An autopsy case of pneumococcal Waterhouse-Friderichsen syndrome with possible functional asplenia/hyposplenia.
We report an autopsy case of rapid progressive Waterhouse-Friderichsen syndrome (WFS) associated with Streptococcus pneumonia infection in a previously healthy man. Although he once visited a hospital about 6 hours before death, the both physical and serological examination did not show any sign of overwhelming infection. Autopsy showed massive adrenal hemorrhage without inflammation, and showed proliferation of gram positive cocci and microthrombosis in the vessels of many organs. The pathological change of respiratory tract was extremely minimal. Size and weight of the spleen possible decreased than normal. However, histological examination showed that obscuration of germinal center and decreasing the immunological cells of mantle and marginal zone. Immunohisitochemically, marked decreasing the marginal zone macrophages, which are positive for specific intercellular adhesion molecule grabbing nonintegrin receptor-1 (SIGN-R1) and macrophage receptor with collagenous structure (MARCO), were decreased comparing with age-matched control case. Polymerase chain reaction (PCR) assay using each DNA, extraction from formalin-fixed paraffin-embedded specimen (FFPE) samples of lung, adrenal gland, heart, spleen, and kidney showed positive the ply gene and the lytA gene specific for Streptococcus pneumonia. Present case showed possible acquired atrophy of spleen, especially decreasing marginal zone macrophage may correlate with rapid progression of sepsis of Streptococcus pneumonia with massive adrenal hemorrhage. In addition, present case showed the usefulness of PCR using FFPE for the postmortem diagnosis of WFS.
Department of Legal Medicine, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama Toyama, Japan.
Yukiko
Y
Hata
Department of Forensic Sciences, Graduate School of Medical Sciences, Akita University Akita, Japan.
Takashi
T
Chiba
Department of Forensic Sciences, Graduate School of Medical Sciences, Akita University Akita, Japan.
Maki
M
Ohtani
Department of Pathology, Toyama Prefectural Central Hospital Toyama, Japan.
Shin
S
Ishizawa
Department of Legal Medicine, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama Toyama, Japan.
Naoki
N
Nishida
D000311:Adrenal Glands / Q000382:microbiology / Q000473:pathology*; D001284:Atrophy; D001344:Autopsy; D001706:Biopsy; D002423:Cause of Death; D017809:Fatal Outcome; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D009929:Organ Size; D011008:Pneumococcal Infections / Q000382:microbiology / Q000473:pathology*; D013154:Spleen / Q000737:chemistry / Q000382:microbiology / Q000473:pathology*; D013296:Streptococcus pneumoniae / Q000302:isolation & purification*; D014884:Waterhouse-Friderichsen Syndrome / Q000382:microbiology / Q000473:pathology*
Asplenia/hyposplenia; Streptococcus pneumonia; Waterhouse-Friderichsen syndrome; autopsy