Hypertriglyceridemia associated with anticancer therapy based on asparaginase and steroids: a retrospective single center study of children with acute lymphoblastic leukemia and lymphoblastic lymphoma

Introduction: Hypertriglyceridemia (HTG) is one of the common complications of the regimens based on steroids and asparaginase used in the treatment of acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LBL) in children. The aim of this cross-sectional retrospective study was the analysis of the prevalence, clinical course and management of hypertriglyceridemia following the administration of asparaginase and steroids according to the binding protocols. Material and methods: A cohort of 75 children with ALL or LBL was analyzed with reference to anthropometric and laboratory parameters, clinical symptoms, implemented treatment, and complications. Results: The prevalence of HTG in the analyzed cohort was 29.3%. Risk factors for HTG development were older age, cachexia and lower body mass index, but there was no correlation with high risk group. Patients with HTG presented with elevated lipase acivity and total cholesterol concentration and decreased antithrombin, albumin, sodium and high-density lipoprotein cholesterol. Reported symptoms were unspecific. Management of HTG included: omega 3 fatty acids, fibrates, insulin and plasmapheresis. Conclusions: Hypertriglyceridemia is a significant complication of ALL and LBL treatment, and can lead to acute and late complications and cause unwelcome interruptions to therapy that can lead to poorer outcomes of treatment. As the course of HTG is oligosymptomatic, but can have undesirable repercussions, every patient receiving asparaginase with steroids should be monitored for HTG. It is also noteworthy that HTG can occur three or more weeks after asparaginase administration

Półpasiec trzewny u pacjenta po allogenicznym przeszczepie komórek hematopoetycznych szpiku – nieuwzględniana przyczyna w diagnostyce różnicowej ostrego brzucha

We report a case of 18-year-old male patient who 5.5 months after allogeneic hematopoietic stem cell transplant (HSCT) developed severe abdominal pain not responding to high dose of opioids. The pain was accompanied by gradually increasing activity of liver enzymes and bilirubin concentration. The patient had a history of acute GVHD and was on steroid taper. Importantly, he was also temporarily off standard acyclovir prophylaxis. Provisional diagnosis of acute cholecystitis was made, however, cholecystectomy did not improve patient's condition. Clinical picture of severe abdominal pain without clear surgical cause, resistant to high doses of opiates with increasing activity of liver enzymes was highly suspicious of visceral varicella zoster virus (VZV) reactivation. Immediate introduction of intravenous acyclovir led to full recovery and complete resolution of abdominal pain. We conclude that reactivation of latent VZV with absent or delayed occurrence of characteristic skin vesicles may still pose a diagnostic challenge resulting in delay of the proper diagnosis and start of life saving antiviral treatment. Severe intractable pain in HSCT recipients with increasing activity of liver enzymes should evoke high index of suspicion of the possible disseminated VZV and impose start of empirical treatment with high dose acyclovir

Nowoczesne obrazowanie z wykorzystaniem techniki wydruku 3D — technologia wspomagająca leczenie złożonych wrodzonych wad serca u dzieci

3D printing is more and more often applicated in modern medicine. We summarized present state of art regarding 3Dprinting technology and its capability in therapy of complex congenital heart diseases.  Techniki wydruku 3D coraz częściej wykorzystuje się we współczesnej medycynie. Podsumowano aktualny stan wiedzydotyczący tej technologii i możliwości jej zastosowania w leczeniu złożonych wrodzonych wad serca u dzieci

Polish recommendations for diagnosis and therapy of paediatric stroke

Stroke remains one of the greatest health challenges worldwide, due to a high mortality rate and, despite great progress in its treatment, the significant disability that it causes. Studies conducted around the world show that the diagnosis of stroke in children is often significantly delayed. Paediatric ischaemic arterial stroke (PAIS) is not only a problem that varies greatly in frequency compared to the adult population, it is also completely different in terms of its risk factors, clinical course and outcome. The main reason for the lack of a rapid diagnosis of PAIS is a lack of access to neuroimaging under general anaesthesia. The insufficient knowledge regarding PAIS in society as a whole is also of great importance. Parents and carers of children should always bear in mind that paediatric age is not a factor that excludes a diagnosis of stroke. The aim of this article was to develop recommendations for the management of children with acute neurological symptoms suspected of ischaemic stroke and further treatment after confirmation of the ischaemic aetiology of the problem. These recommendations are based on current global recommendations for the management of children with stroke, but our goal was also to match them as closely as possible to the needs and technical diagnostic and therapeutic possibilities encountered in Poland. Due to the multifactorial problem of stroke in children, not only paediatric neurologists but also a neurologist, a paediatric cardiologist, a paediatric haematologist and a radiologist took part in the preparation of these recommendations

Improvement of cure after hematopoietic stem cel transplantations in children

Background. Hematopoietic stem cell transplantation (HSCT) is an established procedure for many acquired and congenital disorders of the hematopoietic system, including malignancies, bone marrow failure syndromes, disorders of the immune system, and metabolic disorders. Objective. Analysis of results of hematopoietic stem cell transplantations performed over a period of 12 years in a single pediatric center. Patients and methods. All transplants performed between 2003 and 2015 in the Department of Pediatric Hematology and Oncology in Bydgoszcz were included in this analysis. The results of therapy with stem cell transplantation were analyzed in three time periods: 2004-2007, 2008-2011 and 2012-2015. Results. A total number of transplants was 318, including 132 auto-HSCT and 186 allo-HSCT. Among allogeneic transplants, 68 were done from matched-sibling donor and 118 from alternative donor. The mean survival for all patients estimated by Kaplan-Meier method was 8.1 years. Probability of overall survival (pOS) after all transplants was 0.64±0.03. pOS after allo-HSCT was 0.62±0.04, and 0.67±0.05 after auto-HSCT. Overall survival for patients transplanted in the second (2008-2011) and third (2012-2015) time period was comparable both for auto- and allo-HSCT. However, it was significantly higher than for patients transplanted in the first period of time (2004-2007) for all patients, and for those undergoing auto-HSCT. In allo-HSCT patients, in spite of increase of over 20% in pOS (43% vs 66% vs 64% in respective time periods), the difference was not statistically significant. Conclusion. Presented results of HSCT obtained in our center are comparable with those from other international registries and centers.Wstęp. Transplantacja komórek krwiotwórczych (HSCT) jest ważną metodą terapeutyczną w wielu wrodzonych i nabytych chorobach, w tym nowotworowych, zespołach niewydolności szpiku oraz zaburzeniach immunologicznych i metabolicznych. Celem pracy jest analiza wyników HSCT w pojedynczym ośrodku pediatrycznym w okresie 12 lat. Pacjenci i metodyka. Analizie poddano wyniki przeszczepień wykonanych w latach 2003-2015 w Klinice Pediatrii, Hematologii i Onkologii w Bydgoszczy. Transplantacje analizowano w trzech przedziałach czasowych: 2004-2007, 2008-2011 oraz 2012-2015. Wyniki. Wykonano 318 HSCT, w tym 186 alloge-nicznych (68 zgodnych rodzinnych i 118 od dawców alternatywnych) oraz 132 autologiczne. Średnie przeżycie po HSCT, wyznaczone metodą Kaplana-Meiera wyniosło 8,1 lat. Całkowite prawdopodobieństwo przeżycia (pOS) wyniosło 0,64±0,03; pOS po allo-HSCT wynosi 0,62±0,04, a po auto-HSCT 0,67±0,05. Nie wykazano znamiennych różnic w pOS zarówno po allo-HSCT, jak i po auto-HSCT pomiędzy drugim (2008-2011) i trzecim (2012-2015) analizowanym okresie. Jednakże, pOS było wyższe w dru-gim i trzecim okresie w stosunku do okresu pierwszego (2004-2007), zarówno dla wszystkich pacjentów, jak i u pacjentów po auto-HSCT. W grupie pacjentów allo-HSCT, uzyskano wzrost pOS o ponad 20% (43% vs 66% vs 64% w kolejnych przedziałach czasu; ns). Wnioski. Wyniki HSCT uzyskiwane aktualnie w na-szym ośrodku są porównywalne z wynikami podawanymi w międzynarodowych rejestrach

Znaczenie zgodności HLA na wyniki transplantacji komórek hematopoetycznych od dawców niespokrewnionych u dzieci z ostrymi białaczkami i niewydolnościami szpiku

BackgroundIn case of the lack of matched family donors (MFD), hematopoietic stem cell transplantation (HSCT) from unrelated donor (UD) is an established procedure for many acquired and congenital disorders of the hematopoietic system, including malignancies and bone marrow failure (BMF) syndromes.ObjectiveThe analysis of the results of HSCT in patients with acute leukemia or BMF syndromes from UDs with respect to human leukocyte antigen (HLA) match.Patients and methodsA total number of 97 of HSCT from UDs performed in single center between 2007 and 2015 in children and adolescents with acute lymphoblastic (ALL) or myeloblastic leukemia (AML) and BMF syndromes were included into this analysis. HLA match between donor and recipient was analyzed at the allele level and classified as 10/10, 9/10 or 8/10. Data were compared to results of 56 MFD-HSCTs. Probability of overall survival (pOS) was given for 3-year and 1-year (as required by JACIE standards) time periods.ResultsThe mean survival for all patients estimated by Kaplan–Meier method was 4.8 years (95%CI=4.1–5.5 years). The 3-year pOS after all UD-HSCT was 0,60±0,05, and with respect to 10/10, 9/10 and 8/10 HLA match: 0,61±0,06; 0,59±0,09 and 0,60±0,22, respectively (ns). In patients with AML, 3-year pOS reached 52%, 60% and 60%, respectively. In patients with ALL, 3-year pOS was 73% and 62% (ns) for 10/10 and 9/10 HLA match, respectively, while for BMF syndromes 86% and 57% (ns), respectively.ConclusionCurrent data suggest that results of mismatched and matched UD-HSCT in children with acute leukemia might be comparable

Clinical spectrum and outcome of invasive mucormycosis in children and adults: Polish experience of the decade 2010–2019

No epidemiological data exist so far on invasive mucormycosis (IM) in Polish hematopoietic cell transplantation (HCT) and pediatric hemato-oncology (PHO) centers. The objective of this study was to analyze the incidence, clinical course, therapy, and outcome of IM in pediatric and adult patients undergoing HCT and children with hemato-oncological diseases in Poland. A total number of 12425 at-risk patients were retrospectively analyzed, and the period between 2010 and 2019 was included. Patients were analyzed in three groups: nontransplant children with malignancies, children undergoing HCT, and adults after HCT. Twenty-one patients were diagnosed with IM, including 15 children (10 non-HCT, 5 HCT) and 6 HCT adults. Proven IM was confirmed in 18 patients, probable in 2 patients, and possible in 1 patient. Proven IM was diagnosed in 7.1% of all patients with invasive fungal diseases (IFDs), including 8.1% among PHO patients, 5.4% among pediatric HCT patients, and 7.0% among adult HCT patients. Clinically, pneumonia was diagnosed in 10 (47.6%) patients, involvement of the paranasal sinuses was found in 3 (14.3%) patients, gastrointestinal disease was noted in 2 (9.5%) patients, and disseminated mucormycosis was found in 6 (28.6%) patients. The probability of overall survival in IM patients was 0.50 ± 0.11. Infection-related mortality (IRM) was 10% for pediatric nontransplant IM patients and 82% for transplant IM (pediatric + adult) patients ( = 0.004). Among the transplant patients, all adults died within 120 days. IRM for pediatric HCT patients was 60% ( = 0.038). The only prognostic factor was HCT, which adversely influenced survival in IM patients