A5102734129- Glycosylation and Glycoproteins Research
- Histiocytic Disorders and Treatments
- Autoimmune and Inflammatory Disorders Research
- Immune Cell Function and Interaction
- Parvovirus B19 Infection Studies
- Neuroblastoma Research and Treatments
- Viral-associated cancers and disorders
- Erythrocyte Function and Pathophysiology
- Vascular Malformations and Hemangiomas
- Immunotherapy and Immune Responses
- Cancer, Hypoxia, and Metabolism
- Hematopoietic Stem Cell Transplantation
- Genital Health and Disease
- Carbohydrate Chemistry and Synthesis
- Sphingolipid Metabolism and Signaling
- Mast cells and histamine
- Blood disorders and treatments
- Immune Response and Inflammation
- Tumors and Oncological Cases
- T-cell and B-cell Immunology
- Neuroendocrine Tumor Research Advances
- Diabetes and associated disorders
- Family and Disability Support Research
- Cytomegalovirus and herpesvirus research
- Galectins and Cancer Biology
Children's National
2010-2024
George Washington University
1994-2024
Children’s Institute
2005-2017
Washington University Medical Center
2010
Medical Research Council
2005
Oregon Health & Science University
2001
Johannes Gutenberg University Mainz
2001
Uppsala University Hospital
2001
University of Pavia
2001
St Anna Children's Hospital
2001
In HLH-94, the first prospective international treatment study for hemophagocytic lymphohistiocytosis (HLH), diagnosis was based on five criteria (fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, and hemophagocytosis). HLH-2004 three additional are introduced; low/absent NK-cell-activity, hyperferritinemia, high-soluble interleukin-2-receptor levels. Altogether of these eight must be fulfilled, unless family history or molecular is consistent with HLH....
Abstract Treatment of Langerhans cell histiocytosis (LCH) remains problematic. To test the hypothesis that rapid initiation and longterm continuation chemotherapy can improve survival reduce recurrence late consequences disseminated LCH, we have completed a prospective clinical trial (DAL HX‐83). One hundred six newly diagnosed patients were stratified into three risk groups (A: multifocal bone disease [n = 28]; B: soft tissue involvement without organ dysfunction 57]; C: 21]). All received...
Diabetes insipidus (DI) is the most frequent central nervous system (CNS)-related permanent consequence in Langerhans cell histiocytosis (LCH), which mostly requires life-long hormone replacement therapy. In an attempt to define population at risk for DI, 1,741 patients with LCH registered on trials DALHX 83 and 90, I II were studied.Overall 212 of (12%) was reported have DI. 102 (6%) DI present diagnosis LCH. One thousand one hundred eighty three 1,539 without had follow up information. ten...
Summary Haemophagocytic lymphohistiocytosis (HLH) poses major therapeutic challenges, and the primary inherited form, familial haemophagocytic (FHL), is usually fatal. We evaluated, including Cox regression analysis, survival in 86 children (29 familial) that received HLH‐94‐therapy (etoposide, dexamethasone, ciclosporin) followed by allogeneic stem cell transplantation (SCT) between 1995 2000. The overall estimated 3‐year‐survival post‐SCT was 64% [confidence interval (CI) = ±10%] ( n 86);...
The role of tumor cell membrane gangliosides in formation was probed using a series cloned murine AKR lymphoma lines. Tumor directly related to high expression and shedding gangliosides. In vivo, as little 1 pmol purified total highly tumorigenic cells, injected intradermally with poorly cells (which lacked did not shed gangliosides), markedly increased the tumorigenicity these syngeneic normal mice. Thus, by are previously unrecognized, extremely potent enhancer vivo.
Abstract Substantial concentrations of the cell‐surface glycosphingolipid, disialoganglioside G D2 , are uniformly present in human neuroblastoma tumors. This ganglioside can also be detected plasma patients with by direct thin‐layer chromatographic analysis. Among 32 all clinical stages studied prior to initiation treatment, 27 (84%) showed measurably elevated (≥50 pmol/ml). The mean level (545 ± 108 pmol/ml) was more than 50 times normal concentration ≤ 10 pmol/ml. Circulating not related,...
A prospective phase II Histiocyte Society study, LCH-S-98, evaluated the efficacy of 2-chlorodeoxyadenosine (2-CdA) monotherapy as salvage therapy in Langerhans cell histiocytosis (LCH).Patients with poor and intermediate risk LCH not responsive to initial patients low-risk chronic recurrent were for response survival after treatment 2-6 courses 2-CdA.Forty-six (55%) had involvement organs; lung, liver, spleen, or hematopoetic system (RO+), 37 (45%) RO-. Twenty-two percent RO+ a good while...
Gangliosides are potent inhibitors of lymphoproliferative responses. Selectively greater inhibitory effects gangliosides on antigen-induced (vs. mitogen-induced) proliferation have been documented; e.g., 50 nmol highly purified bovine brain (BBG)/ml caused than or equal to 87% inhibition proliferative responses human peripheral blood mononuclear cells (PBMC) three soluble specific antigens (Candida, streptokinase-streptodornase, and tetanus toxoid) vs. less 37% nonspecific mitogens...
Abstract An international randomized trial in Langerhans cell histiocytosis (LCH) has been initiated by the Histiocyte Society. This report reviews rationale, design, and progress of LCH‐I, which compares etoposide (VP‐16) vinblastine treatment disseminated LCH. Data on risk etoposide‐associated (therapy‐induced) malignancy, setting histiocytosis, are reviewed. The available evidence leads to recommendation that study LCH should be continued. © 1994 Wiley‐Liss, Inc.