Hemophagocytic lymphohistiocytosis in a patient with rheumatoid arthritis secondary to septic shock

Luis Mario González-Galván; Pedro Arredondo-Ruiz; Germán Chac-Lezama; Angelina América Molina-Villena; Vanesa Ortiz-Falfan; Oscar Santana-Castillo; José Ramón Benítez-Tirado; Martín Sáenz-Castro; Juan Antonio Pérez-García; Daniel Alonso-Martínez; Carlos Alberto Peña-Pérez

2017, Number 1

Med Int Mex 2017; 33 (1)

Hemophagocytic lymphohistiocytosis in a patient with rheumatoid arthritis secondary to septic shock

González-Galván LM, Arredondo-Ruiz P, Chac-Lezama G, Molina-Villena AA, Ortiz-Falfan V, Santana-Castillo O, Benítez-Tirado JR, Sáenz-Castro M, Pérez-García JA, Alonso-Martínez D, Peña-Pérez CA

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ABSTRACT

Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome and not an independent disease. Hemophagocytic lymphohistiocytosis is a terminal hyperinflammatory reaction that can occur in different conditions. The hemophagocytic lymphohistiocytosis genetic forms are due to a defect in the transport and operation of the cytotoxic granules of NK cells and cytotoxic T lymphocytes and are not only restricted in childhood. Acquired forms of hemophagocytic lymphohistiocytosis are found in infections, auto-inflammatory and autoimmune diseases, cancers and acquired immune deficiencies. The treatment is aimed at suppressing the activity of cytokines and eliminate infected and activated cells.