A5050515003- Autoimmune and Inflammatory Disorders Research
- Immune Cell Function and Interaction
- Parvovirus B19 Infection Studies
- Immunodeficiency and Autoimmune Disorders
- Adolescent and Pediatric Healthcare
- Family and Disability Support Research
- Blood disorders and treatments
- Acute Lymphoblastic Leukemia research
- Chronic Lymphocytic Leukemia Research
- Kawasaki Disease and Coronary Complications
- Neurogenetic and Muscular Disorders Research
- Hematopoietic Stem Cell Transplantation
- Platelet Disorders and Treatments
- Lysosomal Storage Disorders Research
- Cytomegalovirus and herpesvirus research
- Viral-associated cancers and disorders
- Histiocytic Disorders and Treatments
- Virus-based gene therapy research
- Lymphoma Diagnosis and Treatment
- RNA modifications and cancer
- Acute Myeloid Leukemia Research
- Congenital Ear and Nasal Anomalies
- Childhood Cancer Survivors' Quality of Life
- Inflammasome and immune disorders
- Virology and Viral Diseases
University Medical Center Hamburg-Eppendorf
2016-2025
Universität Hamburg
2016-2025
Rockefeller University
2024
St. Jude Children's Research Hospital
2021
Kinderkrebs-Zentrum Hamburg
2009-2020
Society of Paediatric Oncology and Haematology
2016-2019
Martini-Klinik
2017
Infektionsmedizinisches Centrum Hamburg
2014
University Hospital and Clinics
2013
University Medical Center
2013
Objective To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). Methods A multistep process, based on a combination expert consensus and analysis real patient data, was conducted. panel 28 experts first asked to classify 428 profiles as having or not MAS, clinical laboratory features at time disease onset. The comprised 161 JIA–associated MAS 267 condition that could potentially be confused (active...
To describe the clinical, laboratory, and histopathologic features, current treatment, outcome of patients with macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (JIA).In this multinational, multicenter study, pediatric rheumatologists hemato-oncologists entered patient data collected retrospectively into a web-based database.A total 362 patients, 22% whom had MAS at onset JIA, were included in study by 95 investigators from 33 countries. The most...
To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). A multistep process, based on a combination expert consensus and analysis real patient data, was conducted. panel 28 experts first asked to classify 428 profiles as having or not MAS, clinical laboratory features at time disease onset. The comprised 161 JIA—associated MAS 267 condition that could potentially be confused (active JIA without evidence...
Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome defined by clinical and laboratory criteria. Current criteria were created to identify patients with familial hemophagocytic lmyphohistiocytosis in immediate need of immunosuppressive therapy. However, these also infection-associated inflammatory states lacking genetic defects typically predisposing lymphohistiocytosis. These include those primary immunodeficiencies, whom the pathogenesis may be distinctive aggressive...
The hyperinflammatory syndrome hemophagocytic lymphohistiocytosis can occur in the context of malignancies. Malignancy-triggered should be regarded separately from during chemotherapeutic treatment, which is frequently associated with an infectious trigger. substantial overlap between features neoplasms makes its identification difficult when it occurs malignant conditions. To facilitate recognition and diagnostic workup, provide guidance regarding treatment malignancy-associated...
Summary Haemophagocytic lymphohistiocytosis ( HLH ) in the context of malignancy is mainly considered a challenge adult haematology. While this association also observed children, little known regarding inciting factors, appropriate treatment and prognosis. We retrospectively analysed 29 paediatric adolescent patients for presenting features, type neoplasm or preceding chemotherapy, outcome. was triggered by (M‐ 21 patients, most whom had T‐ n = 12) B‐cell neoplasms 7), with Epstein–Barr...
Abstract Purpose Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome emerging from deregulated immune response due to various triggers. In adults, systematic data are sparse, which why recommendations on diagnosis and management have been adopted pediatric guidelines. A nationwide clinical registry with associated consulting service as collaborative initiative of HLH-specialized pediatricians hematologists was initiated better characterize HLH in adults. Methods...
Objective To identify which laboratory tests that change over time are most valuable for the timely diagnosis of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA). Methods A multistep process, based on a combination expert consensus and analysis real patient data, was conducted. panel experts first asked to evaluate 115 profiles patients with MAS, included values at pre-MAS visit MAS onset, in between two points. The were choose 5 important rank...
Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome that often requires critical care support and remains difficult to diagnose. These guidelines are meant aid in the early recognition, diagnosis, supportive care, treatment of patients with hemophagocytic ICUs.The literature searches were performed PubMed (MEDLINE).Keywords medical subject headings terms for search included "macrophage activation syndrome," lymphohistiocytosis," "hemophagocytic syndrome."The Histiocyte Society...
T-cell receptor excision circle (TREC)-based newborn screening (NBS) for severe combined immunodeficiencies (SCID) was introduced in Germany August 2019.
Abstract Current hemophagocytic lymphohistiocytosis 2004 (HLH-2004)–based diagnostic criteria for familial (FHL) are based on expert opinion. Here, we performed a case-control study to test and possibly improve these criteria. We also developed 2 complementary opinion–based strategies FHL in patients with signs/symptoms suggestive of HLH, genetic cellular cytotoxicity assays. The cases (N = 366) were children aged <16 years verified and/or (n 341) or Griscelli syndrome type 25); 276...
Hyperferritinemia is a hallmark of hemophagocytic lymphohistiocytosis (HLH). In the HLH-2004 criteria, ferritin value >500 µg/L considered positive. However, this level was not determined based on evidence. We compared 123 patients with HLH and 320 other hyperferritinemic conditions, calculated receiver-operating characteristic, sensitivity specificity for different values. At 2,000 trade-off reached at 70% 68%. If familial virus-associated acquired are analyzed separately, similar level....
Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening disturbance of immunoregulation. HLH comprises primary and acquired forms with different disease severity. A large proportion deaths occur early into treatment. We investigated association death for laboratory clinical parameters before the start 2 weeks therapy.A total 232 children from Scandinavia, Germany or Italy, fulfilling diagnostic criteria and/or familial HLH-causing mutations, receiving treatment 1994-2008 were...