Sex: F
Vaccine: MEN, UNK, UNK
Symptoms: Haemolysis, Haptoglobin decreased, Hypotension, Intensive care, International normalised ratio increased
Clinical narrative: menigococcal sepsis; malaise; nausea; vomiting; diarrhea; rash; lethargic; hypotensive; diffuse purpuric rash; conjunctival hemorrhages; jaundice; Suspected vaccination failure; Fulminant Meningococcemia; hemolysis; elevated bilirubin; ele vated LDH; This case was reported in a literature article and described the occurrence of vaccination failure in a 18-year-old female patient who received Men ACWY-CRM NVS.  Co-suspect products included Men B NVS (Meningococcal B vaccine) (batch number UNK, expiry date unknown) for prophylaxis and eculizumab (batch number UNK, expiry date unknown) for paroxysmal nocturnal hemoglobinuria.   The patient's past medical history included budd chiari syndrome, hepatic vein thrombosis (treated with a trans jugular intrahepatic portosystemic shunt) and anemia. Previously administered products included warfarin (initiated  for secondary prevention for further thrombosis). Concurrent medical conditions included paroxysmal nocturnal hemoglobinuria. Concomitant products included cyanocobalamin (Vitamin B12) and folic acid (Folate).   On an unknown date, the patient received Meningococcal ACWY Vaccine (potential Menveo), Meningococcal B vaccine and eculizumab at an unknown dose and frequency. On an unknown date, unknown after receiving Meningococcal ACWY Vaccine (potential Menveo), not applicable after receiving Meningococcal B vaccine and an unknown time after starting hydrocortisone, the patient experienced vaccination failure (serious criteria hospitalization, GSK medically significant and life threatening), fulminant meningococcemia (serious criteria GSK medically significant and life threatening), meningococcal sepsis (serious criteria hospitalization, GSK medically significant and life threatening), malaise (serious criteria hospitalization), nausea (serious criteria hospitalization), vomiting (serious criteria hospitalization), diarrhea (serious criteria hospitalization), rash (serious criteria hospitalization), lethargy (serious criteria hospitalization), hypotension (serious criteria hospitalization), purpuric rash (serious criteria hospitalization), conjunctival hemorrhage (serious criteria hospitalization and GSK medically significant), jaundice (serious criteria hospitalization), hemolysis (serious criteria GSK medically significant), bilirubin increased and lactate dehydrogenase increased. The patient was treated with cefepime, ampicillin trihydrate (Ampicillin), hydrocortisone, ciprofloxacin, vancomycin, chemotherapy, nos (Adjuvant Therapy (Nos)), antibiotics nos (Antibiotic (Details Unknown)) and phenoxymethylpenicillin potassium (Penicillin-Vk). The action taken with eculizumab was unknown. On an unknown date, the outcome of the vaccination failure, purpuric rash, hemolysis, bilirubin increased and lactate dehydrogenase increased were unknown and the outcome of the fulminant meningococcemia, meningococcal sepsis, malaise, nausea, vomiting, diarrhea, rash, lethargy, hypotension, conjunctival hemorrhage and jaundice were recovered/resolved.   The reporter considered the vaccination failure, fulminant meningococcemia, meningococcal sepsis, malaise, nausea, vomiting, diarrhea, rash, lethargy, hypotension, purpuric rash, conjunctival hemorrhage, jaundice, hemolysis, bilirubin increased and lactate dehydrogenase increased to be related to Meningococcal ACWY Vaccine (potential Menveo). The reporter considered the hemolysis, bilirubin increased and lactate dehydrogenase increased to be related to Meningococcal B vaccine.  Additional details were provided as follows: This case was reported in a literature article and described the suspected vaccination failure in a 18-year-old female patient who was vaccinated with unspecified meningococcal ACWY conjugate vaccine (manufacturer unknown) for prophylaxis.   The patient was with current condition of paroxysmal nocturnal hemoglobinuria (PNH) which was diagnosed two years prior to presentation in the setting of primary Budd- Chiari syndrome caused by hepatic vein thrombosis in March 2013. The patient was also noted to be anemic at that time. The hepatic vein thrombosis was treated with a trans jugular intrahepatic portosystemic shunt (TIPS) and anticoagulation with warfarin was initiated as secondary prevention for further thrombosis. In addition to warfarin, her other medications included eculizumab (initiated upon diagnosis of PNH), vitamin B12, and folate. In 2013, the patient's lactate dehydrogenase 938 U/L and hemoglobin 8.3 gm/dl. No information on patient's family history.   On an unspecified date in 2012, before beginning therapy with eculizumab, the patient received Men ACWY conjugated vaccine (administration route and site unspecified, dosage unknown; batch number not provided). The age of vaccination was not provided. It was reported that the vaccine against serogroup B was not yet approved for market use.   On an unspecified date, unknown period after vaccination the patient presented to the emergency department with one day of progressively worsening malaise, nausea, vomiting, diarrhea, fever, and rash. Upon presentation to the emergency department, the patient was noted to be lethargic and hypotensive with a diffuse purpuric rash. The patient's initial vital signs were consistent with distributive shock: blood pressure 63/39mmHg, heart rate 129 bpm, respiratory rate 35 bpm, and oxygen saturation 95%. Pertinent physical findings included bilateral conjunctival hemorrhages, jaundice, and diffuse purpura. She had a left subclavian port that was clean with no surrounding erythema, exudate, or induration. Laboratory values were notable for white blood cell count of 18.1 k/??L, platelets 67 k/??L, lactate 8.9mmol/L, total bilirubin 21.8mg/dL (direct dL), haptoglobin less than 7mg/dL (reference range 20 -135mg/dL), and an INR of 5.18 (reference range 0.33-1.19). An abdominal ultrasound with Doppler revealed patent TIPS and patent hepatic vasculature including the hepatic vein. The patient was immediately intubated for airway protection. Resuscitation was initiated with intravenous fluids and vasopressors. The patient's blood pressure remained low despite maximum doses of vasopressors. Intravenous antibiotics were administered including vancomycin, cefepime, and ampicillin. Hydrocortisone was administered as adjunctive therapy in the setting of sepsis and concern for Waterhouse- Friderichsen syndrome. The patient was admitted to the intensive care unit for further management. The patient hemodynamics stabilized twenty-four hours after her initial presentation. Blood cultures grew Gram-negative diplococci identified as Neisseria meningitidis. The bacterial isolate was sent to the state health department laboratory for serogrouping and was determined to be nongroupable. Upon confirming Neisseria meningitidis infection, a contact investigation was launched by the hospital epidemiology team to identify those requiring meningococcal prophylaxis. After Neisseria meningitidis was identified, the antibiotics were narrowed to ceftriaxone 2 grams every 12 hours and then later switched to cefotaxime 2 grams every 6 hours to avoid potential hepatic toxicity. High dose steroids were carefully tapered off and the patient displayed no signs of Waterhouse-Friderichsen syndrome. Eculizumab dosing was continued at her normal dosing interval. In November 2014, the patient's lactate dehydrogenase 278 U/L and hemoglobin 11.2 gm/dl. The patient was diagnosed with menigococcal sepsis. In January 2015, the patient's lactate dehydrogenase 624 U/L and hemoglobin 6 gm/dl. In March 2015 sepsis resolved, the patient's lactate dehydrogenase 310 U/L and hemoglobin 9.6 gm/dl.  Intravenous antibiotics were continued for a total of 14 days, after which she began taking penicillin-VK250mg orally twice daily to be continued indefinitely for Neisseria meningitidis prophylaxis. Once available, the patient also received the vaccine against Neisseria meningitidis serogroup B. The patient will require a booster of the conjugated MenACWY vaccine every 5 years. She was given a prescription for oral ciprofloxacin at a dose of 750 mg to have on hand for "rescue therapy" if symptoms of meningococcal infection recur, with strict recommendations to also seek immediate medical attention in this circumstance. Threatening event with no further infectious issues. The patient's TIPS continues to function well and the patient had normal liver function. She continues to have evidence of mild hemolysis (slightly elevated bilirubin and LDH) on eculizumab but overall her symptoms are controlled. This case presentation highlights a life-threatening case of meningococcal disease complicated by purpura fulminans. In April 2017, the patient's lactate dehydrogenase 273 U/L and hemoglobin 10.3 gm/dl. This case considered as suspected vaccination failure as time to onset was and serogroup was unknown.  This case considered as serious due to life threatening; suspected vaccination failure and hospitalization. The author commented, "This case presentation highlights a life-threatening case Of meningococcal disease complicated by purpura fulminans. As of November 2014, there were sixteen reported cases of meningococcal infections associated with the use of eculizumab and this case would be the seventeenth. Our patient acquired a nongroupable strain of meningococcal disease while on eculizumab. She was not protected from her prior vaccination as MenACWY vaccine would not be expected to provide protection against."