Paroxysmal Nocturnal Hemoglobinuria
Paroxysmal Nocturnal Hemoglobinuria

December 2010
Paroxysmal nocturnal hemoglobinuria is a rare form of acquired hemolytic anemia. The red blood cells get destroyed by complement-mediated lysis which can lead to hemoglobinuria, renal failure and pulmonary hypertension, as well as symptoms such as dysphagia and lethargy. The most serious complication of PNH is venous thrombosis; it often presents in unusual sites such as hepatic, splenic and mesenteric veins. Finally, the disease is characterized by an underlying bone marrow failure presented as severe aplastic anemia in extreme cases. From this variety of symptoms, as well as from the laboratory studies, it is now understood that PNH is a hematopoietic stem cell disorder affecting all blood cell lineages.
Paroxysmal Nocturnal Hemoglobinuria |
Jump to section:
- Introduction
- Paroxysmal Nocturnal Hemoglobinuria
- Prevalence
- Pathogenesis
- Paroxysmal Nocturnal Hemoglobinuria
- Progression
- New Developments
- New Developments, cont.
- Role of Laboratory Testing
- International PNH Group Recommendations: Who to Test?
- Diagnosis
- International PNH Group Recommendation: How to Test?
- Normal
- New WBC PNH
- Sensitivity and Reference Interval
- Interpretation and Reporting
- PNH—Summary
- Questions?


