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Current therapeutic practices in children with immune thrombocytopenia — Polish nationwide survey results
No full textIntroduction: Primary immune thrombocytopenia (ITP) is the most common thrombocytopenic disorder in children. The aim of this study was to assess the epidemiology and current therapeutic practices in children with ITP in Poland.
Material and methods: A survey was conducted in 19 pediatric hematology centers. The analysis covered the period of 2022–2023. The survey included 14 questions regarding the number of patients diagnosed and treated for ITP. The centers provided separate data for each year.
Results: In the years 2022–2023, a new diagnosis of primary immune thrombocytopenia (nITP) was made in 814 patients. A subgroup of 130 children with persistent ITP (16%) and a total of 452 with chronic ITP were treated. nITP was treated with IVIG (627; 77%) or steroids (154; 19%). TPO agonist drugs were administered as follows: eltrombopag in 215 (26%) patients, romiplostim in 115 (14%) patients, and avatrombopag in 6 (0.7%) patients. Other conservative therapies were administered to 38 (4.6%) patients, including: mycophenolate mofetil (n = 16), rituximab (n = 9), azathioprine (n = 3), sirolimus (n = 2), vincristine (n = 1), vinblastine (n = 1), cyclophosphamide (n = 1), mercaptopurine (n = 1), chronic steroid therapy (n = 2), and dexamethasone + eltrombopag (n = 1). Surgical treatment was performed in 4 patients, including splenectomy (n = 3) and splenic artery embolization (n = 1).
Discussion: A low level of adherence to the ASH 2019 recommendations regarding the use of steroids in nITP was noted. Oral TPO agonists were used twice as frequently compared to the subcutaneous form.
Conclusions: In the years 2022–2023, ITP was diagnosed in an average of 407 children annually. Immunosuppressive treatment was applied in 4.6% and surgical treatment in 0.5% of children with ITP
Management of acquired von Willebrand syndrome
No full textAcquired von Willebrand syndrome (AvWS) is a rare and often underdiagnosed bleeding disorder, estimated to affect up to 0.04% of the population. Unlike the hereditary form of von Willebrand disease (vWD), AvWS typically presents in adulthood in patients with no prior history of bleeding. First described in 1968 in a patient with systemic lupus erythematosus, AvWS is now increasingly recognized in association with hematologic malignancies (especially lymphoproliferative disorders), cardiovascular diseases (notably aortic stenosis and ventricular assist devices), and autoimmune conditions. The diagnosis remains challenging due to overlapping features with inherited vWD and the absence of a single definitive laboratory test. Key diagnostic steps include the assessment of von Willebrand factor antigen (vWF:Ag), factor VIII activity (FVIII:C), ristocetin cofactor activity (vWF:RCo), collagen binding assay (vWF:CB), and analysis of high-molecular-weight multimers (HMWM). The presence of an acquired inhibitor, shortened vWF half-life, or abnormal multimer pattern may support the diagnosis. Treatment should be directed at both the bleeding symptoms and the underlying disease. Therapeutic options include desmopressin, vWF/FVIII concentrates, intravenous immunoglobulin (IVIG), corticosteroids, and, in selected cases, plasmapheresis or immunosuppressive therapy. Definitive treatment of the comorbid condition (e.g., chemotherapy for lymphoma, valve replacement for aortic stenosis) often results in resolution of AvWS.
In this article, we propose a practical diagnostic and therapeutic algorithm for clinicians managing patients with suspected or confirmed AvWS, with particular focus on patients with hematologic malignancies. Clinical scenarios, laboratory interpretation, and response-guided treatment strategies are also discussed.
A dosimetric and treatment delivery comparisons of the Halcyon and Truebeam stereotactic plans for one or more brain metastases
No full textBackground: The purpose of this investigation was to determine if the Halcyon linear accelerator could provide a comparable level of treatment quality and efficiency to the TrueBeam linear accelerator when used for brain stereotactic radiosurgery (SRS) and stereotactic radiotherapy (SRT) in individuals with multiple brain metastases. The goal was to evaluate Halcyon as a potential substitute for TrueBeam.
Materials and methods: A review of prior treatments was performed, examining data from 30 individuals (encompassing 64 lesions) who had undergone TrueBeam SRS and SRT procedures. These existing treatment plans were then replanned for the Halcyon platform. Both treatment platforms employed 6 MV-FFF beams; however, the TrueBeam utilized a maximum dose rate of 1400 MU/min, while the Halcyon operated at 800 MU/min. Various dosimetric parameters, such as target coverage, doses to organs at risk, and gradient index, were compared, along with treatment delivery efficiency metrics, including monitor units and beam-on time. Statistical analysis was used for comparisons. Portal dosimetry was implemented for quality assurance.
Results: Both platforms achieved comparable target coverage and organ at risk (OAR) sparing, meeting Hypofractionated Treatment Effects in the Clinic (HyTEC), Quantitative Analyses of Normal Tissue Effects in the Clinic (QUANTEC), and the American Association of Physicists in Medicine Task Group 101 (AAPM TG-101) guidelines. Halcyon showed statistically equivalent gros tumor volume/planning target volume (GTV/PTV) doses, but a slightly higher gradient index (clinically insignificant). Beam-on time was longer for Halcyon due to its lower dose rate, but overall treatment time was potentially shorter due to efficient setup. Halcyon exhibited better QA pass rates.
Conclusion: Halcyon offers comparable dosimetric quality and treatment efficiency to TrueBeam for brain SRS/SRT. Its streamlined workflow and reduced setup time offer clinical advantages in high-volume centers, despite limitations like the lack of rotational couch correction and lower maximum dose rate
A review of interaction of radiotherapy and implants in head and neck cancer — implications and strategies to improve outcomes
No full textOral rehabilitation after the curative treatment of oral cancer is challenging. Improvement of functional and cosmetic rehabilitation following surgery and radiotherapy for head and neck cancers has been continuously attempted. Advances in dental and prosthetic implants have significantly improved quality of life. To achieve the best oncological, functional, and aesthetic outcomes, a multidisciplinary approach should be used to treat patients with oral cancer. Oral surgeons, reconstructive surgeons, implantologists, and radiation oncologists should have a good understanding of each other’s specialties. Radiation can interact with metallic implants and potentially increase the dose to tissues close to the implant, leading to undesirable complications, such as osteoradionecrosis. Radiation decreases implant survival by affecting osseointegration. Implant survival depends on the location and timing of the implant placement and the radiation dose received. Outcomes can be improved using adjunct hyperbaric oxygen, robust dental care before and during radiation, and modern radiotherapy techniques such as intensity-modulated radiotherapy
Rzadki przypadek wieloogniskowej żołtakoziarniniakowatości młodzieńczej — rozpoznanie ukryte między wierszami
No full textŻółtakoziarniniakowatość młodzieńcza (JXG, juvenile xanthogranuloma) jest rzadką jednostką chorobową, stanowiącą jednak zarazem najczęstszą histiocytozę z komórek nie-Langerhansa. Typowym objawem JXG jest pojedynczy, żółtawy guzek zlokalizowany w obrębie głowy lub szyi, rzadziej natomiast występują liczne, rozsiane zmiany. Pozaskórna lokalizacja zmian stanowi rzadkość. Pojawiające się u dzieci różowawe lub żółtawe grudki mogą wprowadzać w błąd i wydłużać czas do ustalenia rozpoznania. Pomimo że choroba ta występuje rzadko, powinno się ją rozważać w diagnostyce różnicowej, a z uwagi na różnorodność objawów oraz możliwość lokalizacji pozaskórnej należy przeprowadzać szczegółową diagnostykę. Wieloogniskowe układowe zmiany w przebiegu JXG mogą potencjalnie prowadzić do zgonu, a przebieg choroby zależy od umiejscowienia zmian. Zaleca się podejście multidyscyplinarne, obejmujące chemioterapię, leczenie operacyjne oraz immunosupresyjne
Surprising echocardiographic qualification for vascular surgery: A “twin heart syndrome”
No full textInadvertent implantation of a conduction system pacing lead into the left ventricle via patent foramen ovale in a dual-chamber pacemaker
No full textConstruction and validation of a histone-related gene signature for the diagnosis of endometriosis
No full textObjectives: Endometriosis is a common chronic disease in childbearing women and a major cause of infertility. Our study aimed to identify and validate a novel gene signature for diagnosing endometriosis based on histone-related genes (HRGs), and to investigate their biological functions in endometriosis.
Material and methods: RNA sequence data were downloaded from the Gene Expression Omnibus database, and HRGs were retrieved from the GeneCards database. We identified differentially expressed genes using the limma package, and constructed a diagnostic model using the rms package. Kyoto Encyclopedia of Genes and Genomes (KEGG) and Gene Ontology (GO) enrichment analyses were performed for visualization, annotation, and integrated discovery. Subsequently, we validated the model using the recall and decision curve analysis (DCA). Additionally, we analyzed the immune microenvironment features using CIBERSORT.
Results: A total of 18 differentially expressed HRGs were identified in patients with endometriosis compared with controls. GO and KEGG enrichment was mainly in spindle organization, positive regulation of the cell cycle process, progesterone-mediated oocyte maturation, and cellular senescence and cell cycle. We obtained a signature of four HRGs (JUNB, FRY, LMNB1, and SPAG1). DCA revealed that the diagnostic model benefits patients with endometriosis, regardless of the incidence. CIBERSORT analysis showed that the number of plasma cells increased significantly in endometriosis samples from all four datasets.
Conclusions: Our findings provide novel insights into the function of HRGs in the development of endometriosis and identify a new signature of four HRGs that may serve as valuable diagnostic markers and therapeutic targets for this disease