Ultomiris

Adult & ped patients w/ paroxysmal nocturnal hemoglobinuria (PNH) who presents w/ clinical symptoms indicative of high disease activity; who are clinically stable after eculizumab treatment for at least the past 6 mth. Adult & ped patients w/ atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy (TMA). Add-on to standard therapy for adult patients w/ generalized myasthenia gravis (gMG) who are anti-acetylcholine receptor Ab +ve.

IV Adult & ped weighing ≥100 kg Loading dose: 3,000 mg. Maintenance dose: 3,600 mg every 8 wk to be given 2 wk after loading dose, ≥60 to <100 kg Loading dose: 2,700 mg. Maintenance dose: 3,300 mg every 8 wk to be given 2 wk after loading dose, ≥40 to <60 kg Loading dose: 2,400 mg. Maintenance dose: 3,000 mg every 8 wk to be given 2 wk after loading dose. PNH & aHUS Adult & ped weighing ≥30 to <40 kg Loading dose: 1,200 mg. Maintenance dose: 2,700 mg every 8 wk to be given 2 wk after loading dose, ≥20 to <30 kg Loading dose: 900 mg. Maintenance dose: 2,100 mg every 8 wk to be given 2 wk after loading dose, ≥10 to <20 kg Loading dose: 600 mg. Maintenance dose: 600 mg every 4 wk to be given 2 wk after loading dose, ≥5 to <10 kg Loading dose: 600 mg. Maintenance dose: 300 mg every 4 wk to be given 2 wk after loading dose.

Do not initiate in patients w/ unresolved Neisseria meningitidis infection. Patients who are not currently vaccinated against Neisseria meningitidis unless they receive prophylactic treatment w/ appropriate antibiotics until 2 wk after vaccination.

Possible systemic infusion reactions (infusion-related) causing allergic or hypersensitivity reactions including anaphylaxis. Not intended for SC; not to be administered as IV push or bolus inj. Not indicated for patients w/ Shiga toxin E. coli related hemolytic uremic syndrome. Increased patient's susceptibility to meningococcal infection/sepsis. Vaccinate against meningococcal infections at least 2 wk prior to or at the time of treatment initiation. Monitor all patients for early signs of meningococcal infection & sepsis; immediately evaluate if infection is suspected & treat w/ appropriate antibiotics. Closely monitor patients for disease symptoms after recommended vaccination. Patients w/ active systemic infections. Possible serious infections w/ Neisseria species (other than Neisseria meningitidis) including disseminated gonococcal infections. Advise patients about gonorrhoea prevention. Vaccinate patients <18 yr against H. influenzae & pneumococcal infections & adhere strictly to national vaccination recommendations for their age group. Interrupt use & institute appropriate supportive measures if signs of CV instability or resp compromise occur. May induce immune response w/ any therapeutic protein. Closely monitor for signs & symptoms of hemolysis in patients w/ PNH who discontinue treatment. Consider restarting Ultomiris if signs & symptoms of hemolysis including elevated LDH occur after discontinuation. Patients at higher risk for TMA recurrence may require chronic therapy. Closely monitor for signs & symptoms of TMA on an on-going basis if patients must discontinue treatment. Consider restarting Ultomiris if symptoms of gMG occur after discontinuation. Women of childbearing potential should use effective contraception methods during & up to 8 mth after treatment. Pregnancy. Discontinue breastfeeding during & up to 8 mth after treatment. Childn <9 yr w/ PNH. Ped patients w/ gMG.

Diarrhoea, nausea; fatigue; URTI, nasopharyngitis; headache. Vomiting, abdominal pain, dyspepsia; pyrexia, flu-like illness, asthenia; infusion-related reaction; arthralgia, back pain, myalgia, muscle spasms; dizziness; urticaria, pruritus, rash.

Haemorrheologicals / Neuromuscular Disorder Drugs

L04AJ02 - ravulizumab ; Belongs to the class of complement inhibitors. Used as immunosuppressants.

POM