Paroxysmal Nocturnal Hemoglobinuria

Updated: June 28, 2024

Background

Epidemiology

Anatomy

Pathophysiology

Etiology

Genetics

Prognostic Factors

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

eculizumab

Dosages 1-4: 600 mg Intravenous every week for the first four weeks, then
Dosage 5: 900 mg Intravenous one week later, following
900 mg Intravenous every two weeks thereafter
Give at or within two days of the recommended dosage regime time points

ravulizumab

The dosing is based on weight, starting with a single loading dose intravenously and then after 2 weeks starting the maintenance dose every 8 weeks
Loading dose-
For 40-60 kg, 2400 mg intravenously
For 60-100 kg- 2700 mg intravenously
For more than 100 kg- 3000 mg intravenously
Maintenance dose-
For intravenous-
Start the maintenance dose 2 weeks later the loading dose
For 40-60 kg, 3000 mg intravenously every 8 weeks
For 60-100 kg- 3300 mg intravenously every 8 weeks
For more than 100 kg- 3600 mg intravenously every 8 weeks
For subcutaneous-
Start the maintenance dose 2 weeks later than the intravenous loading dose
or
8 weeks later, after the last intravenous maintenance dose
For more than 40 kg- 490 mg subcutaneously every week
490 mg through 2 on-body delivery systems
Each system consists of a prefilled cartridge (245 mg)

ravulizumab

Indicated for the treatment in children of more than 1 month
Loading dose-
For 5-10 kg, 600 mg intravenously
For 10-20 kg- 600 mg intravenously
For 20-30 kg, 900 mg intravenously
For 30-40 kg- 1200 mg intravenously
For 40-60 kg, 2400 mg intravenously
For 60-100 kg- 2700 mg intravenously
For more than 100 kg- 3000 mg intravenously
Maintenance dose-
Start the maintenance dose 2 weeks later the loading dose
For 5-10 kg, 300 mg intravenously every 4 weeks
For 10-20 kg- 600 mg intravenously every 4 weeks
For 20-30 kg, 2100 mg intravenously every 8 weeks
For 30-40 kg- 2700 mg intravenously every 8 weeks
For 40-60 kg, 3000 mg intravenously every 8 weeks
For 60-100 kg- 3300 mg intravenously every 8 weeks
For more than 100 kg- 3600 mg intravenously every 8 weeks