Pathophysiology, diagnosis, and treatment of paroxysmal nocturnal hemoglobinuria: a review.

Abstract:

:Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder of the hematopoietic stem cell that makes blood cells more sensitive to the action of complement. Patients experience intravascular hemolysis, smooth muscle dystonia, renal failure, arterial and pulmonary hypertension, recurrent infectious diseases and an increased risk of notably dreadful thrombotic complications. The diagnosis is made by flow cytometry. Efforts have been recently performed to improve the sensitivity and the standardization of this technique. PNH is frequently associated with aplastic anemia or low-risk myelodysplasia and may be asymptomatic. Management of the classical form of PNH has been dramatically revolutionized by the development of eculizumab, which brings benefits in terms of hemolysis, quality of life, renal function, thrombotic risk, and life expectancy. Prophylaxis and treatment of arterial and venous thrombosis currently remain a challenge in PNH.

journal_name

Eur J Haematol

authors

Devalet B,Mullier F,Chatelain B,Dogné JM,Chatelain C

doi

10.1111/ejh.12543

subject

Has Abstract

pub_date

2015-09-01 00:00:00

pages

190-8

issue

3

eissn

0902-4441

issn

1600-0609

journal_volume

95

pub_type

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