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10.3324/haematol.2014.121608 http://scihub22266oqcxt.onion/10.3324/haematol.2014.121608 C4486233!4486233 !26022711     free     free     free Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=26022711 &cmd=llinks): Failed to open stream: HTTP request failed! 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The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis |pdf=|usr=}}{{26022711 }} gab.com Text The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis JO: Haematologica http://www.kidney.de/pget.php?&tw=1&pmid=26022711 #FOAvip 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 criteria also identify patients with infection-associated hemophagocytic inflammatory states lacking genetic defects typically predisposing to hemophagocytic lymphohistiocytosis. These patients include those with primary immunodeficiencies, in whom the pathogenesis of the inflammatory syndrome may be distinctive and aggressive immunosuppression is contraindicated. To better characterize hemophagocytic inflammation associated with immunodeficiencies, we combined an international survey with a literature search and identified 63 patients with primary immunodeficiencies other than cytotoxicity defects or X-linked lymphoproliferative disorders, presenting with conditions fulfilling current criteria for hemophagocytic lymphohistiocytosis. Twelve patients had severe combined immunodeficiency with <100/?L T cells, 18 had partial T-cell deficiencies; episodes of hemophagocytic lymphohistiocytosis were mostly associated with viral infections. Twenty-two patients had chronic granulomatous disease with hemophagocytic episodes mainly associated with bacterial infections. Compared to patients with cytotoxicity defects, patients with T-cell deficiencies had lower levels of soluble CD25 and higher ferritin concentrations. Other criteria for hemophagocytoc lymphohistiocytosis were not discriminative. Thus: (i) a hemophagocytic inflammatory syndrome fulfilling criteria for hemophagocytic lymphohistiocytosis can be the initial manifestation of primary immunodeficiencies; (ii) this syndrome can develop despite severe deficiency of T and NK cells, implying that the pathophysiology is distinct and not appropriately described as "lympho"-histiocytosis in these patients; and (iii) current criteria for hemophagocytoc lymphohistiocytosis are insufficient to differentiate hemophagocytic inflammatory syndromes with different pathogeneses. This is important because of implications for therapy, in particular for protocols targeting T cells. Twit Text FOAVip The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis ????Haematologica http://www.kidney.de/pget.php?&tw=1&pmid=26022711 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=26022711 #FOAvip English Wikipedia <ref>{{Cite pmid|26022711 }}</ref> The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis #MMPMID26022711 Bode SF ; Ammann S ; Al-Herz W ; Bataneant M ; Dvorak CC ; Gehring S ; Gennery A ; Gilmour KC ; Gonzalez-Granado LI ; Groß-Wieltsch U ; Ifversen M ; Lingman-Framme J ; Matthes-Martin S ; Mesters R ; Meyts I ; van Montfrans JM ; Pachlopnik Schmid J ; Pai SY ; Soler-Palacin P ; Schuermann U ; Schuster V ; Seidel MG ; Speckmann C ; Stepensky P ; Sykora KW ; Tesi B ; Vraetz T ; Waruiru C ; Bryceson YT ; Moshous D ; Lehmberg K ; Jordan MB ; Ehl S Haematologica 2015[Jul]; 100 (7 ): 978-88 PMID26022711 show ga 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 criteria also identify patients with infection-associated hemophagocytic inflammatory states lacking genetic defects typically predisposing to hemophagocytic lymphohistiocytosis. These patients include those with primary immunodeficiencies, in whom the pathogenesis of the inflammatory syndrome may be distinctive and aggressive immunosuppression is contraindicated. To better characterize hemophagocytic inflammation associated with immunodeficiencies, we combined an international survey with a literature search and identified 63 patients with primary immunodeficiencies other than cytotoxicity defects or X-linked lymphoproliferative disorders, presenting with conditions fulfilling current criteria for hemophagocytic lymphohistiocytosis. Twelve patients had severe combined immunodeficiency with <100/?L T cells, 18 had partial T-cell deficiencies; episodes of hemophagocytic lymphohistiocytosis were mostly associated with viral infections. Twenty-two patients had chronic granulomatous disease with hemophagocytic episodes mainly associated with bacterial infections. Compared to patients with cytotoxicity defects, patients with T-cell deficiencies had lower levels of soluble CD25 and higher ferritin concentrations. Other criteria for hemophagocytoc lymphohistiocytosis were not discriminative. Thus: (i) a hemophagocytic inflammatory syndrome fulfilling criteria for hemophagocytic lymphohistiocytosis can be the initial manifestation of primary immunodeficiencies; (ii) this syndrome can develop despite severe deficiency of T and NK cells, implying that the pathophysiology is distinct and not appropriately described as "lympho"-histiocytosis in these patients; and (iii) current criteria for hemophagocytoc lymphohistiocytosis are insufficient to differentiate hemophagocytic inflammatory syndromes with different pathogeneses. This is important because of implications for therapy, in particular for protocols targeting T cells. |*Registries [MESH] |Adolescent [MESH] |Adult [MESH] |Bacterial Infections/complications/drug therapy/immunology [MESH] |Child [MESH] |Child, Preschool [MESH] |Diagnosis, Differential [MESH] |Europe [MESH] |Female [MESH] |Humans [MESH] |Immunoglobulins, Intravenous/therapeutic use [MESH] |Immunologic Deficiency Syndromes/complications/*diagnosis/drug therapy/immunology [MESH] |Immunologic Factors/therapeutic use [MESH] |Infant [MESH] |Infant, Newborn [MESH] |Killer Cells, Natural/drug effects/immunology/pathology [MESH] |Leishmaniasis/complications/drug therapy/immunology [MESH] |Lymphohistiocytosis, Hemophagocytic/*diagnosis/drug therapy/immunology/pathology [MESH] |Lymphoproliferative Disorders/complications/*diagnosis/drug therapy/immunology [MESH] |Male [MESH] |Mycoses/complications/drug therapy/immunology [MESH] |Opportunistic Infections/complications/drug therapy/immunology [MESH] |Steroids/therapeutic use [MESH] |T-Lymphocytes/drug effects/immunology/pathology [MESH] |Terminology as Topic [MESH]The syndrome of hemophagocytic lymphohistiocytosis in primary immunode(epmc).pdf DeepDyve Pubget Overpricing