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  • 1
    Language: English
    In: Blood, 05 November 2015, Vol.126(19), pp.2169-71
    Keywords: Antineoplastic Combined Chemotherapy Protocols -- Administration & Dosage ; Lymphohistiocytosis, Hemophagocytic -- Mortality ; Salvage Therapy -- Methods
    ISSN: 00064971
    E-ISSN: 1528-0020
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  • 2
    Language: English
    In: Blood, 31 March 2016, Vol.127(13), pp.1626-7
    Description: In this issue of Blood, Das and colleagues report their results on the use of the Janus kinase 1/2 (JAK1/2) inhibitor ruxolitinib in murine models of hemophagocytic lymphohistiocytosis (HLH), and the HLH-sibling macrophage activation syndrome (MAS).
    Keywords: Inflammation -- Prevention & Control ; Janus Kinases -- Antagonists & Inhibitors ; Lymphocyte Activation -- Drug Effects ; Lymphohistiocytosis, Hemophagocytic -- Genetics ; Pyrazoles -- Pharmacology
    ISSN: 00064971
    E-ISSN: 1528-0020
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  • 3
    Language: English
    In: LaboratoriumsMedizin / Journal of Laboratory Medicine, Sept, 2013, Vol.37(5), p.217(9)
    Description: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by genetic and acquired defects of the molecular machinery, which regulate the cellular immune synapse. Rapid recognition of symptoms resembling HLH, and a targeted diagnostic approach are essential in improving outcome. The condition is associated with a mortality rate between 40% and 70%. For the clinician, the most important step towards diagnosing HLH is to include it in the list of potential differential diagnoses. The leading triad of symptoms consists of prolonged fever of unknown origin, hepatosplenomegaly, and bi- or pancytopeniae. A known family history or known gene mutations require rapid confirmatory testing, which facilitates the initiation of a life saving risk-adapted treatment that includes stem cell transplantation. In adults with de-novo infection with Epstein-Barr virus, a broad diagnostic approach is required to identify potential late-onset hereditary HLH. HLH is not a diagnosis, per se, but represents a common terminal pathway of diseases with the ability to trigger HLH. Such diseases include infections, malignant disorders, autoimmune or autoinflammatory diseases, and iatrogenic triggers such as immunosuppressive treatment or stem cell transplantation itself. This broad spectrum of HLH pathogenesis must be considered in order to achieve rapid diagnosis, which is a prerequisite for the initiation of rationale treatment. Keywords: hemophagocytic lymphohistiocytosis (HLH); macrophage activation syndrome (MAS); hemophagocytosis. Die hamophagozytische Lymphohistiozytose (HLH) ist ein HyperinflammationsSyndrom, welchem neben genetischen Defekten insbesondere in Genen der die Immunsynapse regulierenden Proteine auch erworbene Defekte der effektiven Pathogen-Elimination zugrunde liegen. Das rasche Erkennen und zielgerichtete Diagnostizieren einer HLH ist bei weiterhin hoher Mortalitatsrate zwischen 40%-70% essentiell, um Therapieverbesserungen zu erreichen. Hierfur ist der wichtigste Schritt fur den Kliniker, an eine HLH zu denken. Prolongiertes Fieber unklarer Genese, eine Hepatosplenomegalie und eine Bi-oder Panzytopenie sind die fuhrende Symptomentrias. Bei bekannter Familienanamnese oder bekanntem Gendefekt sind rasche bestatigende Untersuchungen einzuleiten, um die haufig notwendige Stammzelltransplantation nicht zu verzogern. Insbesondere bei Erwachsenen, bei denen auch genetische Defekte mit verzogerter Manifestation vorliegen konnen (v.a. bei de novo EBV-Infektion), muss eine breite Diagnostik zur Ursachenforschung einer HLH angestrengt werden. Die HLH ist keine eigenstandige Erkrankung. Sie ist gemeinsame Endstrecke eines Immundefekts, welcher genetisch bedingt, oder durch infektiose, autoimmune, autoinflammatorische, maligne oder auch iatrogene Trigger (Immunsuppression, Stammzelltransplantation) erworben werden kann. Diesem breiten Spektrum der Pathogenese der HLH muss die labormedizinische Diagnostik Rechnung tragen, um dem Kliniker sehr zeitnah die klinisch zu stellende Verdachtsdiagnose zu erharten und schnellstmoglich die Therapie einleiten zu konnen. Schlusselworter: Hamophagozytische Lymphohistiozytose (HLH); Makrophagenaktivierungssyndrom (MAS); Hamophagozytose.
    ISSN: 0342-3026
    Source: Cengage Learning, Inc.
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  • 4
    Language: English
    In: Hematology. American Society of Hematology. Education Program, 2015, Vol.2015, pp.190-6
    Description: Treatment of hemophagocytic lymphohistiocytosis (HLH) has been developed primarily in pediatric centers, where familial HLH (FHL) is the leading cause of HLH in newborns and toddlers. The Histiocyte Society Study Group for HLH developed the HLH-94 and HLH-2004 treatment protocols, and these are frequently also used by centers treating HLH in adults (aHLH). These protocols contain etoposide, dexamethasone, and cyclosporine A; these agents all have strong activity against proliferation of cytotoxic T/NK-cells and macrophages, as well as inhibitory activity against the cytokine storm that induces, and maintains HLH. In children with predominantly hereditary disease, the HLH-94 protocol can be regarded as a "one size fits all" algorithm. HLH in adults is a much more heterogeneous syndrome requiring a more individualized approach depending on the underlying trigger, disease severity and course, as well as genetic background. Additionally, treatment in adults usually needs to be modified in the face of the preceding disease history and comorbidities. Interdisciplinary patient care with rheumatologists, gastroenterologists, neurologists, pediatricians, the transplant team, and pathologists is a prerequisite to successful treatment. The preferred approach should reflect a disease- and risk-adapted treatment that includes rigorous supportive care with continuous reassessment of sequential therapeutic measures. It should be recognized that the algorithm of HLH treatment in adults is based more on expert opinion than on extensive scientific evidence.
    Keywords: Lymphohistiocytosis, Hemophagocytic -- Therapy
    E-ISSN: 1520-4383
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  • 5
    Language: German
    In: DMW - Deutsche Medizinische Wochenschrift, 2016, Vol.141(16), pp.1141-1143
    Description: Die Hämophagozytische Lymphohistiozytose (HLH) ist ein schweres Hyperinflammationssyndrom, das bei Kindern durch validierte Diagnosekriterien und den Nachweis spezifischer Mutationen in immunrelevanten Genen definiert ist. Die erworbene HLH des Erwachsenen (adulte HLH, aHLH) ist im Gegensatz dazu aufgrund der Heterogenität der Trigger (Infektionen, maligne Erkrankungen und Autoimmunopathien) und genetischen Prädispositionen in Klinik und Therapie sehr variabel. Es besteht sowohl die Gefahr der Unter- als auch der Übertherapie. Wegen der Unsicherheit in der Diagnosestellung der aHLH kommt es nicht selten zu Diagnose- und Therapieverzögerung. Der vorliegende Artikel stellt den aktuellen Stand und neue Entwicklungen hinsichtlich Epidemiologie, Pathophysiologie, Diagnose und Therapie der aHLH dar.
    Keywords: Hämatologie und Onkologie ; Hämophagozytische lymphohistiozytose ; Makrophagenaktivierungssyndrom ; Hemophagocytic lymphohistiocytosis ; Macrophage activation syndrome
    ISSN: 0012-0472
    E-ISSN: 1439-4413
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  • 6
    Language: English
    In: Laryngoscope, February 2013, Vol.123(2), pp.362-365
    Description: Hemophagocytic lymphohistiocytosis (HLH) is a rare, severe, and often fatal disorder. Its hereditary and sporadic form can present as a significant diagnostic challenge to the otolaryngologist. This report describes two fatal cases of adult patients with HLH initially presented as infectious mononucleosis to an otorhinolaryngologist. The clinical presentation, serological and histological features, and management are discussed.
    Keywords: Epstein‐Barr Virus ; Tonsillitis ; Hemophagocytic Lymphohistiocytosis ; Sh2d1a Gene ; Level Of Evidence: 4
    ISSN: 0023-852X
    E-ISSN: 1531-4995
    Source: John Wiley & Sons, Inc.
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  • 7
    Article
    Article
    Language: English
    In: Karger Kompass Onkologie, 2/9/2018, Vol.5(1), pp.10-10
    ISSN: 2296-5416
    E-ISSN: 2296-5386
    Source: S. Karger AG (Via CrossRef)
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  • 8
    Language: English
    In: Karger Kompass Onkologie, 2/9/2018, Vol.5(1), pp.6-9
    ISSN: 2296-5416
    E-ISSN: 2296-5386
    Source: S. Karger AG (Via CrossRef)
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  • 9
    In: Current Opinion in Hematology, 2010, Vol.17(2), pp.91-96
    Description: PURPOSE OF REVIEW: The molecular pathogenesis of resistance against tyrosine kinase inhibitors imatinib, nilotinib, or dasatinib in patients treated for chronic myeloid leukemia is best understood based on mutations within the ABL-kinase domain. However, in about 50% of patients, clinical resistance so far cannot be linked to known mutations. Mutation-independent resistance development is imparted by a multifactorial array of mechanisms. The purpose of this review is to summarize recent publications on molecular mechanisms that govern the development of clinical resistance.Studies on the second-line inhibitors dasatinib and nilotinib addressing clinical efficacy in the presence of preexisting kinase mutations have largely confirmed the in-vitro prediction. With regard to mutation-independent resistance, new insights into the multifactorial resistance regulation were gained. Preclinical and clinical findings have revitalized the interest in interferon-α as a potentially useful adjunct for tyrosine kinase inhibitor-based treatment. The dogma of continuous kinase inhibition necessary for optimal efficacy has been challenged by clinical and preclinical data. SUMMARY: Elucidation of the complexity of resistance development most likely will help to preempt evolution of resistant disease. Clinical studies now focus on dose modifications, drug scheduling, optimized inhibitors, and drug combinations aiming to prevent resistance development.
    Keywords: Drug Resistance, Neoplasm ; Enzyme Inhibitors -- Therapeutic Use ; Leukemia, Myelogenous, Chronic, BCR-Abl Positive -- Drug Therapy ; Piperazines -- Therapeutic Use ; Protein-Tyrosine Kinases -- Antagonists & Inhibitors ; Pyrimidines -- Therapeutic Use;
    ISSN: 1065-6251
    E-ISSN: 15317048
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  • 10
    Language: English
    In: Annals of Hematology, 2013, Vol.92(6), pp.825-830
    Description: Acquired severe aplastic anaemia (AA) is a serious condition caused by immune-triggered bone marrow failure. For patients not eligible for bone marrow transplantation, treatment of choice is immunosuppression by a combined treatment with antithymocyte globulin (ATG) and cyclosporine. The debate on treatment optimization in AA is focused on conflicting data regarding ATG preparations from horse (h-ATG) versus rabbit (r-ATG), recently favouring h-ATG. H-ATG has been withdrawn from the European market in 2007. Reimbursement for imported preparations from outside Europe is frequently denied in negotiations with statutory health insurance companies. This raises the question of whether h-ATG is cost effective and a sensible investment with regard to healthcare budgets as well as patient health. We modelled the cost effectiveness of r-ATG versus h-ATG based on a recent randomized trial and cost data provided by the hospital pharmacy of Jena University Hospital. We calculated the amount of life years gained and the average incremental costs per life year gained when comparing h-ATG and r-ATG. Our calculations revealed average incremental costs per life year gained of €11,033.80 for the examined patient population treated with h-ATG when compared to r-ATG. Assuming a cost effectiveness threshold of €25,000–35,000 per life year gained, our calculations demonstrate cost effectiveness of h-ATG as compared to r-ATG.
    Keywords: Economics ; h-ATG ; r-ATG ; Aplastic anaemia
    ISSN: 0939-5555
    E-ISSN: 1432-0584
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