Key result
Automated hematology analyzer detects higher BASO-WX and BASO-WY scattergram values in ATGL deficiency.
Why the study?
Adipose triglyceride lipase deficiency manifesting neutral lipid storage disease with myopathy/triglyceride deposit cardiomyovasculopathy presents Jordans' anomaly in peripheral leukocytes, but an automatic notification system for this anomaly was not established.
Can an automated hematology analyzer detect Jordans' anomaly in patients with ATGL deficiency?
Observational
Can an automated hematology analyzer detect Jordans' anomaly in patients with ATGL deficiency?
Automated hematology analyzers may provide an important diagnostic clue for homozygous ATGL deficiency by detecting Jordans' anomaly via elevated BASO-WX and BASO-WY values.
Elevated BASO-WX/WY on XE-5000 may flag Jordans' anomaly; leaves open automated detection utility pending validation.
Adipose triglyceride lipase (ATGL) deficiency manifesting neutral lipid storage disease with myopathy/triglyceride deposit cardiomyovasculopathy presents distinct fat-containing vacuoles known as Jordans' anomaly in peripheral leucocytes. To develop an automatic notification system for Jordans' anomaly in ATGL-deficient patients, we analyzed circulatory leukocyte scattergrams on automated hematology analyzer XE-5000. The BASO-WX and BASO-WY values were found to be significantly higher in patients than those in non-affected subjects. The two parameters measured by automated hematology analyzer may be expected to provide an important diagnostic clue for homozygous ATGL deficiency.
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Suzuki et al. (2014) conducted an observational in Adipose triglyceride lipase (ATGL) deficiency. Automated hematology analyzer XE-5000 vs. Non-affected subjects was evaluated on BASO-WX and BASO-WY values. Automated hematology analyzer XE-5000 detected significantly higher BASO-WX and BASO-WY values in patients with ATGL deficiency compared to non-affected subjects.
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