What this South Korea PNH treatment-cost guide calculates
Paroxysmal nocturnal hemoglobinuria, usually shortened to PNH, is an acquired hematopoietic stem-cell disorder in which affected blood cells become vulnerable to complement-mediated destruction.
The name can sound as if dark urine at night is the whole disease, but clinical review also considers hemolysis, the size of the PNH clone, anemia, thrombosis, kidney injury, pulmonary complications, pain and bone-marrow failure.
PNH is not another name for blood cancer, and this calculator does not diagnose PNH, classify marrow failure or predict survival.
Soliris, the brand used here for eculizumab, and Ultomiris, the brand used here for ravulizumab, are intravenous complement C5 inhibitors.
Their licensed schedules differ sharply: adult PNH eculizumab has four weekly induction doses followed by dosing every two weeks, while adult ravulizumab uses a weight band and then an eight-week maintenance interval.
South Korean reimbursement also depends on documented clinical criteria, continued monitoring, special-case registration and the actual HIRA review rather than on the medicine name alone.
Schedule
Generate reference infusion dates, doses and vial counts for eculizumab or ravulizumab.
Coverage checks
Separate the initial common pathway, the ravulizumab switch pathway, exclusions and continuation checks.
Cost layers
Keep covered drug cost, outside-criteria drug cost, other covered care and non-covered spending separate.
Licensed adult PNH schedules used in the model
Comparison of licensed adult PNH eculizumab and ravulizumab schedules in South Korea| Medicine | Starting phase | Maintenance phase | Reference interval |
|---|
| Eculizumab | 600 mg on days 0, 7, 14 and 21 | 900 mg on day 28, then 900 mg | 14 days |
| Ravulizumab | Weight-band loading dose on day 0 | First maintenance on day 14, then weight-band maintenance | 56 days |
Eculizumab reference calculation
The Korean MFDS label for the Soliris 300 mg per 30 mL product gives adult PNH patients 600 mg every seven days for the first four weeks.
A 900 mg dose follows seven days after the fourth induction dose and then repeats every fourteen days.
With the HIRA public ceiling price of KRW 3,600,000 per 300 mg vial, a 600 mg dose uses two vials and has a public-list cost of KRW 7,200,000.
A 900 mg dose uses three vials and has a public-list cost of KRW 10,800,000.
A twelve-calendar-month window beginning July 23, 2026 contains twenty-nine modeled infusions and a KRW 298,800,000 public-list drug total.
Ravulizumab reference calculation
Adult PNH ravulizumab uses a 2,400 mg loading dose and 3,000 mg maintenance dose from 40 kg to below 60 kg.
From 60 kg to below 100 kg, the doses are 2,700 mg and 3,300 mg, and at 100 kg or above they are 3,000 mg and 3,600 mg.
The first maintenance dose is fourteen days after loading and subsequent maintenance doses occur every eight weeks.
The HIRA public ceiling prices used here are KRW 5,044,087 for a 300 mg per 3 mL vial and KRW 17,149,895 for a 1,100 mg per 11 mL vial.
At 70 kg, the model chooses two 1,100 mg vials and two 300 mg vials to cover the 2,700 mg loading dose for KRW 44,387,964.
It chooses three 1,100 mg vials for each 3,300 mg maintenance dose at KRW 51,449,685.
A twelve-month reference window contains one loading and seven maintenance infusions, producing the reproducible public-list total of KRW 404,535,759.
Generated dates are not appointments
The labels allow limited schedule movement, but this calculator generates the unshifted reference dates so scenarios remain comparable.
A treating team must decide any actual change related to infection, surgery, pregnancy, laboratory findings or provider scheduling.
Extra ravulizumab doses that may be required after plasma exchange, plasmapheresis, fresh frozen plasma or intravenous immunoglobulin are outside this model.
How the HIRA initial-coverage screen is represented
The public PNH criteria reviewed for this calculator use a common initial pathway with four core elements.
The patient must be an adult, the PNH granulocyte clone must be at least 10 percent, lactate dehydrogenase must be at least 1.5 times the local upper limit of normal, and at least one specified clinical complication must be documented.
The calculator compares those thresholds with user-entered facts but cannot verify a laboratory report, imaging study, diagnosis or claim submission.
Laboratory and age
- Age 18 years or older
- PNH granulocyte clone at least 10 percent
- LDH at least 1.5 times the upper limit of normal
At least one complication
- Thrombosis or embolism requiring therapeutic anticoagulation
- Qualifying pulmonary impairment
- Qualifying renal impairment
- Severe recurrent smooth-muscle spasm
Exclusion screen
- Two or more severe aplastic-anemia findings
- Life-threatening disease limiting long-term benefit
- Another condition likely to markedly reduce response
- Ravulizumab pregnancy or early-postpartum exclusion
The clinical-complication check is not a symptom checklist
The thrombosis pathway refers to objectively confirmed thrombosis or embolism that needs therapeutic anticoagulation.
The pulmonary pathway uses severe functional symptoms with an NT-proBNP value above 160 pg/mL or confirmed pulmonary hypertension.
The renal pathway refers to the specified eGFR history and related evidence, or acute renal failure requiring dialysis, while the smooth-muscle pathway refers to severe recurrent pain requiring admission or narcotic analgesia.
A user should select a box only when the treating team has linked the documented facts to that public criterion.
Severe aplastic-anemia exclusion boundary
The model asks for a count from zero to four because the public exclusion screen uses two or more listed findings.
Those findings are an absolute neutrophil count below 0.5 times 10⁹/L, platelets below 20 times 10⁹/L, reticulocytes below 25 times 10⁹/L and severe marrow hypocellularity.
The calculator does not derive this count from raw laboratory values because marrow context and clinical interpretation belong to hematology care.
Ravulizumab switch pathway
Prior covered eculizumab plus controlled LDH
The model provides a separate switch route when ravulizumab is selected.
It requires at least six months of covered eculizumab treatment and an LDH value at or below 1.5 times the upper limit of normal.
This route can satisfy the initial-pathway side of the cost model even when the original high-LDH common pathway is no longer met, but the exclusion screen still applies.
A positive switch screen is not a recommendation to change medicine and is not proof that HIRA will approve the claim.
The MFDS label states that a ravulizumab loading dose used in an eculizumab switch is given two weeks after the final eculizumab infusion.
The generated schedule begins on the date that the user enters, so that date must be the intended ravulizumab loading date rather than the date of the final eculizumab dose.
Actual selection and timing must come from the prescribing hematology team.
Continuation review after the first six months
High-cost PNH medicine reimbursement is not represented as a one-time approval.
The reviewed public criteria call for regular clinical and laboratory submissions, with LDH, complete blood count, reticulocytes, transfusion history, iron measures, renal measures and clinical history among the recurring data.
Vaccination records, the clinical feature that supported initial treatment and PNH clone information also remain relevant.
Monitoring submission
The required recurring evidence is marked as submitted.
LDH control
LDH is documented as sustainably at or below 1.5 times the upper limit of normal.
Missed doses
The model checks three or more missed eculizumab doses and one or more missed ravulizumab doses per six months.
Renal deterioration
Ongoing dialysis after worsening renal function is treated as a continuation warning.
New major thrombosis
A new life-threatening thrombotic event is treated as a continuation warning.
Marrow, transplant and year-one state
The severe aplastic count, post-transplant improvement and combined year-one clone and LDH state are kept visible.
For transparent budgeting, infusions before the calendar six-month point use the modeled initial status and later infusions use the modeled continuation status.
If a continuation check is not met, later public-list drug cost is moved into the outside-criteria column at 100 percent rather than silently disappearing.
This is a conservative scenario boundary, not the actual HIRA review date, and it does not model every exception or additional committee review.
A failed screen must never be used as an instruction to delay or stop treatment.
D59.5, V187 and the 10 percent special-case share
The current Ministry of Health and Welfare special-case notice lists paroxysmal nocturnal hemoglobinuria under disease code D59.5 and special-case code V187.
Registered rare-disease patients generally pay 10 percent for covered care related to the registered condition under this provision.
The diagnosis name alone does not turn on the 10 percent rate, and unrelated care, non-covered care and medicine outside a coverage criterion do not automatically receive that treatment.
The calculator therefore leaves V187 registration off by default and asks the user to confirm both registration and related covered billing.
When it is selected, covered PNH drug and other covered PNH care use 10 percent in the estimate.
When it is not selected, the chosen ordinary reference rate is used: 20 percent inpatient or 30, 40, 50 or 60 percent depending on the simplified outpatient provider level.
Registration timing and billing scope matter
The current notice describes retroactive application from the confirmed diagnosis date when an application is made within the specified thirty-day period, and application-date treatment when it is made later.
This calculator does not reconstruct that timing or determine whether a particular claim is related to PNH.
Users should confirm the registration record, effective date and claim classification with the hospital and National Health Insurance Service.
Why the public price is not the final patient bill
HIRA public ceiling prices make the formulas reproducible.
The reference values verified for this guide are KRW 3,600,000 for Soliris 300 mg, KRW 5,044,087 for Ultomiris 300 mg and KRW 17,149,895 for Ultomiris 1,100 mg.
Multiplying these figures by vial counts gives the public-list drug total shown in the result.
That total is not necessarily the amount paid by the patient, the insurer or the hospital.
High-cost medicines can be subject to risk-sharing arrangements, flexible pricing or a separately agreed ceiling that is not publicly available to an unauthorised user.
Hospital billing units, approved coverage, infusion services, laboratory care and later settlement also change the final amount.
The editable unit-price fields exist so a verified provider quote can replace the public benchmark without changing the schedule formula.
The three drug-only comparison rows
- The V187 row applies 10 percent to the entire public-list drug total as a simple registered-and-covered comparison.
- The ordinary row applies the selected general rate to the same drug total as a special-case-not-registered comparison.
- The outside-criteria row shows 100 percent of the public-list total as a conservative self-pay comparison.
Those three rows deliberately ignore the calendar-year ceiling, mixed coverage phases, other care and non-covered costs.
They answer one narrow question about rate sensitivity, while the main result answers the broader mixed-scenario budget question.
Calendar-year ceiling estimate
A treatment window can cross January 1, so the model groups each infusion and each monthly care entry by calendar year.
Covered drug cost and other covered care are multiplied by the selected covered copay rate.
When the optional ceiling switch is on, each calendar-year covered copay is limited to the selected 2026 income-decile reference amount.
Outside-criteria drug cost and non-covered spending remain outside that cap and are added in full.
2026 simplified annual copay ceiling values used in the South Korea PNH model| Income decile | Modeled annual ceiling | Important limitation |
|---|
| 1 | KRW 900,000 | 2026 reference reused for each modeled year |
| 2–3 | KRW 1,120,000 | 2026 reference reused for each modeled year |
| 4–5 | KRW 1,730,000 | 2026 reference reused for each modeled year |
| 6–7 | KRW 3,260,000 | 2026 reference reused for each modeled year |
| 8 | KRW 4,460,000 | 2026 reference reused for each modeled year |
| 9 | KRW 5,360,000 | 2026 reference reused for each modeled year |
| 10 | KRW 8,430,000 | 2026 reference reused for each modeled year |
This optional output is not an NHIS refund decision.
It does not know next-year ceilings, qualifying nursing-hospital days, family and insurance-premium details, other providers, advance versus later reimbursement, or every excluded payment.
It is most useful as a transparent upper-bound sensitivity check before the user obtains an official NHIS statement.
Step-by-step use
- Choose eculizumab or ravulizumab and enter the intended first infusion date. For a ravulizumab switch, use the intended ravulizumab loading date rather than the final eculizumab date.
- Enter current weight and the planning period. Weight changes the ravulizumab band but does not change the modeled adult eculizumab dose.
- Keep the public prices for a reproducible benchmark or replace them with a verified hospital figure. Do not invent a confidential risk-sharing price.
- Copy the age, PNH granulocyte clone and LDH-to-ULN ratio from clinical records. Make sure the LDH denominator is the upper limit printed by the same laboratory.
- Select a clinical complication only when objective supporting documentation exists. Symptoms alone do not reproduce the HIRA criterion.
- Review exclusions and continuation information conservatively. An unknown item should become a question for the treating team, not an assumed pass.
- Turn on V187 only after confirming registration and related covered billing. Add infusion services, other covered care and non-covered household costs in separate fields.
- Compare the generated dates and public-list totals with the provider plan. Ask which difference comes from a schedule, vial, agreed price, coverage classification or later NHIS settlement.
Worked planning scenarios
70 kg ravulizumab for twelve months
The model generates a 2,700 mg loading infusion and seven 3,300 mg maintenance infusions.
Eight infusions have a combined public-list drug value of KRW 404,535,759 at the verified default vial prices.
Applying 10 percent to that drug total alone gives the comparison value KRW 40,453,576 after rounding.
The main output can differ because it separately considers initial and continuation status, infusion services, other care, non-covered spending and the optional ceiling.
Twelve-month visit-frequency comparison
A twelve-month eculizumab reference plan contains twenty-nine modeled infusions, while the 70 kg ravulizumab example contains eight.
The difference can inform travel, work-leave and caregiver planning, but it does not prove that one medicine is medically better.
Response, pregnancy, prior therapy, infection risk, patient circumstances and actual coverage remain clinical and administrative decisions.
Planning before continuation data exist
Before treatment starts, future six-month LDH control and monitoring cannot be known.
A user can first view a scenario that assumes timely monitoring and sustained control, then turn off a continuation check to see the conservative outside-criteria exposure.
The range is a budgeting sensitivity analysis, not a forecast of biological response.
Reconciling a hospital quote
If a hospital estimate differs substantially, compare the medicine strength and vial count first.
Then ask whether the figure uses the public ceiling or an agreed amount, includes infusion services, assumes approved V187-related coverage, or already anticipates a ceiling settlement.
A difference identifies a question; it does not automatically identify an error.
Meningococcal and emergency safeguards
Vaccination does not eliminate meningococcal risk
Both product labels warn about serious meningococcal infection.
The ordinary plan is to complete meningococcal vaccination at least two weeks before treatment, unless the treating clinician concludes that delaying therapy presents the greater risk.
When treatment must begin within that period, the label describes additional management such as appropriate prophylactic antibiotics until the vaccination interval has passed.
Vaccine choice, timing, prophylaxis and booster decisions belong to the clinical team.
Fever with severe headache, neck stiffness, light sensitivity, confusion, rash or marked muscle pain can require immediate emergency assessment even after vaccination.
New chest pain, breathlessness, unilateral limb swelling, a sudden neurological deficit, severe abdominal pain, rapidly darkening urine or profound weakness can signal thrombosis, infection or worsening hemolysis and should not wait for a cost calculation.
A patient must not delay, switch or stop complement-inhibitor therapy without the prescribing team because abrupt interruption can carry serious risk.
Frequently asked questions
Does a 10 percent PNH clone automatically qualify for reimbursement?
No. The clone boundary is only one part of the common pathway. Age, LDH, a documented qualifying complication, exclusions, submitted evidence and the actual HIRA review all matter.
Does V187 make every medical bill 10 percent?
No. It applies to covered care related to the registered PNH condition under the applicable rules. Unrelated care, non-covered care and outside-criteria medicine can be treated differently.
Is the public-list total the amount the patient pays?
No. It is a reproducible benchmark based on public ceiling prices. Coverage approval, a separately agreed or risk-sharing amount, provider billing and later ceiling settlement can change the final payment.
Is ravulizumab always preferable because visits are less frequent?
No. The schedule is only one factor. Medical response, prior treatment, pregnancy, infection risk, patient preference and actual coverage require specialist review.
Does the calculator include pegcetacoplan or iptacopan?
No. This version follows the requested scope and models only the C5 inhibitors eculizumab and ravulizumab. Another medicine must not be substituted into these doses or criteria.
Can generated dates be used as confirmed appointments?
No. They are unshifted label-based reference dates. The prescribing provider decides actual appointments, permitted adjustments and any extra dosing.
What happens when one continuation box is not met?
The model moves later public-list drug cost to the 100 percent outside-criteria column for a conservative budget scenario. It does not instruct the patient to stop medicine and does not reproduce every HIRA exception.
Records to bring to a coverage and cost discussion
- Bring the flow-cytometry report and identify which cell-line clone percentage is being used.
- Bring the LDH value and the upper limit of normal printed by the same laboratory so the ratio is reproducible.
- Keep imaging, anticoagulation, pulmonary, renal and severe-pain records that support any selected clinical pathway.
- Record meningococcal vaccine products and dates, and keep the emergency contact instructions supplied by the clinical team.
- Ask the provider to separate medicine, infusion, laboratory, non-covered, travel and caregiver amounts in a quote.
- Use an official NHIS statement when estimating an annual ceiling because other providers and other covered patient payments can affect settlement.
Official South Korean sources and verification date
The Korean implementation and this English guide were aligned to sources checked on July 23, 2026.
They include the HIRA public PNH drug criteria, the MFDS labels for Soliris item code 201000790 and Ultomiris item code 202204679, the KDCA rare-disease entry for D59.5, Ministry of Health and Welfare Notice No. 2026-101 for V187, and the National Health Insurance Act copay framework.
Prices, criteria, labels and annual ceiling rules can change, so the current official text should be checked again before a new treatment or renewal decision.
Turn a difficult cost discussion into a checkable plan
Use the Korean calculator with the verified records and provider quote to compare infusion dates, public-list drug cost, modeled coverage status, V187 and calendar-year budgeting.
The result is not medical advice, a prescription or a coverage decision, but it can make the next hematology conversation more specific and productive.