The test is carried out using a ;piece of equipment called a Chrono-
In addition two other chemicals are added to the samples, called Luciferin and Luciferase. These are extracted from Fireflies). If ATP is released from the platelets then the additional chemicals give of light (bioluminescence) that is detected by the machine.
HPS is a difficult syndrome to accurately test for and diagnose. The severity of the bleeding disorder can vary greatly between patients. Also diagnostic tests can have limitations. For this reason it is best if both Lumi-
The results from Lumi-
However, it is possible for dense granules to be present but NOT releasing, or secreting, ATP and ADP. The results could look very similar to those for HPS. For this reason a Nucleotide assay should be carried out as a ‘double check’.
A nucleotide assay involves preparing a sample of blood platelets and measuring the amounts of ATP and ADP in them. If a patient has HPS then their should be far less ADP than is found in normal platelets. Also, there should be less ATP than normal but the amount of ATP is still greater than the amount of ADP
There are a number of methods used to test for Hermansky-
It is the first two that are mostly widely used in the UK and they are discussed on this page. Details about electron microscopy can be found on another page. These tests are not available in smaller hospitals because they require specialist equipment and laboratories. There are a number of larger hospitals throughout the UK which have specialist Comprehensive Care Centres (CCC) where these tests can be carried out (Click here).
Above: Results for HPS patient and normal control.