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Donath-Landsteiner Hemolytic Anemia

Donath-Landsteiner Hemolytic Anemia

a.k.a. Paroxysmal Cold Haemoglobinuria

Paroxysmal Cold Haemoglobinuria (PCH) is an autoimmune hemolytic anemia, caused by cold-reacting immunoglobulins. It primarily affects children and tends to cause a quite severe, but transient, disease. The usual trigger for the formation of the cold-reacting Polyclonal Immunogloblulin G (IgG) autoantibodies is an episode of infection. The episode usually, but not always, follows a period of exposure to cold. It is usually a transient postinfectious or postvaccination problem that resolves, but it may cause significant morbidity. The degree of haemolysis is variable but may lead to an acute onset of intravascular haemolysis and haemoglobiuria.

Cross-reactivity (between antibodies formed against microbial antigens and the red-cell membrane P-antigen) inducees intravascular haemolysis through complement activation. The antibodies detectable in the blood of those who suffer from PCH are known as Donath-Landsteiner antibodies.

It is a very rare illness; there are no reliable population-incidence figures, but it is believed to be respnosible for about 40% of all autoimmune haemolytic anaemias that affect children (lower percentage for adults). Although the vast majority of cases occur in children, adults may rarely be affected. The acute, transient form of PCH, by far the most usual modern presentation, is much more common in children.

Illness related:
Viral infections – measles, mumps, adenovirus, chickenpox, influenza A, cytomegalovirus, Epstein-Barr virus.
Bacterial infections – Haemophilus influenzae, Mycoplasma pneumoniae and Treponema pallidum (a chroni recurrent form is associated with secondary, tertiary or congentila syphilis).
Postvaccination syndrome – particularly measles vaccine.
Rarely, when it affecs adults, it may be precipitated by an underlying neoplasm.
Very rare chronic idiopatic/primary autoimmune disease form (usually seen in adults).

Links to websites for further research
www.patient.co.uk/doctor/paroxysmal-cold-haemoglobinuria

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Nuggets of Wisdom

Yes, there is such a thing as having an allergic type reaction to temperatures. This is defined as a physical urticaria. Other physical urticarias include Solar, Aquagenic, Pressure, Vibration and Exercise.

Most reactions are pseudo-allergic. By definition, an allergy involves inhaling or consuming an allergen. Physical urticarias have no known allergen. Despite the terminology and medical definitions, systemic reactions can be life threatening.

Cold has an arbitrary definition based on an individual feeling. For a person with a cold urticaria, cold can be defined as any temperature cooler than their own body temperature.

You do not have to be cold to have a reaction to the cold; contact with cold can trigger a reaction.

You can have an allergic type reaction to both cold and heat simultaneously.

Most reactions considered anaphylactic are really anaphylactoid by definition.

Moving to a warmer climate as a treatment for Cold Urticaria is a myth. Warmer climates present their own issues for those with Cold Urticaria.

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