A Disease That Causes Ongoing Red Blood Cell Destruction (Haemolysis)
Paroxysmal nocturnal heamoglobinuria (PNH) is an acquired disease that causes some
or all of your body’s red blood cells (RBCs) to be destroyed by a process called
haemolysis.1-3 PNH is a complex disease with signs and symptoms
that are nonspecific, unpredictable, and often similar to those of other diseases.
In addition, PNH presents uniquely in each person. If you have PNH, some or all
of your RBCs may be missing an important protective protein. Without this protein,
some RBCs are prone to destruction by a part of your body's defence system called
complement.1-3
Even though you can't feel it, haemolysis is constant, silent1, and can
be life threatening.1-3 Like other chronic diseases, such as diabetes
or hypertension, PNH can result in serious health problems if not treated. Common
symptoms associated with PNH include stomach pain, difficulty swallowing, anaemia,
shortness of breath, and tiredness (fatigue). More serious complications can include
blood clots, kidney failure, and damage to vital organs.1-3 People with
PNH may have different symptoms that can unpredictably get worse (such as during
times of stress) or better from time to time. However, all people with PNH experience
chronic haemolysis.
People with PNH may have other medical conditions that affect the function of their
bone marrow such as aplastic anaemia (AA) or myelodysplastic syndromes (MDS). Unlike
PNH, which destroys RBCs, these diseases may reduce the production of blood cells
and further complicate PNH.1-3-4 If you have PNH in combination with
AA or MDS, talk to your doctor — it's important to effectively treat all the conditions
you may have.
PNH RBC
PNH RBCs lack an
important protein.
Complement Attack
Without this protein, some RBCs can be destroyed by complement, one of the body's
defence systems.
PNH RBC Lysis (haemolysis)
PNH RBCs are destroyed, and the toxic contents are released into surrounding plasma
(yellow-colored liquid component of blood).