The Prevalence of Paroxysmal Nocturnal Hemoglobinuria Clone in Adult Patients with Idiopathic Pulmonary Hypertension

Abstract

Aim: Paroxysmal nocturnal hemoglobinuria (PNH) a is a clonal disorder that may lead to several conditions such as thromboses, Budd-Chiari syndrome, renal failure, impotence, and pulmonary hypertension (pHT). Since the presentation of PNH may be occult, monitoring for clonal evolution is recommended in certain situations including aplastic anemia, Myelodysplastic syndrome, and unexplained cytopenia, and thrombosis. The prevalence of PNH clone in patients with idiopathic pHT is unknown. We designed a study to determine the prevalence of PNH clone in patients with idiopathic pHT, since it may be the first isolated presentation of the disease. Methods: A total of 45 patients with pHT were screened for PNH clone by proaerolysin conjugated with fluorescein. Results: Only two out of 45 patients had elevated lactate dehydrogenase (LDH) levels at presentation. PNH clone was detected in none of the patients. Conclusion: Screening for PNH clone in patients with pHT, who have normal LDH levels is unnecessary.

Keywords

• Paroxysmal nocturnal hemoglobinuria
• pulmonary hypertension
• clone