For more information about and help in caring for your patient with PNH, download . the Clinical Assessment for Risks of Early Mortality.
A comprehensive critical assessment is crucial to determine the risk for morbidities and premature mortality in your patient with PNH3,4,6,7,9
In addition to looking at outward symptoms, use lab values to help determine your PNH patient’s
risk for serious morbidities and premature mortality. Has your PNH patient presented with any of these lab values or symptoms?
PNH case studies show how lab values and symptoms can be indicative of underlying, life-threatening complications
The following patients underwent more extensive tests when their lab signs and common symptoms indicated the possibility of serious
PNH-related morbidities. Click on their test results to find out what underlying and life-threatening complication was revealed.
Patient with elevated SCr8
- No RBCs in urine under microscope
- Renal impairment
- Elevated creatinine: 4.0 mg/dL (female: 0.6-1.1 mg/dL)
- Signs of hemolysis
- Elevated LDH: 2454 IU/L (105-333 IU/L)
- Indirect hyperbilirubinemia: total/direct bilirubin, 3.01/0.71 mg/dL (total, 0.3 to 1.9 mg/dL; direct, 0 to 0.3 mg/dL)
- A renal biopsy of the patient, following diagnosis, revealed extensive kidney damage and hemosiderin accumulation caused by cell-free hemoglobin. (A) Pigment deposits.* (B) Tubular necrosis and degeneration.
*Arrows indicate pigment deposits.
Patient with dyspnea1
- Lab values from patients
with PNH showed:
- Elevated LDH
- Elevated NT-proBNP
- Multiple perfusion defects in posterior aspects of lungs of a patient with paroxysmal nocturnal hemoglobinuria.*
- Patients had no reported clinical evidence of thrombosis in their medical history
*Arrows indicate areas that are dark due to interrupted perfusion.
Patient with abdominal pain5
- PNH patient presented with lab
values and symptoms including:
- Elevated LDH
- Elevated bilirubin
- Low hemoglobin
- Abdominal pain
- Abdominal CT scan of the case patient at admission. The scan shows parietal thickening of the second portion of the duodenum (arrows).
- No arterial or venous thromboses were seen on angiographic CT examination
Support scientific collaboration in the PNH community, by helping offer the international community greater insight into an uncommon disease with potentially devastating consequences. Enroll your patients in the PNH Registry today.
References: 1. Hill A, Sapsford RJ, Scally A, et al. Under-recognized complications in patients with paroxysmal nocturnal haemoglobinuria: raised pulmonary pressure and reduced right ventricular function. Br J Haematol. 2012;158:409-414. 2. Rother RP, Bell L, Hillmen P, et al. The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease. JAMA. 2005;293:1653-1662. 3. Hill A, Richards SJ, Hillmen P. Recent developments in the understanding and management of paroxysmal nocturnal haemoglobinuria. Br J Haematol. 2007;137:181-192. 4. Nishimura J-I, Kanakura Y, Ware RE, et al. Clinical course and flow cytometric analysis of paroxysmal nocturnal hemoglobinuria in the United States and Japan. Medicine. 2004;83:193-207. 5. Torres J., De Vroey 8, Noel MP, et al. Nat Rev Gastroenterol Hepalol. 2010;7:410-414. 6.Parker C, Omine M, Richards S, et al; for International PNH Interest Group. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood. 2005;106:3699-3709. 7. Hill A, Rother RP, Wang X, et al. Br J Haematol. 2010;149:414-425. 8. Tsai C-W, Wu V-C, Lin W-C, et al. Acute renal failure in a patient with paroxysmal nocturnal hemoglobinuria. Kidney Int. 2007;71:1187. 9. Weitz I, Meyers G, Lamy T, et al. Cross-sectional validation study of patient-reported outcomes in patients with paroxysmal nocturnal haemoglobinuria. Intern Med J. 2013;43:298-307.