Sex: F
Vaccine: MENB, MNQ
Symptoms: Neutrophil count increased, Pain, Pancytopenia, Paroxysmal nocturnal haemoglobinuria, Purpura
Clinical narrative: Initial information received on 02-Aug-2018 regarding an unsolicited valid serious case issued from a literature article. Description: Patients receiving eculizumab have an increased risk for meningococcal disease, but most reported cases are attributable to encapsulated meningococcal strains. We describe a case in which a nongroupable meningococcal strain, which rarely causes disease in healthy persons, caused fatal disease in an eculizumab recipient despite meningococcal vaccination. This case involves a 16 years old female patient who experienced consistent with waterhouse-friderichsen syndrome and paroxysmal nocturnal hemoglobinuria, while she received vaccines BEXSERO and MENACTRA and while treated with ECULIZUMAB. The patient's past medical history included Abdominal pain, Pancytopenia, Haemolysis and Paroxysmal nocturnal haemoglobinuria. The patient's past medical treatment(s), vaccination(s) and family history were not provided. Note: A bone marrow biopsy demonstrated cellularity nearly equals to 40%-50% and complete myeloid and erythroid maturation without evidence of dysplasia, aplasia, or an aberrant cell population. On an unknown date, after the vaccinations and administration of the drug, during the first 4 months after diagnosis, the patient exhibited mild pancytopenia and compensated hemolysis but remained transfusion-independent. However, 6 months after PNH diagnosis, she began eculizumab treatment because of worsening symptoms. Twenty-two hours after her second dose of eculizumab, the patient reported to a local emergency department with generalized body pain, headache, and emesis. She was afebrile, and her physical examination was unremarkable except for tachycardia of 124 beats per minute. Her leukocyte count was 8.9 ?-- 109 cells/L (within reference limits); absolute neutrophil count was 8.46 ?-- 109/L (upper limit 8.00 ?-- 109 cells/L). Her symptoms were attributed to side effects from eculizumab and resolved after treatment with prochlorperazine, ketorolac, diphenhydramine, and intravenous fluids. She was discharged to home after 2 hours of observation. Approximately 12 hours later, the patient reported weakness and purpura developed. She experienced cardiac arrest in transit to another emergency department, and resuscitative efforts were unsuccessful. Autopsy revealed hemorrhagic necrosis of the adrenal glands and focal hemorrhagic skin purpura, consistent with Waterhouse-Friderichsen syndrome.Relevant laboratory test results included: Other lab test were not reported. Final diagnosis was paroxysmal nocturnal hemoglobinuria and (fatal) consistent with waterhouse-friderichsen syndrome. ECULIZUMAB (ECULIZUMAB) was action taken unknown. Other corrective treatment was not reported. The patient outcome is reported as Unknown for paroxysmal nocturnal hemoglobinuria, as Unknown for vaccination failure, as Fatal on an unknown date for tachycardia, as Fatal on an unknown date for generalized body pain, as Fatal on an unknown date for headache, as Fatal on an unknown date for emesis, as Fatal on an unknown date for consistent with waterhouse-friderichsen syndrome, as Fatal on an unknown date for weakness, as Fatal on an unknown date for cardiac arrest, as Fatal on an unknown date for hemorrhagic necrosis of the adrenal glands, as Fatal on an unknown date for focal hemorrhagic skin purpura and as Unknown for worsening symptoms. An autopsy was done. The cause of death was reported as Adrenal cortex necrosis, Purpura and Waterhouse-Friderichsen syndrome.; Sender's Comments: This case involves a 16-year-old female, who reportedly experienced meningococcal disease with a fatal outcome caused by non-groupable Neisseria meningitidis 6 months after being diagnosed with Paroxysmal Nocturnal Hemoglobinuria (PNH). Patient had received both Menactra vaccine (booster vaccination) and a 2-dose series of a Meningococcal B vaccine after PNH diagnosis, and prior to initiation of treatment with eculizumab. Eculizumab is a complement inhibitor and therefore increases patient risk for meningococcal infections. Patients treated with eculizumab and without a history of prior vaccination are recommended to  receive a meningococcal vaccine at least 2 weeks prior to receiving the first dose of eculizumab and be revaccinated according to current medical guidelines for vaccine use. In the current case; vaccination occurred prior to initiating treatment with eculizumab. As with any other vaccine, Menactra vaccine may not protect 100% of individuals.  This case does not represent a confirmed vaccine failure based on the information provided; the specific serogroup involved was not identified.  Furthermore, exact latency was not specified.; Reported Cause(s) of Death: hemorrhagic necrosis of the adrenal glands; focal hemorrhagic skin purpura; consistent with Waterhouse-Friderichsen syndrome.