Fatal overwhelming postsplenectomy infection due to Streptococcus pneumoniae serotype 10A with atypical polysaccharide capsule in a patient with chromosome 22q11.2 deletion syndrome: A case report. Issue 3 (March 2019)

• Record Type: Journal Article
• Authors: Matsuoka, Keiko; Yanagihara, Itaru; Kawazu, Yukiko; Sato, Maho; Nakayama, Masahiro; Nakura, Yukiko; Kanagawa, Nao; Akeda, Yukihiro; Fujiwara, Futoshi; Itoh, Kazutoshi; Kawata, Hiroaki; Takeuchi, Makoto
• Is Part Of: Journal of infection and chemotherapy. Volume 25:Issue 3(2019:Mar.)
• Page Start: 192
• Page End: 196
• Publication Date: 2019-03
• Subjects: Overwhelming postsplenectomy infection -- Streptococcus pneumoniae -- Pneumococcal vaccine -- Serotype 10A -- Chromosome 22q11.2 deletion syndrome; PPV23 23-valent pneumococcal polysaccharide vaccine -- IPD Invasive pneumococcal disease -- OPSI Overwhelming postsplenectomy infection -- MLST Multilocus sequence typing -- ST sequence type -- PCV13 13-valent pneumococcal conjugate vaccine -- IDSA Infectious Diseases Society of America; Chemotherapy -- Periodicals; Infection -- Periodicals; Communicable diseases -- Chemotherapy -- Periodicals; 615.5805
• DOI: 10.1016/j.jiac.2018.07.015
• Languages: English
• ISSNs: 1341-321X

Abstract

Abstract: We report the first case of a teenage patient with chromosome 22q11.2 deletion syndrome who died of overwhelming postsplenectomy infection (OPSI) by Streptococcus pneumoniae despite appropriate prevention by pneumococcal vaccine. He had congenital heart disease and underwent several surgeries. Immunodeficiency had not been noticed clinically. Two years prior to death, splenectomy was performed for a drug-resistant idiopathic thrombocytopenic purpura and he was immunized with 23-valent pneumococcal polysaccharide vaccine (PPV23) 4 months after splenectomy. He died suddenly after a mild flu-like symptom. Autopsy was performed and OPSI was diagnosed. Blood culture was positive for S. pneumoniae . This isolated S. pneumoniae strain was serotypically un-typable by polyvalent serum agglutination test. On the contrary, multilocus sequence typing followed by DNA sequencing indicated the molecular serotype as 10A. Additional testing using monovalent and factor-specific sera confirmed the strain as serotype 10A. Ultrastructural observation of this S. pneumoniae strain showed that the polysaccharide capsule was thin and sparse. We speculate that the abnormal morphology of the capsule may have accounted for the polyvalent serum agglutination failure and may possibly be associated with severity of OPSI observed in this case. Chromosome 22q11.2 deletion syndrome is associated with certain immunodeficiency, especially susceptible to S. pneumoniae infections; however, fatal OPSI has not been reported. In addition to vaccination, prophylactic antibiotics may be necessary for these patients who are at risk of immunodeficiency.