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HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis
TLDR
HLH‐2004 chemo‐immunotherapy includes etoposide, dexamethasone, cyclosporine A upfront and, in selected patients, intrathecal therapy with methotrexate and corticosteroids, and subsequent hematopoietic stem cell transplantation is recommended for patients with familial disease or molecular diagnosis, and patients with severe and persistent, or reactivated, disease. Expand
Outcome of treatment in children with Philadelphia chromosome-positive acute lymphoblastic leukemia.
TLDR
Transplantation of bone marrow from an HLA-matched related donor is superior to other types of transplantation and to intensive chemotherapy alone in prolonging initial complete remissions and protecting patients from relapse or other adverse events. Expand
Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net
TLDR
The first recommendations for management of adult patients with LCH, a number of experts in this field cooperated to develop, were developed according to the clinical relevance focusing on diagnostic work up, therapy, and follow up. Expand
Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation.
TLDR
HLH-94 is very effective, allowing BMT in most patients, and survival of children with HLH has been greatly improved. Expand
Central diabetes insipidus in children and young adults.
TLDR
Most children and young adults with acquired central diabetes insipidus have abnormal findings on MRI scans of the head, which may change over time, and at least half have anterior pituitary hormone deficiencies during follow-up. Expand
Chronic myelomonocytic leukemia in childhood. A retrospective analysis of 110 cases.
TLDR
Platelet count, age, and hemoglobin F at diagnosis were the main predicting factors for the length of survival in the non-BMT group, and there is a strong need for a broad agreement on nomenclature in children with myelodysplastic syndromes (MDS). Expand
Contemporary classification of histiocytic disorders
Pathologists and pediatric hematologist/ oncologists of the World Health Organization's Committee on Histiocytic/Reticulum Cell Proliferations and the Reclassification Working Group of the HistiocyteExpand
Langerhans cell histiocytosis reveals a new IL-17A–dependent pathway of dendritic cell fusion
TLDR
It is found that there were high serum levels of IL-17A during active LCH and unexpected IL- 17A synthesis by dendritic cells (DCs), the major cell type in LCH lesions, and an IL–17A–dependent pathway for DC fusion, which was highly potentiated by IFN-γ and led to giant cells expressing three major tissue-destructive enzymes. Expand
NK-dependent DC maturation is mediated by TNFalpha and IFNgamma released upon engagement of the NKp30 triggering receptor.
TLDR
It is shown that also the NK-mediated induction of DC maturation is dependent on NKp30, and the ability to promote maturation was essentially confined to NK cells expressing the killer immunoglobulin-like receptor-negative (KIR-) NKG2A(dull) phenotype. Expand
Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society.
TLDR
Langerhans cell histiocytosis in adults is most often a multisystem disease with the highest mortality seen in patients with isolated pulmonary involvement, and should be included in the differential diagnosis of disseminated or localised disease of the bone, skin and mucosa, as well as the lung and the endocrine and central nervous system, regardless of the age of the patient. Expand
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