PAROXYSMAL HAEMOGLOBINURIA

Paroxysmal Nocturnal Haemoglobinuria

A Clinico-Pathological Conference held in the University of Bristol on the 6th November 1962 chairman: professor t. f. hewer Dr. Crow: This lady was 57 at the time of death and although her early history isi little scanty it seems likely that the disease which ultimately caused her death first presented around the age of 21. We have nothing like complete notes up to 1953, but even so her notes ...

Paroxysmal cold haemoglobinuria and measles.

With the increasing control of congenital syphilis, paroxysmal cold haemoglobinuria has become a very rare disorder in childhood. Cases which occur now are more likely to be associated with acute infectious diseases (Kissmeyer-Nielsen and Schleisner, 1963). However, despite the frequency of measles in children, paroxysmal cold haemoglobinuria (PCH) has only previously been described as a compli...

Eculizumab for paroxysmal nocturnal haemoglobinuria.

The complement system plays a central part in both innate and acquired immunity, but the contribution of complement activation to pathobiology is largely ancillary. An exception to the non-dominant role of complement in disease is the haemolytic anaemia of paroxysmal nocturnal haemoglobinuria (PNH). The intravascular haemolysis that is the clinical hallmark of PNH is a consequence of deficiency...

Paroxysmal cold haemoglobinuria in syphilis patients.

Paroxysmal nocturnal haemoglobinuria: nature's gene therapy?

The development of paroxysmal nocturnal haemoglobinuria (PNH) requires two coincident factors: somatic mutation of the PIG-A gene in one or more haemopoietic stem cells and an abnormal, hypoplastic bone marrow environment. When both of these conditions are met, the fledgling PNH clone may flourish. This review will discuss the pathophysiology of this disease, which has recently been elucidated ...