Tests and Treatment for Paroxysmal Cold Haemoglobinuria (PCH)

Paroxysmal Cold Haemoglobinuria (PCH) is a rare blood disorder that destroys red blood cells at cold temperatures. This article discusses the tests used to diagnose PCH, including the Donath-Landsteiner antibody test, and the treatment options available, such as immunosuppressive drugs. Precautionary measures and close monitoring of the patient are also important. There are no long-term complications, and transfusion is not possible due to the rarity of the P-negative blood group.

Running head: BACTERIAL PATHOGENESIS
BACTERIAL PATHOGENESIS
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1BACTERIAL PATHOGENESIS
Tests for PCH:
PCH: Paroxysmal cold haemoglobinuria has been defined as a rare blood disorder
that leads to the formation of antibodies which destroy the red blood cells of the body
(Vagace et al. 2014). It manifests itself at cold temperatures and majorly affects the hands
and feet of an affected individual. The antibodies present in the body attach themselves to the
blood cells and cause destruction of the blood cells as they move ahead in the body. The
destroyed blood cells are collectively released through the urine outside the body.
With the help of certain laboratory diagnostic tests, the presence of the disease can be
detected(Vagace et al. 2014).
 Negative result of Coomb’s test
 Elevated level of Lactate dehydrogenase
 Elevated level of Bilirubin in urine and blood
 Positive result of Donath-landstiener antibody test
 Detection of anaemia on the basis of complete blood count test
The Donath-Landsteiner antibody test is used most commonly as the diagnostic
confirmatory test for PCH. Positive test result is characterized by the detection of Donath
Landsteiner antibodies within the blood sample whereas negative test result is characterized
by the absence of the antibodies (Vagace et al. 2014). This medical test is based upon the fact
that the blood of the patients comprises of complement dependent iso and haemolysin
antibodies. These complements unite with the red blood cells only at low temperatures
approximately around 2 to 10 degree centigrade. The process of haemolysis then occurs at
around 37 degree centigrade (Vagace et al. 2014). The direct antiglobulin test is negative for
the disease condition. This is because the human clonal antibody Eculizumab inhibits the C5
complement and tests negative for the condition of intravascular haemolysis.

2BACTERIAL PATHOGENESIS
Treatment of PCH
The treatment of PCH is based upon a wide range of medication and therapies. The
medication primarily prescribed in order to treat the disease is based upon a wide range of
immunosuppressive drugs. Immunosuppressive drugs work by inhibiting the functioning of
the immune system. Immuno-suppresants work by reducing the strength of the immune
system to elicit a stronger immune response or produce stronger antibodies (Shanbhag and
Spivak 2015). At the same time, the best treatment procedure for the disease condition is to
treat the underlying condition of the disease. For instance, research studies state that if the
disease condition is a result of another medical condition such as Syphilis, then treating the
disease condition would help in the treatment of PCH.
Since the condition is primarily characterized by intense cold, therefore it can be said
that adopting precautionary measures and staying away from cold can help in preventing the
possibility of developing the disease (Höchsmann et al. 2012). In addition to this close
monitoring of the diagnosed patient is important to trace the minute progression of the
disease. Some new methods such as the use of B-cell receptor inhibitors and targeting IgG for
extravascular haemolysis are at the trial stages and are yet to be assimilated in the treatment
procedure.
There are no long-term complications and the treatment intervention for the disease
condition is supportive. It should be noted here that that there is no possibility of transfusion
because the P- negative blood group is rare (Barcellini and Fattizzo 2018).

3BACTERIAL PATHOGENESIS
References:
Barcellini, W., and Fattizzo, B. 2018. Autoimmune hemolyticanemia-progress in emerging
treatment options. Expert Opinion on Orphan Drugs, 6(4), 273-282.
Höchsmann, B., Leichtle, R., von Zabern, I., Kaiser, S., Flegel, W. A., and Schrezenmeier, H.
2012. Paroxysmal nocturnal haemoglobinuria treatment with eculizumab is associated
with a positive direct antiglobulin test. Voxsanguinis, 102(2), 159-166.
Shanbhag, S., and Spivak, J. 2015. Paroxysmal cold hemoglobinuria. Hematology/Oncology
Clinics, 29(3), 473-478.
Vagace, J. M., Bajo, R., and Gervasini, G. 2014. Diagnostic and therapeutic challenges of
primary autoimmune haemolytic anaemia in children. Archives of disease in
childhood, 99(7), 668-673.