Three Autopsy Cases of Non-Meningococcal Waterhouse-Friderichsen Syndrome with Hypoplastic Spleen or Post-Splenectomy Status.

We report three cases of Waterhouse-Friderichsen syndrome (WFS) that were confirmed during forensic autopsies. Case 1 involved a man in his 50s post-splenectomy. Bacteriological examination revealed Streptococcus pneumoniae (S. pneumonia). The patient was considered to have died of asphyxiation after aspirating vomit. Case 2 involved a man in his 40s. Bacteriological examination again revealed S. pneumoniae. Histopathological examination showed hypoplasia of the spleen. This patient was considered to have died of multiple-organ failure due to sepsis, disseminated intravascular coagulation, and WFS. Case 3 involved a post-splenectomy woman in her 60s with a history of systemic lupus erythematosus. Bacteriological examination revealed Streptococcus oralis. This patient was considered to have died of multiple-organ failure due to sepsis, disseminated intravascular coagulation, and WFS. These three cases were included among forensic autopsies conducted in the last 5 years. WFS has been considered a rare disease, but may be more frequent than previously assumed. If a mildly ill patient displays a sudden change in status and dies within a short period of time, we consider it necessary to perform not only bacteriological examinations, but also histopathological examination of the spleen during autopsy.

The importance of the postmortem interval for the diagnosis of Waterhouse­Friderichsen syndrome by Neisseria meningitidis in a series of forensic cases

The effective value of microbiological post-mortem examinations stands as fundamental in forensic cases involving microbiology. We ran these analyses on five victims, who suddenly died after showing persistent fever. The examinations were conducted between 48 hours and 10 days after death, and adrenal gland apoplexy was detected in all the cases. Microbiological examinations identified Neisseria meningitidis, which was accountable for Waterhouse­Friderichsen syndrome. Diplococci were isolated from three cadavers that underwent forensic dissection between 2 and 3 days after death. The remaining two cadavers showed polymicrobial contamination, and a polymerase chain reaction technique was necessary to identify the pathogen. We assumed that the microbial overlap could lead to diagnostic mistakes and conceal the identification of the lethal pathogen. Therefore, we suggest using molecular techniques for a postmortem interval (PMI) longer than 72 hours. Classical microbiological examination should be performed for PMI within 72 hours.

A case report of spontaneous staphylococcal meningitis in a cynomolgus monkey.

This report describes a suppurative meningitis in a young cynomolgus. The animal had neutrophil aggregation in the subarachnoid space and hemorrhage in bilateral adrenal glands. Staphylococcus was identified by FISH in brain. To our knowledge, this is the first case of staphylococcal meningitis with Waterhouse-Friderichsen syndrome in a cynomolgus monkey.

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.

A rare case of Waterhouse-Friderichsen syndrome caused by Capnocytophaga canimorsus in an immunocompetent patient.

A 53-year-old Caucasian male with hypertension and active tobacco abuse presented to a community hospital with a 2-day history of vague abdominal pain, myalgia and increased lethargy after being bitten on his right hand by the family dog while camping just 3 days prior to symptom onset. He expired within 90 min upon arrival to our intensive care unit. Pre-mortem blood cultures grew a fastidious Gram-negative aerobic rod that was identified as Capnocytophaga canimorsus. Autopsy findings showed multi-organ disseminated intravascular coagulopathy with microthrombi along with bilateral adrenal hemorrhage and necrosis of the adrenal glands consistent with Waterhouse-Friderichsen syndrome. This case contributes to the medical literature as a rare presentation of Capnocytophaga canimorsus infection in an otherwise immunocompetent patient and stresses the importance of a thorough history taking and physical examination by clinicians along with prompt administration of appropriate antibiotics.

Muerte súbita por sepsis meningocócica: diagnóstico post mórtem / Sudden death from meningococcal sepsis: postmortem diagnosis

La presentación más común de la enfermedad meningocócica es la meningitis, con una tasa de incidencia de 0,92 por 100.000 habitantes y una letalidad del 40,3% en España. Un cuadro meníngeo puede cursar con síntomas inespecíficos como la cefalea, entre otros, de tal forma que en ocasiones progresa rápidamente en cuestión de horas y provoca la muerte del paciente antes de haber recibido una atención médica adecuada. Además de la meningitis clásica, el meningococo produce una enfermedad sistémica que incluye la sepsis meningocócica y la coagulopatía intravascular diseminada. En pacientes con sepsis meningocócica se ha descrito el síndrome de Waterhouse-Friderichsen, que se caracteriza por hemorragia suprarrenal bilateral, coagulación intravascular diseminada e hígado de shock, y que produce una bacteriemia grave. Esta combinación provoca unshock fulminante y, si no se trata, puede producir muerte súbita. Presentamos el caso de una paciente con odinofagia, fiebre, vómitos y deposiciones diarreicas de 24 horas de evolución, que a pesar de recibir asistencia médica ambulatoria y hospitalaria fallece como consecuencia de una sepsis fulminante por Neisseria meningitidis y síndrome de Waterhouse-Friderichsen no diagnosticado clínicamente (AU)

The most common presentation of meningococcal disease is meningitis, with an incidence rate of 0.92 per 100,000 inhabitants and a mortality rate of 40.3% in Spain. Meningeal disease may present with nonspecific symptoms such as headache, among others, sometimes progressing rapidly to a fatal outcome before the patient receives adequate medical care. Besides classical meningitis, Neisseria meningitis produces a systemic disease that includes meningococcal sepsis and disseminated intravascular coagulopathy. In patients with meningococcal sepsis the Waterhouse-Friderichsen syndrome is described, which is characterized by bilateral adrenal hemorrhage, intravascular coagulation and shock liver, producing a severe bacteremia. This combination causes a fulminant shock and, if untreated, it may cause sudden death. We report the case of a female patient with sore throat, fever, vomiting and diarrhea of 24 hours of evolution. Despite receiving inpatient and outpatient medical care, she died as a result of N. meningitidis fulminant sepsis and clinically undiagnosed Waterhouse-Friderichsen syndrome (AU)

[A 52-year-old woman with acute shock and purpura fulminans. Pneumococcal sepsis]. / 52-jährige Patientin mit akuter Schocksymptomatik und Purpura fulminans. Pneumokokkensepsis.

We report a 52-year-old female patient admitted with fever, chills, and myalgias since the previous day. On the day of admission she had a generalized seizure. The patient had no previous illnesses. Laboratory investigations showed consumptive coagulopathy with clinical manifestations of shock and development of multiple organ failure. Pneumococci were detected in blood cultures. Furthermore the skin showed purpura fulminans all over. The patient died within 24 h after admission in the intensive care unit. On autopsy, in addition to adrenal and myocardial hemorrhages, hypoplasia of the spleen was found. Fulminant pneumococcal sepsis is a life-threatening disease that occurs in patients with risk factors like splenic hypoplasia or asplenia. Sometimes a fulminant pneumococcal sepsis may be the first clinical manifestation of a hitherto unknown splenic hypoplasia. In this context the general recommendation of vaccination against pneumococci in patients with risk factors like splenic hypoplasia or asplenia, in patients older than 60, and in children from 2 months onward has to be emphasized.

Staphylococcus aureus sepsis and the Waterhouse-Friderichsen syndrome in children.

Staphylococcus aureus has increasingly been recognized as a cause of severe invasive illness. We describe three children who died at our institution after rapidly progressive clinical deterioration from this infection, with necrotizing pneumonia and multiple-organ-system involvement. The identification of bilateral adrenal hemorrhage at autopsy was characteristic of the Waterhouse-Friderichsen syndrome, a constellation of findings usually associated with fulminant meningococcemia. The close genetic relationship among the three responsible isolates of S. aureus, one susceptible to methicillin and two resistant to methicillin, underscores the close relationship between virulent methicillin-susceptible S. aureus and methicillin-resistant S. aureus isolates now circulating in the community.

[Waterhouse-Friderichsen cases in the archives of ZMS PAM--epidemiological conditions and diagnostic difficulties]. / Przypadki zespolu Waterhouse'a-Friderichsena w materialach ZMS PAM--uwarunkowania epidemiologiczne oraz trudnosci diagnostyczne.

15 cases of meningococcal infections from 2003-2004 were discussed in the study. Their therapy ended in failure and they had been typically autopsied in our Forensic Med. Department. During the autopsy material for classical microbiological investigations was collected. That type of death is characterised with pathognomonic and autopsy image. The problem is the microbiological identification of the pathogen group and type. It is connected with the characteristic of the analysed material and the biological features of Neisseria meningitidis. The molecular and genetic methods show their usefulness in this type of diagnostic of the properly selected and collected material which typically are: cerebrospinal fluid, brain tissue with the arachnoid membranes and spleen tissue.

OPSI (overwhelming postsplenectomy infection) syndrome: a case report.

Splenectomized patients are likely to suffer from severe infections, such as sepsis and meningitis. This syndrome is called overwhelming postsplenectomy infection (OPSI) in Europe and America. We present an adult case of OPSI syndrome, which occurred as respiratory insufficiency, and thrombocytopenia. The course is rapid, the clinical symptoms are serious, and the prognosis is very poor. Clinical examination showed cyanosis, mandibular hypertonia, psychomotor anxiety and purpura. Laboratory findings were thrombocytopenia, leukocytosis, hypoglycemia and altered coagulation parameters. A chest X-ray showed right pulmonary aspecific thickening. The autopsy findings occurred as Waterhouse-Friderichsen syndrome.

Spontaneous Waterhouse-Friderichsen syndrome in a gang-housed baboon.

Waterhouse-Friderichsen syndrome can cause acute death in the baboon without specific signs. Furthermore, this syndrome could result from stress-related intestinal permeability changes that allow macromolecules and/or microbiological entities to enter the systemic circulation. The resulting sepsis could cause adrenocortical insufficiency, hypotension and shock leading to death.

Waterhouse-Friderichsen syndrome secondary to Capnocytophaga canimorsus septicemia and demonstration of bacteremia by peripheral blood smear.

Waterhouse-Friderichsen syndrome caused by Capnocytophaga canimorsus septicemia was fatal in a previously healthy 47-year-old woman. The patient died suddenly in less than 12 hours after presentation, in spite of supportive measures, including ventilation, antibiotic coverage, pressor therapy, and multiple transfusions of blood products. The diagnosis of infection due to an unusual organism was suspected earlier in the course of management after review of the peripheral blood smear. The importance of the findings in the blood smear and their correlation with infection due to this organism are discussed.

[A fulminant course of infection after splenectomy]. / Foudroyant verlaufende Sepsis nach Splenektomie.

A report is given of two patients with a history of splenectomy many years previously due to traumatic rupture. No vaccination was given to either patient. From a state of good health, both patients developed fulminant, therapy-resistant sepsis with proof of Streptococcus pneumoniae in the blood culture. Autopsy findings were similar to Water-house-Friderichsen-syndrome. In conjunction with the history of splenectomy, the final pathological diagnosis was a so-called OPSI syndrome. This postsplenectomy sepsis is discussed further.

[Fulminant infection outcome after splenectomy]. / Foudroyant verlaufende Sepsis nach Splenektomie.

A report is given of two patients with a history of splenectomy many years previously due to traumatic rupture. No vaccination was given to either patient. From a state of good health, both patients developed fulminant, therapy-resistant sepsis with proof of Streptococcus pneumonlae in the blood culture. Autopsy findings were similar to Waterhouse-Friderichsen-syndrome. In conjunction with the history of splenectomy, the final pathological diagnosis was a so-called OPSI syndrome. This postsplenectomy sepsis is discussed further.