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RESEARCH PRODUCT
Sepsis due to Streptococcus pneumoniae associated with secondary hemophagocytic lymphohistiocytosis in a splenectomized patient for spherocytosis
Rares Mircea BirlutiuVictoria Birlutiusubject
Secondary Hemophagocytic LymphohistiocytosisAdultMaleendocrine systemsplenectomized patientmedicine.medical_treatmentSpherocytosisSplenectomySpherocytosis Hereditarymedicine.disease_causeLymphohistiocytosis HemophagocyticPneumococcal InfectionsSepsis03 medical and health sciences0302 clinical medicineImmune systemFatal Outcomehemic and lymphatic diseasesSepsisStreptococcus pneumoniaemedicineCytotoxic T cellHumans030212 general & internal medicineClinical Case ReportHemophagocytic lymphohistiocytosisbusiness.industry4900General Medicinemedicine.diseaseStreptococcus pneumoniaehemophagocytic lymphohistiocytosis030220 oncology & carcinogenesisImmunologySplenectomybusinessResearch ArticleHLHdescription
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.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2017-07-01 | Medicine |