Sepsis due to : A case reportStreptococcus pneumoniae: A case report associated with secondary hemophagocytic lymphohistiocytosis in a splenectomized patient for spherocytosis: A case report

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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.

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