Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz

Logo MUG-Forschungsportal

Gewählte Publikation:

SHR Neuro Krebs Kardio Lipid

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; Inborn Errors Working Party of the EBMT.
The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis.
Haematologica. 2015; 100(7):978-988 [OPEN ACCESS]
Web of Science PubMed PUBMED Central FullText FullText_MUG


Autor/innen der Med Uni Graz:
Seidel Markus

Dimensions Citations:

Plum Analytics:
Number of Figures: 2
| |
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/μL 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. Copyright© Ferrata Storti Foundation.
Find related publications in this database (using NLM MeSH Indexing)
Adolescent -
Adult -
Bacterial Infections - complications
Bacterial Infections - drug therapy
Bacterial Infections - immunology
Child -
Child, Preschool -
Diagnosis, Differential -
Diagnosis, Differential -
Female -
Humans -
Immunoglobulins, Intravenous - therapeutic use
Immunologic Deficiency Syndromes - complications
Immunologic Deficiency Syndromes - diagnosis
Immunologic Deficiency Syndromes - drug therapy
Immunologic Deficiency Syndromes - immunology
Immunologic Factors - therapeutic use
Infant -
Infant, Newborn -
Killer Cells, Natural - drug effects
Killer Cells, Natural - immunology
Killer Cells, Natural - pathology
Leishmaniasis - complications
Leishmaniasis - drug therapy
Leishmaniasis - immunology
Lymphohistiocytosis, Hemophagocytic - diagnosis
Lymphohistiocytosis, Hemophagocytic - drug therapy
Lymphohistiocytosis, Hemophagocytic - immunology
Lymphohistiocytosis, Hemophagocytic - pathology
Lymphoproliferative Disorders - complications
Lymphoproliferative Disorders - diagnosis
Lymphoproliferative Disorders - drug therapy
Lymphoproliferative Disorders - immunology
Male -
Mycoses - complications
Mycoses - drug therapy
Mycoses - immunology
Opportunistic Infections - complications
Opportunistic Infections - drug therapy
Opportunistic Infections - immunology
Registries -
Steroids - therapeutic use
T-Lymphocytes - drug effects
T-Lymphocytes - immunology
T-Lymphocytes - pathology
Terminology as Topic -
Virus Diseases - complications
Virus Diseases - drug therapy
Virus Diseases - immunology

© Med Uni Graz Impressum