Sex: F
Vaccine: HPV9
Symptoms: Hypermobility syndrome, Injection site swelling, Injection site urticaria, Joint swelling, Laparoscopy
Clinical narrative: Mast Cell Activation Syndrome; Postural orthostatic tachycardia syndrome; This literature marketed information has been received from the authors in a published article title as stated below and refers to a 30-year-old female patient. The patient had history of frequent infections, constipation, asthma, migraines, and food sensitivities dating to her earliest memories, and, more recently, a dozen-years' worsening of other symptoms, including abdominal pain, nausea, vomiting, diarrhea, weight loss due to dietary limitations, tinnitus, orthostatic tachycardia, fainting, flushing, itching, psoriasis, migratory bone pain, and frequent anaphylaxis, in addition to other reactions to many foods, medication products, and environmental exposures. The family history was remarkable, containing a brother with severe seasonal allergies, a mother with irritable bowel syndrome, seasonal allergies, recurrent eye irritation, and easy bruising, and a father with rheumatoid arthritis, flushing, and oxalate nephrolithiasis.  On an unspecified date, she received her first dose of hpv rl1 6 11 16 18 31 33 45 52 58 vlp vaccine (yeast)(GARDASIL 9) (lot #, expiration date, dose, route of administration and anatomical location of vaccine were not reported) for prophylaxis at age 18-years-old (shortly after coitarche, and shortly after initially testing positive for HPV), developing hives and swelling near the injection site within hours. Orthostatic tachycardia newly emerged within four weeks after the second dose and became chronic (ultimately diagnosed as postural orthostatic tachycardia syndrome (POTS) several years later). Abdominal pain became chronic, with frequent acute severe exacerbations. Extensive evaluations over the next dozen years revealed no cause. Although exploratory laparoscopies at ages 18 and 22 years-old had been unrevealing, widespread endometriosis and interstitial cystitis were found at an ovarian cyst resection at age 29-years-old, by which point the patient also had proven intolerant of indwelling intravenous (IV) catheters, ureteral stents, and an implanted medication pump. Additionally, at 29-years-old, cervical adenocarcinoma in situ was diagnosed and surgically treated high-risk human papilloma virus was found too but was not serotype 16 or 18/45). By presentation, her diet had become limited to just chicken, two vegetables, and breadall other foods caused diffuse abdominal pain and nausea. Her body mass index (BMI) decreased from 17.5 in 2011 to 14.2 in 2021.  Physical examination at presentation was remarkable for a chronically ill-appearing, underweight (BMI 14.4 kg/m2) woman with a blood pressure of 179/90, a pulse of 60 which did not increase with standing, flushing about her chest, moderate diffuse abdominal tenderness, and hypermobile joints (Beighton score 8/9). MCAS was diagnosed at 32 upon the recognition of her MCAS-consistent history and the finding of an elevated 24 h urinary N-methylhistamine level of 296 mcg/g Cr (normal 30200) in addition to an elevated serum chromogranin A of 146 ng/mL (normal < 102, and without confounding factors). With cetirizine and famotidine twice daily, nausea, flushing, pruritus, sneezing, coughing, and rashes significantly decreased. Hydroxyurea helped bone pain, and gabapentin helped neuropathic pain, tingling, abdominal pain, and sleep. Low dose naltrexone (LDN) helped joint pain, bone pain, and joint swelling. Nortriptyline helped abdominal pain, bilious diarrhea, and sleep. Salt supplements, midodrine, and propranolol helped her POTS (maintaining blood pressure and heart rate and reducing dizziness, fainting, and nausea). Alprazolam helped as needed with severe nausea and pain. Ondansetron helped as needed for nausea. Diphenhydramine and epinephrine helped as needed for flares and anaphylaxis. Oral Cromoglicic acid (CROMOLYN) in commercially available and compounded formulations was not tolerated. Diet remains unimproved, including intolerance of many nutrition supplementation products, but no clinical signs or laboratory evidence of micronutrient deficiencies have emerged yet.   Literature report:   Upon internal review the of Mast Cell Activation Syndrome (MCAs) was considered as medically significant.   This is one of several report from the same literature article.   A copy of the published article is attached as further documentation of the patient's experience.