnej. Zespół PRES objawia się bólami głowy, padaczką, zaburzeniami świadomości, zaburzeniami widzenia, nadciśnieniem tętniczym oraz wymiotami. W badaniu rezonansu magnetycznego głowy widoczne są charakterystyczne zmiany w istocie białej mózgu, szczególnie w płatach tylnych

Allogeneic bone marrow transplantation in children with acute lymphoblastic leukaemia in the first and second complete remission conditioned with fractionated total body irradiation and cyclophosphamide or etoposide

Patients and methodsFrom 1993 to 2001 thirty-two children underwent bone marrow transplantation (BMT) for acute lymphoblastic leukaemia (ALL) (12 in I complete remission /I CR/of high-risk/HR/ALL, and 20 in II CR after early bone marrow or combined bone marrow/organ relapse). Except for two syngeneic all others were matched sibling donor transplants. All patients (pts) were conditioned with fractionated total body irradiation (FTBI) at a total dose of 12,6 Gy, given in 8 fractions during 4 days with lung shielding (9,4 Gy) and cyclophosphamide (CY) 60 mg/kg i.v for 2 days (total dose 120 mg/kg) (n = 1 in I CR and n = 11 in II CR) or etoposide (VP) 60 mg/kg i.v (n = 11 in I CR and n = 9 in II CR). Patients in I CR were given 1,1–4,9×108 nucleated cells /kg (med. 2,7×108/kg), while pts in II CR 1,9–4,0×108 nucleated cells/kg (med. 2,7×108/kg). For graft versus host disease (GvHD) prevention cyclosporin A (CsA) 3 mg/kg/d i.v was administered alone in 22 pts (n = 9 in I CR and n = 13 in II CR) or in combination with “short” methotrexate +/− prednisone in 8 pts (n = 3 in I CR and n = 5 in II CR). Two pts transplanted with syngeneic BM received no GvHD prevention. The regimen related toxicity (RRT) was graded according to the system developed by Bearman et al. (1988).ResultsOnly mild or moderate expression of RRT was observed (GI toxicity I0 – 80%, II0 – 4%; stomatitis I0 – 40%, II0 – 20%; hepatic toxicity I0 – 28%; renal, bladder and cardiac toxicity I0 – 4%) and no transplant related deaths occurred (TRM = 0%). Among 12 pts transplanted in I CR only one child relapsed 4 months from BMT, while the remaining 11 pts are alive in continuous complete remission (CCR) with a median follow-up of 33 months (range 6 to 66 months) and 92% probability of a 5-year event free survival (pEFS). Of 20 children transplanted in II CR 6 relapsed 1–14 months from BMT (median 6,5 months). Thirteen of them remain in CCR with a median follow-up of 19.5 months (range 1 to 96 months) and with 66% probability of a 8-year EFS.Conclusions1. In children with ALL the FTBI-12,6 Gy-containing regimen is well tolerated without life-threatening toxic complications. 2. The FTBI-12,6 Gy-containing regimen demonstrates very good antileukaemic efficacy for HR-ALL in I CR, but only limited efficacy for ALL in II CR. 3. In the context of good tolerance of FTBI in a total dose of 12,6 Gy and its limited antileukaemic efficacy in children with ALL in II CR the escalation of FTBI total dose from 12,6 Gy to at least 13,2 Gy appears to be justified in those children

Optimal activity of [18F]FDG for Hodgkin lymphoma imaging performed on PET/CT camera with BGO crystals

Background: We aimed to find the minimum feasible activity of fluorodeoxyglucose ([18F]FDG) in positron emission tomography/computed tomography (PET/CT) of Hodgkin lymphoma patients performed on a camera with bismuth germanate (BGO) crystals. Material and methods: Ninety-one [18F]FDG PET/CT scans (each in seven Bayesian Penalized Likelihood [BPL] reconstructions with varying acquisition time per bed position — 2 min, 1.5 min, 1 min, 50 s, 40 s, 30 s, and 20 s) were independently assessed by three physicians to evaluate image quality. Mean administered activity was 3.0 ± 0.1 MBq/kg and mean uptake time was 54.0 ± 8.7 min. The series quality was subjectively marked on a 1–10 scale and then ranked 1–7 based on the mean mark. Interobserver rank correlation and intraclass correlation within each series for the three observers were calculated. Phantom studies were also performed to determine if reduced acquisition time can be directly translated into a reduced activity. Results: Time series were marked and ranked unanimously — the longer the time of acquisition the higher the mark and rank. The interobserver agreement in the ranking was excellent (100%) with a kappa coefficient of 1.00 (95% CI [0.83–1.0]). The general intraclass correlation coefficient (agreement between the marks observers gave each time series) was very high (0.945, 95% CI [0.936–0.952]) and was higher the shorter the time per bed. According to all three observers only the series with 2 min and 1.5 min acquisition time were appropriate for assessment (mean mark ≥ 7). In phantom studies there was a linear correlation between time per bed, administered activity, and number of total prompts detected by a scanner. Hence, a reduction of acquisition time of 25% (from 2 min to 1.5 min) could be directly translated into a 25% activity reduction (from 3.0 to 2.25 MBq/kg). Conclusions: In patients with HL, [18F]FDG activity can be reduced by up to 25% when using a BGO crystal camera, without substantial impact on image quality

Allogeneic Stem Cell Transplantation From HLA-Mismatched Donors for Pediatric Patients with Acute Lymphoblastic Leukemia Treated According to the 2003 BFM and 2007 International-BFM Studies: Impact of Disease Risk on Outcomes.

Summary Rational Allogeneic HSCT is beneficial for pediatric patients with relapsed or (very) high-risk ALL in remission. A total of 1115 consecutive patients were included in the ALL SCT 2003 BFM study and the ALL SCT 2007-International study and were stratified according to relapse risk (Standard vs. High vs. Very High Risk of Relapse) and donor type (Matched Sibling vs. Matched Donor vs. Mismatched Donor). Patients and methods A total of 148 patients (60% male, median age 8.7 years; B-cell precursor ALL: 75%) were transplanted from MMD, which was defined as either less than 9/10 HLA-compatible donors or less than 5/6 unrelated cord blood after myelo-ablative conditioning regimen (TBI-based: 67%) for HRR (n=42) or VHRR disease (n=106). The stem cell source was either BM (n=31), unmanipulated PBSCs (n=28), T-cell ex vivo depleted PBSCs (n=59) or cord blood (n=25). The median follow-up was 5.1 years. Results The 4-year OS and EFS was 56±4% and 52±4%, respectively, for the entire cohort. Patients transplanted from MMD for HRR disease obtained remarkable 4-y OS and EFS values of 82±6% and 80±6%, respectively, while VHRR patients obtained values of 45±5% and 42±5% (p Conclusion HSCT with a mismatched donor is feasible in pediatric ALL patients but leads to inferior results compared to HSCT with better matched donors, at least for patients transplanted for VHRR. The results are strongly affected by disease status. The main cause of treatment failure is still relapse, highlighting the urgent need for interventional strategies after HSCT for patients with residual leukemia before and/or after transplantatio

The impact of donor type on the outcome of pediatric patients with very high risk acute lymphoblastic leukemia. A study of the ALL SCT 2003 BFM-SG and 2007-BFM-International SG

Allogeneic HSCT represents the only potentially curative treatment for very high risk (VHR) ALL. Two consecutive international prospective studies, ALL-SCT-(I)BFM 2003 and 2007 were conducted in 1150 pediatric patients. 569 presented with VHR disease leading to any kind of HSCT. All patients >2 year old were transplanted after TBI-based MAC. The median follow-up was 5 years. 463 patients were transplanted from matched donor (MD) and 106 from mismatched donor (MMD). 214 were in CR1. Stem cell source was unmanipulated BM for 330 patients, unmanipulated PBSC for 135, ex vivo T-cell depleted PBSC for 62 and cord-blood for 26. There were more advanced disease, more ex vivo T-cell depletion, and more chemotherapy based conditioning regimen for patients transplanted from MMD as compared to those transplanted from MSD or MD. Median follow up (reversed Kaplan Meier estimator) was 4.99 years, median follow up of survivals was 4.88, range (0.01–11.72) years. The 4-year CI of extensive cGvHD was 13 ± 2% and 17 ± 4% (p = NS) for the patients transplanted from MD and MMD, respectively. 4-year EFS was statistically better for patients transplanted from MD (60 ± 2% vs. 42 ± 5%, p < 0.001) for the whole cohort. This difference does not exist if considering separately patients treated in the most recent study. There was no difference in 4-year CI of relapse. The 4-year NRM was lower for patients transplanted from MD (9 ± 1% vs. 23 ± 4%, p < 0.001). In multivariate analysis, donor-type appears as a negative risk-factor for OS, EFS, and NRM. This paper demonstrates the impact of donor type on overall results of allogeneic stem cell transplantation for very-high risk pediatric acute lymphoblastic leukemia with worse results when using MMD stem cell source

Cord blood transplantations in Polish pediatric centers: report of the Polish Pediatric Group for Hematopoietic Stem Cell Transplantation

WstępPrzeszczepianie komórek krwiotwórczych krwi pępowinowej (CBT) jest uznaną metodą terapeutyczną, wykonywaną od roku 1988. Pierwsze przeszczepienie w Polsce wyłącznie z krwi pępowinowej wykonano 12 października 2000 r. w Poznaniu.Cel pracyAnaliza wyników przeszczepiania komórek krwiotwórczych w polskich ośrodkach pediatrycznych.Pacjenci i metodykaDo badań włączono 19 pacjentów (5 dziewcząt, 14 chłopców), w wieku 0,1–10 lat (mediana 4,3 roku), u których w latach 2000–2011 w polskich ośrodkach pediatrycznych wykonano przeszczepienie krwi pępowinowej. Pacjentów kierowano do CBT z powodu następujących rozpoznań: ostra białaczka limfoblastyczna (n=6), ostra białaczka mieloblastyczna (n=1), zespół mielodysplastyczny (n=2), zespół Wiskotta i Aldricha (n=3), niedokrwistość Fanconiego (n=2), adrenoleukodystrofia (n=1), histiocytoza Langerhansa (n=1), przewlekła choroba ziarniniakowa (n=1), zespół Kostmanna (n=1), zespół Sandhoffa (n=1). Zastosowano kondycjonowanie mieloablacyjne u 9 pacjentów oraz o zredukowanej toksyczności u 10 pacjentów. Krew pępowinowa pochodziła od dawcy rodzinnego w 8 przypadkach lub od dawcy niespokrewnionego w 11 przypadkach. Zgodność 6/6 HLA wystąpiła w 10 przypadkach.Wyniki10/19 (52,6%) dzieci żyje, mediana przeżycia 3,1 roku (95%CI=1,4–4,7), prawdopodobieństwo przeżycia 2-letniego wynosi 0,409±0,133. Przyczyny zgonów obejmowały brak przyjęcia przeszczepu (n=2), powikłania infekcyjne (n=3) lub wznowę (n=4). Dwoje dzieci z powodu nieprzyjęcia przeszczepu miało wykonane przeszczepienie haploidentyczne. W analizie wielowariantowej, jedynym czynnikiem prognostycznym mającym wpływ na całkowite przeżycie było wystąpienie udokumentowanego uogólnionego zakażenia do dnia +180.WnioskiU pacjentów niemających zgodnego dawcy CBT jest ważną opcją terapeutyczną, dającą szanse wyleczenia dla około połowy pacjentów.BackgroundCord blood transplantation (CBT) is accepted therapeutic method in transplantology since 1988. The first isolated CBT was performed on 12 October 2000 in Poznań.ObjectiveAnalysis of results of CBT in Polish pediatric centers.Patients and methodsA total numer of 19 patients (5 female, 14 male), aged 0.1–10 years (median 4.3yrs) transplanted with cord blood between 2000–2011 in Polish pediatric centers. The initial diagnosis was: acute lymphoblastic leukemia (n=6), acute myeloid leukemia (n=1), myelodysplstic syndrome (n=2), Wiskott-Aldrich syndrome (n=3), Fanconi anemia (n=2), adrenoleukodystrophy (n=1), Langerhans cell histiocytosis (n=1), chronic granulomatous disease (n=1), Kostmann syndrome (n=1), Sandhoff syndrome (n=1). Pre-transplant conditioning was myeloablative in 9 patients and reduced-intensity in 10 patients. The source of cord blood was family donor in 8 cases or unrelated donor in 11 cases. Histocompatibility 6/6 HLA between donor-recipient was present in 10 cases.Results10/19 (52.6%) children stay alive, median survival 3.1 years (95%CI=1.4–4.7), probability of 2-year survival was 0.409±0.133. The cause of death was primary graft failure (n=2), infectious complications (n=3) or relapse (n=4). Two children with primary graft failure had subsequent haploidentical transplantation. In multivariate analysis, generalized documented infection was the only predictive adverse factor of overall survival.ConclusionCBT is an important therapeutic option for patients lacking matched donor, offering positive outcome for a half of patients