$Systemic and Nodular Hyperinflammation in a Patient with Refractory Familial Hemophagocytic Lymphohistiocytosis 2$

Systemic and Nodular Hyperinflammation in a Patient with Refractory Familial Hemophagocytic Lymphohistiocytosis 2

Familial hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome resulting from defective cytotoxicity. A previously healthy 3-month-old female presented with fever, irritability, abdominal distention, and tachypnea. She ultimately met all eight HLH-2004 diagnostic...

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Veröffentlicht in:	Journal of clinical immunology 2021-07, Vol.41 (5), p.987-991
Hauptverfasser:	Segal, Julia E., Daley, Jessica D., Barnum, Jessie L., Salgado, Claudia M., Reyes-Mugica, Miguel, Schneider, Corinne, Gumus, Serter, Thakrar, Darshit, Allen, Steven W., Canna, Scott W.
Format:	Artikel
Sprache:	eng
Schlagworte:	Alemtuzumab - therapeutic use Anti-Inflammatory Agents - therapeutic use Antibodies, Monoclonal - therapeutic use Antibodies, Neutralizing - therapeutic use Biomarkers Biomedical and Life Sciences Biomedicine Biopsy Bone marrow CD8 antigen Cytokines Cytotoxicity Dexamethasone Dexamethasone - therapeutic use Etoposide Etoposide - therapeutic use Fatal Outcome Female Ferritin Fever Histiocytosis Humans Immunoglobulins Immunoglobulins, Intravenous - therapeutic use Immunologic Factors - therapeutic use Immunology Induction therapy Infant Infectious Diseases Inflammation Inflammation - diagnosis Inflammation - drug therapy Interleukin 1 receptor antagonist Interleukin 1 Receptor Antagonist Protein - therapeutic use Internal Medicine Intravenous administration Laboratories Life Sciences & Biomedicine Lung nodules Lymphatic diseases Lymphocytes Lymphocytes T Lymphocytosis Lymphohistiocytosis, Hemophagocytic - diagnosis Lymphohistiocytosis, Hemophagocytic - drug therapy Magnetic resonance imaging Medical Microbiology Monoclonal antibodies Original Article Pediatrics Perforin Perforin - deficiency Proteins Salvage Therapy Science & Technology Stem cell transplantation T cell receptors Triglycerides
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