Sepsis due to Streptococcus pneumoniae associated with secondary hemophagocytic lymphohistiocytosis in a splenectomized patient for spherocytosis: A case report. Issue 28 (July 2017)

• Record Type: Journal Article
• Authors: Birlutiu, Victoria; Birlutiu, Rares Mircea
• Other Names: Asensi. Victor section editor.
• Is Part Of: Medicine. Volume 96:Issue 28(2017)
• Publication Date: 2017-07
• Subjects: hemophagocytic lymphohistiocytosis -- HLH -- splenectomized patient -- Streptococcus pneumoniae; Medicine -- Periodicals; Medicine -- Periodicals; Médecine -- Périodiques; Geneeskunde; Medicine; Periodicals; Periodicals; 610.5
• DOI: 10.1097/MD.0000000000007520
• Languages: English
• ISSNs: 0025-7974

Abstract

Abstract: Rationale: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome that is characterized by an inappropriate hyperinflammatory immune response – primary, as a consequence of a genetic defect of NK cells and cytotoxic T lymphocytes or – secondary, in the progression of infections, rheumatic or autoimmune diseases, malignancies or metabolic diseases. Patient concerns: We present the case of a secondary HLH due to Streptococcus pneumoniae infection in a splenectomised patient for spherocytosis, a 37-year-old patient who was splenectomised in childhood for spherocytosis, without immuneprophylaxis induced by antipneumococcal vaccine. Outcomes: He developed a severe pneumococcal sepsis associated with secondary HLH, with unfavorable outcome and death. Lessons: To our knowledge, just 2 similar cases had been published in the literature, none in which the secondary HLH was the consequence of an invasive pneumococcal infection in a splenectomized patient for spherocytosis, and the association of splenectomy with HLH is surprizin.