- Research Article
- 10.3324/haematol.2026.300514
Peripheral T-cell lymphoma in adult-onset familial hemophagocytic lymphohistiocytosis type 2 and heterozygous LRBA mutation.
- Mar 19, 2026
- Haematologica
- Marco Tembrink + 3 more +3
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Publications from 2021 to 2026
Showing 10 of 324 papers
Peripheral T-cell lymphoma in adult-onset familial hemophagocytic lymphohistiocytosis type 2 and heterozygous LRBA mutation.
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S3 guideline diagnostics and therapy of alopecia areata - Part 1: Diagnostics and epidemiology.
In the project funded by the Innovation Committee at the G-BA, the S3 guideline for the diagnosis and treatment of AA was developed between 2023 and 2025. The interdisciplinary expert panel consisted of representatives from the German Dermatological Society, in particular from the Pediatric Dermatology Working Group, the Professional Association of German Dermatologists, the German Society for Psychosomatic Medicine and Psychotherapy, the German Society for Pediatrics and Adolescent Medicine, the Austrian Society for Dermatology and Venereology, a hairdresser as an external consultant and two patient representatives. In the first of two parts, we present the key statements on epidemiology and the recommendations for diagnosis. Data from Germany show a population-based prevalence of AA of 0.22% (95%CI 0.21-0.22) for 2016 and 0.21% (95%CI 0.20-0.22) for 2020. This means that 170,000 people in Germany were diagnosed with alopecia areata (AA) in 2020. International data show a pooled prevalence of 2.1%, but with large regional differences. Data on the incidence of AA from Germany show that 72 patients per 100,000 were newly diagnosed with AA in 2020 (RR 0.072%; 95%CI 0.07-0.08). Extrapolated to the German population, this means that around 70,000 people were newly diagnosed with AA in 2020.
Read moreExanthema with eosinophilia induced by medication with lithium and quetiapine
Cutaneous reactions due to lithium medication are well-known, including acneiform eruptions, exacerbations of psoriasis and alopecia. However, an association between lithium treatment and the occurrence of drug-induced exanthema has been reported much less frequently. We report the case of a 55 year old patient with bipolar disorder, who developed a maculopapular exanthema with eosinophilia during treatment with lithium and quetiapine. The skin eruptions persisted despite initial treatment with systemic glucocorticoids as long as the lithium medication was continued. Histology revealed spongiotic dermatitis, a finding consistent with drug-induced exanthema.
Read moreVascular access for initial treatment of adult emergency patients in the resuscitation room: short summary of recommendations from the German national S1 guideline
The initial treatment in the resuscitation room involves vital procedures, including the placement of peripheral intravenous lines, central venous catheters, and arterial lines. As most vascular access guidelines were not developed for resuscitation room settings, clear recommendations were necessary. This guideline has been developed by an interdisciplinary, interprofessional group of experts from five German national medical professional societies. The first part of the guideline provides general recommendations for vascular access in the resuscitation room in adults, whereas the second part describes specific recommendations and strategies for particular emergency situations.
Read moreSuper-Resolution Microscopy Filtration Slit Density Measurements in Various Kidney Diseases: Results from a Real-World Feasibility Study
Fertility in patients with advanced-stage classic Hodgkin lymphoma treated with BrECADD versus eBEACOPP: a secondary analysis of the multicentre, randomised, parallel, open-label, phase 3 HD21 trial.
Efficacy and Safety of a Balanced Gelatine Solution for Fluid Resuscitation in Sepsis: A Prospective, Randomised, Controlled, Double-Blind Trial-GENIUS Trial
Background/Objective: Sepsis is a leading cause of death in noncoronary intensive care units (ICUs). Fluids for intravascular resuscitation include crystalloids and colloids. There is extensive clinical evidence on colloid use, but large trials comparing gelatine with crystalloid regimens in ICU and septic patients are lacking. This study aimed to determine whether early, protocol-driven volume resuscitation using a gelatine-based regimen achieves hemodynamic stability (HDS) more rapidly than a crystalloid-based regimen in septic patients. Methods: This prospective, controlled, randomised, double-blind, multinational phase IV study compared two parallel groups of septic patients receiving a gelatine-based regimen (Gelaspan® 4% and Sterofundin® ISO, B. Braun Melsungen AG each, at a 1:1 ratio) or a crystalloid regimen (Sterofundin® ISO). Primary endpoint was time to first HDS within 48 h after randomisation. Secondary endpoints included fluid overload, fluid balance, and patient outcomes. Results: 167 patients were randomised. HDS was achieved after 4.7 h in the gelatine group and after 5.8 h in the crystalloid group (p = 0.3716). The gelatine group had a more favourable fluid balance at 24 h (medians: 3463.00 mL vs. 4164.00 mL; p = 0.0395) and less fluid overload (medians: 4296.05 vs. 5218.75%; p = 0.0217). No differences were observed in serious adverse events or mortality. Conclusions: The study provided clinical evidence of balanced gelatine solution for volume resuscitation in septic patients, although it was terminated prematurely. The early and protocol-based administration of gelatine was safe and effective in the enrolled patient population. Time to HDS was not different between groups but the gelatine-based regimen led to better fluid balance and less fluid overload.
Read moreSupportive Schmerztherapie in der Dermatologie
ZusammenfassungIn der konservativen Dermatologie treten relevante Schmerzen als begleitendes Symptom komplexer und schwerwiegender Erkrankungen auf und beeinträchtigen die Lebensqualität maßgeblich. Zwei typischerweise mit Schmerzen einhergehende Erkrankungen sind der Herpes zoster und das Pyoderma gangraenosum. Die supportive Therapie umfasst Maßnahmen, die zusätzlich zur krankheitsspezifischen Therapie eingesetzt werden, um krankheitsbegleitende Symptome wie Schmerzen zu lindern und das Wohlbefinden der Patienten zu verbessern. Inhalt dieser Übersichtsarbeit ist die spezifische pharmakologische Schmerztherapie unter Berücksichtigung von Kontraindikationen und Anwendungsbeschränkungen einschließlich Dosierung, Dosisanpassung und Wechselwirkungen. Neben Nichtopioidanalgetika finden auch gezielt ausgewählte Opioide in der Schmerztherapie Anwendung, können jedoch durch Nebenwirkungen wie Übelkeit und Obstipation das Wohlbefinden und die Therapieadhärenz der Behandelten vermindern. Eine supportive Begleittherapie dieser Nebenwirkungen ist daher essenziell. In diesem Artikel werden Prinzipien für die gezielte pharmakologische Schmerztherapie, basierend auf den aktuellen Leitlinien und Empfehlungen der Fachgesellschaften, speziell für den dermatologischen Einsatz aufgearbeitet.
Read moreThe Advanced Organ Support (ADVOS) hemodialysis system fulfills its intended purpose: Analysis of data from 282 patients from the Registry on Extracorporeal Multiple Organ Support (EMOS)
Background Several case series have highlighted the ADVOS hemodialysis system's efficacy in eliminating water-soluble and protein-bound substances across diverse patient populations, such as multiorgan failure, acute-on-chronic liver failure (ACLF), acidosis, and even COVID-19. The EMOS-Registry, a non-interventional, multi-center patient registry, amassed real-world evidence, culminating in the largest patient cohort treated with ADVOS to date. This study aims to present and analyze the final performance and safety outcomes from the entire dataset comprising 282 participants. Methods Data spanning from January 18, 2017, to August 31, 2020, were collected from five German hospitals, encompassing subsets of patients with acidosis and ACLF grade 3. Performance and safety were assessed through vital signs, clinical laboratory parameters and blood gas analyses. The SOFA Score-Standardized Mortality Ratio (SMR) served to evaluate patient outcomes in the absence of a control group. Results Participants, with a median age of 58 years, predominantly male (64%), exhibited a high requirement for mechanical ventilation (68%) and vasopressors (82%) with a median SOFA Score of 15. Notably, a median of 3 (IQR 2, 5) ADVOS sessions per patient were administered. Following the initial treatment, significant reductions were observed in bilirubin (-1.9 [CI 95% -1.3, -2.5]), creatinine (-0.5 [-0.4, -0.6]), and blood urea nitrogen (-13.1 mg/dL [-10.3, -16.0]) levels. Moreover, there were marked improvements in blood pH (7.34 vs. 7.41, p<0.001), HCO3- (19.4 vs. 24.6 mmol/l, p<0.001) and base excess (-5.6 vs. 0.2 mmol/l, p<0.001). The observed mortality rate (66%) was notably lower than the expected rate based on SOFA Score (84%), resulting in a SMR of 0.79 (95% CI: 0.66-0.93), with a calculated number needed to treat (NNT) of 5.8. Conclusions This study emphasizes the ADVOS system's efficacy in eliminating water-soluble and protein-bound substances and correcting acid-base imbalances across a diverse cohort with multiorgan failure. However, further validation through randomized controlled trials is warranted to solidify these findings.
Read moreLiposuktion bei postbariatrischen Patienten