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  1. Article ; Online: Detecting HLH in hematologic malignancies.

    La Rosée, Paul

    Blood

    2022  Volume 139, Issue 7, Page(s) 961–963

    MeSH term(s) Hematologic Neoplasms/complications ; Hematologic Neoplasms/diagnosis ; Hematologic Neoplasms/pathology ; Humans ; Lymphohistiocytosis, Hemophagocytic/pathology
    Language English
    Publishing date 2022-02-15
    Publishing country United States
    Document type Editorial ; Comment
    ZDB-ID 80069-7
    ISSN 1528-0020 ; 0006-4971
    ISSN (online) 1528-0020
    ISSN 0006-4971
    DOI 10.1182/blood.2021014675
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  2. Article ; Online: Die hämophagozytische Lymphohistiozytose bei kritisch kranken Patienten.

    Eichenauer, D A / Lachmann, G / La Rosée, P

    Medizinische Klinik, Intensivmedizin und Notfallmedizin

    2021  Volume 116, Issue 2, Page(s) 129–134

    Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammation syndrome. In adults, secondary HLH is mostly observed. HLH is often triggered by infections, malignancies or autoimmune disorders. However, HLH cases in association with immunotherapies have ...

    Title translation Hemophagocytic lymphohistiocytosis in critically ill patients.
    Abstract Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammation syndrome. In adults, secondary HLH is mostly observed. HLH is often triggered by infections, malignancies or autoimmune disorders. However, HLH cases in association with immunotherapies have been described recently. HLH in critically ill patients is often difficult to differentiate from sepsis. Both conditions can also be present at the same time. Early diagnosis and timely initiation of an adequate immunosuppressive therapy are essential for the further course and the prognosis of HLH. Therefore, HLH should represent a differential diagnosis in critically ill patients with persistent fever and additional symptoms (e.g. enlarged spleen, neurologic symptoms) or laboratory parameters (e.g. hyperferritinemia, cytopenia, increased transaminases) compatible with HLH. The diagnosis of HLH is made using the HLH-2004 criteria. The probability of the presence of HLH can be calculated using the HScore. High-dose corticosteroids represent the cornerstone of HLH treatment. Etoposide, immunoglobulins, anakinra or other drugs are added depending on the trigger. The course of HLH is influenced by the time of treatment initiation, the underlying trigger and the response to treatment. Generally, the prognosis of critically ill HLH patients is poor.
    MeSH term(s) Adult ; Critical Illness ; Diagnosis, Differential ; Humans ; Lymphohistiocytosis, Hemophagocytic/diagnosis ; Lymphohistiocytosis, Hemophagocytic/therapy ; Sepsis
    Language German
    Publishing date 2021-02-12
    Publishing country Germany
    Document type Journal Article ; Review
    ZDB-ID 2636018-4
    ISSN 2193-6226 ; 1435-1420 ; 0723-5003 ; 2193-6218 ; 0175-3851
    ISSN (online) 2193-6226 ; 1435-1420
    ISSN 0723-5003 ; 2193-6218 ; 0175-3851
    DOI 10.1007/s00063-021-00781-9
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  3. Article ; Online: Behandlung der hämophagozytischen Lymphohistiozytose bei Patienten auf der Intensivstation.

    Eichenauer, Dennis A / La Rosée, Paul

    Innere Medizin (Heidelberg, Germany)

    2023  Volume 64, Issue 10, Page(s) 955–960

    Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterized by hyperferritinemia. A differentiation is made between hereditary and acquired forms. In contrast to children, almost all cases in adult patients consist of acquired ... ...

    Title translation Treatment of hemophagocytic lymphohistiocytosis in patients in the intensive care unit.
    Abstract Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterized by hyperferritinemia. A differentiation is made between hereditary and acquired forms. In contrast to children, almost all cases in adult patients consist of acquired secondary HLH. Infections, malignancies and autoimmune diseases are frequent triggers of secondary HLH. More recently, cases of HLH have also been described in association with immunotherapy, e.g., when using chimeric antigen receptor (CAR) T‑cell treatment. In critically ill patients in the intensive care unit (ICU), sepsis represents the major differential diagnosis of HLH due to the frequently similar clinical presentation. Sometimes both sepsis and HLH are present at the same time. An early diagnosis and timely initiation of immunosuppressive treatment are essential for the further course and prognosis of HLH. Therefore, HLH should be considered as a possible diagnosis in critically ill patients with persistent fever and additional compatible symptoms (e.g., splenomegaly, neurological symptoms) or laboratory parameters (e.g., hyperferritinemia, cytopenia of two or three cell lines, increased transaminases). The diagnosis of HLH is made on the basis of the HLH-2004 criteria. The HScore can be used to estimate the probability of the presence of HLH. Corticosteroids given at high doses are the cornerstone of HLH treatment. Furthermore, immunoglobulins, etoposide, anakinra or ruxolitinib can complement treatment depending on the HLH trigger. The course of HLH depends on the timely initiation of treatment, the underlying trigger and the response to treatment. Despite progress in terms of diagnostics and targeted treatment, the prognosis of critically ill HLH patients is still poor.
    MeSH term(s) Adult ; Child ; Humans ; Lymphohistiocytosis, Hemophagocytic/diagnosis ; Critical Illness ; Hyperferritinemia ; Immunotherapy ; Intensive Care Units
    Language German
    Publishing date 2023-09-13
    Publishing country Germany
    Document type English Abstract ; Journal Article ; Review
    ISSN 2731-7099
    ISSN (online) 2731-7099
    DOI 10.1007/s00108-023-01584-0
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  4. Book: Therapie der chronischen myeloischen Leukämie nach Versagen der Erstlinientherapie

    La Rosée, Paul

    (Thieme-Refresher Onkologie ; 2014,3)

    2014  

    Author's details P. la Rosée
    Series title Thieme-Refresher Onkologie ; 2014,3
    Collection
    Language German
    Size S. R38 - R51
    Publisher Thieme
    Publishing place Stuttgart
    Publishing country Germany
    Document type Book
    HBZ-ID HT018631373
    Database Catalogue ZB MED Medicine, Health

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    Shelf markLoan statusLocation
    Zs A 6621 -2014,3- verfügbar in Königswinter Königswinter

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  5. Article ; Online: Rheumatische Krankheitserscheinungen bei hämatologischen Neoplasien.

    Kleinert, S / La Rosée, P / Krüger, K

    Zeitschrift fur Rheumatologie

    2018  Volume 76, Issue Suppl 2, Page(s) 38–45

    Abstract: Paraneoplastic syndromes in lymphatic or myeloid neoplasms can present with musculoskeletal symptoms, vasculitis-like or febrile symptoms. Hematologic diseases are also associated with rheumatic diseases whereas inflammatory rheumatic diseases are often ... ...

    Title translation Rheumatoid symptoms in patients with hematologic neoplasms.
    Abstract Paraneoplastic syndromes in lymphatic or myeloid neoplasms can present with musculoskeletal symptoms, vasculitis-like or febrile symptoms. Hematologic diseases are also associated with rheumatic diseases whereas inflammatory rheumatic diseases are often associated with an increased risk for lymphoproliferative disease. Atypical disease characteristics, lack of disease-specific antibodies or therapeutic response are red flags for diagnosing paraneoplastic or coexistent malignant diseases. New onset of systemic symptoms, worsening of general condition, night sweats or weight loss need to be considered during follow-up and differential diagnostics. This article focuses on musculoskeletal, vasculitis-like and systemic signs of lymphatic or myeloid neoplasms either because of coexistency, tumor association or paraneoplastic disease.
    MeSH term(s) Hematologic Neoplasms/complications ; Humans ; Paraneoplastic Syndromes ; Rheumatic Diseases/complications ; Vasculitis
    Language German
    Publishing date 2018-01-12
    Publishing country Germany
    Document type Journal Article ; Review
    ZDB-ID 124985-x
    ISSN 1435-1250 ; 0340-1855 ; 0301-6382
    ISSN (online) 1435-1250
    ISSN 0340-1855 ; 0301-6382
    DOI 10.1007/s00393-017-0338-9
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  6. Article: Die hämophagozytische Lymphohistiozytose bei kritisch kranken Patienten.

    Eichenauer, Dennis A / Lachmann, Gunnar / La Rosée, Paul

    Wiener klinisches Magazin : Beilage zur Wiener klinischen Wochenschrift

    2021  Volume 24, Issue 6, Page(s) 246–251

    Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammation syndrome. In adults, secondary HLH is mostly observed. HLH is often triggered by infections, malignancies or autoimmune disorders. However, HLH cases in association with immunotherapies have ...

    Title translation Hemophagocytic lymphohistiocytosis in critically ill patients.
    Abstract Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammation syndrome. In adults, secondary HLH is mostly observed. HLH is often triggered by infections, malignancies or autoimmune disorders. However, HLH cases in association with immunotherapies have been described recently. HLH in critically ill patients is often difficult to differentiate from sepsis. Both conditions can also be present at the same time. Early diagnosis and timely initiation of an adequate immunosuppressive therapy are essential for the further course and the prognosis of HLH. Therefore, HLH should represent a differential diagnosis in critically ill patients with persistent fever and additional symptoms (e.g. enlarged spleen, neurologic symptoms) or laboratory parameters (e.g. hyperferritinemia, cytopenia, increased transaminases) compatible with HLH. The diagnosis of HLH is made using the HLH-2004 criteria. The probability of the presence of HLH can be calculated using the HScore. High-dose corticosteroids represent the cornerstone of HLH treatment. Etoposide, immunoglobulins, anakinra or other drugs are added depending on the trigger. The course of HLH is influenced by the time of treatment initiation, the underlying trigger and the response to treatment. Generally, the prognosis of critically ill HLH patients is poor.
    Language German
    Publishing date 2021-10-21
    Publishing country Austria
    Document type English Abstract ; Journal Article
    ZDB-ID 2230871-4
    ISSN 1613-7817 ; 1869-1757 ; 1434-6869
    ISSN (online) 1613-7817
    ISSN 1869-1757 ; 1434-6869
    DOI 10.1007/s00740-021-00414-z
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    Zs.A 902 -Magazin-: Show issues Location:
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    Jg. 1995 - 2021: Lesesall (1.OG)
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  7. Article ; Online: Lymphoma-associated hemophagocytic lymphohistiocytosis (LA-HLH): a scoping review unveils clinical and diagnostic patterns of a lymphoma subgroup with poor prognosis.

    Knauft, Johanna / Schenk, Thomas / Ernst, Thomas / Schnetzke, Ulf / Hochhaus, Andreas / La Rosée, Paul / Birndt, Sebastian

    Leukemia

    2024  Volume 38, Issue 2, Page(s) 235–249

    Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome driven by pathologic activation of cytotoxic T-lymphocytes and macrophages. Despite advances in diagnostics and management, adult patients with lymphoma-associated HLH (LA- ... ...

    Abstract Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome driven by pathologic activation of cytotoxic T-lymphocytes and macrophages. Despite advances in diagnostics and management, adult patients with lymphoma-associated HLH (LA-HLH) harbor particularly poor prognosis and optimal treatment remains challenging. As systematic data on LA-HLH are scarce, we aimed to synthesize research evidence by thorough analysis of the published literature in PubMed (MEDLINE-database) within the context of a scoping review. Of 595 search results, 132 articles providing information on 542 patients were reviewed and analyzed. Median patient age was 60 years (range, 18-98) with male predominance (62.7%). B- and T-NHL were equally represented (45.6% and 45.2%), Hodgkin's lymphoma was reported in 8.9% of the cases. The majority of patients (91.6%) presented in Ann-Arbor-Stages III and IV, and bone marrow infiltration was observed in a significant proportion of patients (61.5%). Soluble CD25 levels were markedly elevated (median 10,000 U/ml), with levels beyond 10,000 U/ml indicating unfavorable prognosis for 30-day and overall survival. 66.8% of the patients died after median 5.1 months. LA-HLH remains a clinical challenge requiring specialized management. Timely diagnosis and appropriate lymphoma-specific treatment are of utmost importance to enhance patient outcomes.
    MeSH term(s) Adult ; Humans ; Male ; Adolescent ; Young Adult ; Middle Aged ; Aged ; Aged, 80 and over ; Female ; Lymphohistiocytosis, Hemophagocytic/diagnosis ; Lymphohistiocytosis, Hemophagocytic/etiology ; Lymphohistiocytosis, Hemophagocytic/pathology ; Lymphoma/complications ; Lymphoma/diagnosis ; Prognosis ; Macrophages/pathology ; Bone Marrow/pathology
    Language English
    Publishing date 2024-01-18
    Publishing country England
    Document type Journal Article ; Review ; Research Support, Non-U.S. Gov't
    ZDB-ID 807030-1
    ISSN 1476-5551 ; 0887-6924
    ISSN (online) 1476-5551
    ISSN 0887-6924
    DOI 10.1038/s41375-024-02135-8
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  8. Article ; Online: Alleviating the storm: ruxolitinib in HLH.

    La Rosée, Paul

    Blood

    2016  Volume 127, Issue 13, Page(s) 1626–1627

    Abstract: 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). ...

    Abstract 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).
    MeSH term(s) Animals ; Inflammation/prevention & control ; Janus Kinases/antagonists & inhibitors ; Lymphocyte Activation/drug effects ; Lymphohistiocytosis, Hemophagocytic/genetics ; Lymphohistiocytosis, Hemophagocytic/pathology ; Pyrazoles/pharmacology
    Chemical Substances Pyrazoles ; Janus Kinases (EC 2.7.10.2)
    Language English
    Publishing date 2016-03-30
    Publishing country United States
    Document type Journal Article ; Comment
    ZDB-ID 80069-7
    ISSN 1528-0020 ; 0006-4971
    ISSN (online) 1528-0020
    ISSN 0006-4971
    DOI 10.1182/blood-2016-02-697151
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    Zs.MO 364: Show issues

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  9. Book ; Online: Hämophagozytische Lymphohistiozytose (HLH)

    La Rosée, Paul / Beutel, Karin / Birndt, Sebastian / Ehl, Stephan / Girschikofsky, Michael / Lehmberg, Kai / Maschmeyer, Georg / Pachlopnik Schmid, Jana / Schenk, Thomas / Weber, Thomas / Janka-Schaub, Gritta

    Leitlinie : Empfehlungen der Fachgesellschaft zur Diagnostik und Therapie hämatologischer und onkologischer Erkrankungen

    (Onkopedia Leitlinien)

    2020  

    Abstract: Die Hämophagozytische Lymphohistiozytose (HLH) und das Makrophagenaktivierungssyndrom (MAS-HLH) sind hyperferritinämische Hyperinflammationssyndrome. Sie sind gekennzeichnet durch einen Zytokinsturm aberrant aktivierter Makrophagen und T-Zellen. Die ... ...

    Institution Deutsche Gesellschaft für Hämatologie und Medizinische Onkologie
    Österreichische Gesellschaft für Hämatologie & Medizinische Onkologie
    Schweizerische Gesellschaft für Medizinische Onkologie
    Schweizerische Gesellschaft für Hämatologie
    Gesellschaft für Pädiatrische Onkologie und Hämatologie
    Author's details DGHO - Deutsche Gesellschaft für Hämatologie und Medizinische Onkologie e.V., OeGHO - Österreichische Gesellschaft für Hämatologie & Medizinische Onkologie, SSMO/SSOM/SGMO - Schweizerische Gesellschaft für Medizinische Onkologie, SGH-SSH - Schweizerische Gesellschaft für Hämatologie/Société Suisse d'Hématologie, GPOH - Gesellschaft für Pädiatrische Onkologie und Hämatologie ; Autoren: Paul Graf La Rosée, Karin Beutel, Sebastian Birndt, Stephan Ehl, Michael Girschikofskiy, Kai Lehmberg, Georg Maschmeyer, Jana Pachlopnik Schmid, Thomas Schenk, Thomas Weber, Gritta Janka
    Series title Onkopedia Leitlinien
    Abstract Die Hämophagozytische Lymphohistiozytose (HLH) und das Makrophagenaktivierungssyndrom (MAS-HLH) sind hyperferritinämische Hyperinflammationssyndrome. Sie sind gekennzeichnet durch einen Zytokinsturm aberrant aktivierter Makrophagen und T-Zellen. Die namengebende Hämophagozytose in Knochenmark, Lymphknoten oder Milz ist nicht bei allen Patienten nachweisbar. Der primären (genetischen) Form, welche in der Regel bei Kindern anzutreffen ist, liegen Genmutationen zugrunde, welche zu einer veränderten Funktion zytotoxischer T- und NKZellen sowie einer gestörten Immunregulation führen. Erwachsene haben fast immer eine sekundäre (erworbene) HLH, die durch Infektionen, Malignome oder autoinflammatorische und autoimmunologische Krankheiten ausgelöst wird. Die Empfehlungen zu Diagnostik und Therapie wurden systematisch in der Pädiatrie durch prospektive Studien entwickelt. Sie werden in angepasster Form auch bei erwachsenen HLH-Patienten angewendet, sind hier jedoch nur kasuistisch validiert. Die Erkrankung ist lebensbedrohlich und macht eine rasche Diagnosestellung und Therapieeinleitung erforderlich. Das klinische Bild unterscheidet sich häufig kaum von einer Sepsis, wobei die klinische Ausprägung und der Verlauf heterogen sind. Die vorliegende Leitlinie gibt einen Überblick über Grundlagen und klinische Phänotypen des Syndroms und beschreibt die notwendige Diagnostik und differenzierte Therapiealgorithmen.
    Subject code 610
    Language German
    Size 1 Online-Ressource (25 Seiten), Diagramme
    Edition Stand: April 2020
    Publisher DGHO Deutsche Gesellschaft für Hämatologie und Medizinische Onkologie e.V
    Publishing place Berlin
    Publishing country Germany
    Document type Book ; Online
    Note Vorherige Autoren: Maximilian Christopeit, Jens Panse, Olaf Penack ; Open Access
    HBZ-ID HT020591650
    DOI 10.4126/FRL01-006423189
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  10. Article ; Online: Ruxolitinib in COVID-19 Hyperinflammation and Haematologic Malignancies.

    La Rosée, Felicitas / La Rosée, Paul

    Acta haematologica

    2020  Volume 144, Issue 3, Page(s) 246–249

    MeSH term(s) COVID-19 ; Hematologic Neoplasms/drug therapy ; Humans ; Pyrazoles/adverse effects ; SARS-CoV-2
    Chemical Substances Pyrazoles ; ruxolitinib (82S8X8XX8H)
    Keywords covid19
    Language English
    Publishing date 2020-08-20
    Publishing country Switzerland
    Document type Editorial ; Comment
    ZDB-ID 80008-9
    ISSN 1421-9662 ; 0001-5792
    ISSN (online) 1421-9662
    ISSN 0001-5792
    DOI 10.1159/000510770
    Database MEDical Literature Analysis and Retrieval System OnLINE

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